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Sample records for parkinson dementia syndromes

  1. Imaging Amyloidopathy in Parkinson Disease and Parkinsonian Dementia Syndromes.

    PubMed

    Frey, Kirk A; Petrou, Myria

    2015-02-01

    Dementia arising in patients with Parkinson disease or parkinsonian neurodegeneration comprises a heterogeneous neuropathology. Clinical labeling of patients with both dementia and Parkinson disease is dichotomous, depending on the temporal development of cognitive impairment and motor parkinsonism. Patients with dementia arising first (or within the first year of PD) are classified as dementia with Lewy bodies; patients with PD for more than one year before cognitive decline are classified as Parkinson disease with dementia. Despite this differential clinical classification, autopsy studies demonstrate variable admixtures of cortical synuicleinopathy, Aβ-amyloidopathy and tau neurofibrillary tangle deposition. There are no routine clinical diagnostic measures that accurately distinguish the underlying neuropathologies in individual patients. In the present paper, we review the published literature describing characteristics of fibrillary Aβ-amyloid deposition on the basis of PET radiotracer imaging in patients with Parkinson disease and in parkinsonian dementia syndromes. Although individual reports often include only small-to-modest subject numbers, there is overall suggestion that PD patients have a lower incidence of Aβ-amyloid deposition than seen amongst elderly normal subjects, and that Parkinson disease with dementia patients have a lower incidence of Aβ-amyloid deposition than do patients with dementia with Lewy bodies. These apparent features contrast the findings of Aβ-amyloid-PET imaging in normal aging and the development of Alzheimer disease, where Aβ-amyloid deposition arises asymptomatically and apparently many years before development of signs or symptoms of dementia. It is proposed that focused, prospective studies are needed to further address and understand the complex role(s) of Aβ-amyloid pathology in Parkinson disease, and that this understanding will be critical to the development of targeted disease-modifying therapy for dementia in

  2. Syndromic parkinsonism and dementia associated with OPA 1 missense mutations

    PubMed Central

    Musumeci, Olimpia; Caporali, Leonardo; Zanna, Claudia; La Morgia, Chiara; Del Dotto, Valentina; Porcelli, Anna Maria; Rugolo, Michela; Valentino, Maria Lucia; Iommarini, Luisa; Maresca, Alessandra; Barboni, Piero; Carbonelli, Michele; Trombetta, Costantino; Valente, Enza Maria; Patergnani, Simone; Giorgi, Carlotta; Pinton, Paolo; Rizzo, Giovanni; Tonon, Caterina; Lodi, Raffaele; Avoni, Patrizia; Liguori, Rocco; Baruzzi, Agostino; Toscano, Antonio; Zeviani, Massimo

    2015-01-01

    Objective Mounting evidence links neurodegenerative disorders such as Parkinson disease and Alzheimer disease with mitochondrial dysfunction, and recent emphasis has focused on mitochondrial dynamics and quality control. Mitochondrial dynamics and mtDNA maintenance is another link recently emerged, implicating mutations in the mitochondrial fusion genes OPA1 and MFN2 in the pathogenesis of multisystem syndromes characterized by neurodegeneration and accumulation of mtDNA multiple deletions in postmitotic tissues. Here, we report 2 Italian families affected by dominant chronic progressive external ophthalmoplegia (CPEO) complicated by parkinsonism and dementia. Methods Patients were extensively studied by optical coherence tomography (OCT) to assess retinal nerve fibers, and underwent muscle and brain magnetic resonance spectroscopy (MRS), and muscle biopsy and fibroblasts were analyzed. Candidate genes were sequenced, and mtDNA was analyzed for rearrangements. Results Affected individuals displayed a slowly progressive syndrome characterized by CPEO, mitochondrial myopathy, sensorineural deafness, peripheral neuropathy, parkinsonism, and/or cognitive impairment, in most cases without visual complains, but with subclinical loss of retinal nerve fibers at OCT. Muscle biopsies showed cytochrome c oxidase‐negative fibers and mtDNA multiple deletions, and MRS displayed defective oxidative metabolism in muscle and brain. We found 2 heterozygous OPA1 missense mutations affecting highly conserved amino acid positions (p.G488R, p.A495V) in the guanosine triphosphatase domain, each segregating with affected individuals. Fibroblast studies showed a reduced amount of OPA1 protein with normal mRNA expression, fragmented mitochondria, impaired bioenergetics, increased autophagy and mitophagy. Interpretation The association of CPEO and parkinsonism/dementia with subclinical optic neuropathy widens the phenotypic spectrum of OPA1 mutations, highlighting the association of

  3. Parkinson Disease and Dementia.

    PubMed

    Garcia-Ptacek, Sara; Kramberger, Milica G

    2016-09-01

    Dementia is a frequent complication of Parkinson disease (PD) with a yearly incidence of around 10% of patients with PD. Lewy body pathology is the most important factor in the development of Parkinson disease dementia (PDD) and there is evidence for a synergistic effect with β-amyloid. The clinical phenotype in PDD extends beyond the dysexecutive syndrome that is often present in early PD and encompasses deficits in recognition memory, attention, and visual perception. Sleep disturbances, hallucinations, neuroleptic sensitivity, and fluctuations are often present. This review provides an update on current knowledge of PDD including aspects of epidemiology, pathology, clinical presentation, management, and prognosis. © The Author(s) 2016.

  4. [Alzheimer's disease with secondary Parkinson's syndrome. Case report of a patient with dementia and Parkinson's syndrome after long-term occupational exposure to insecticides, herbicides, and pesticides].

    PubMed

    Laske, C; Wormstall, H; Einsiedler, K; Buchkremer, G

    2004-11-01

    This case report describes long-term occupational exposure to agricultural insecticides, herbicides, and pesticides as possible environmental risk factors of Alzheimer's disease (AD) and Parkinson's syndrome in a 59-year-old man. Initially the patient complained about disturbances in concentration, mnestic deficits, and problems finding words. In the further course of the disease, he developed Parkinson's syndrome with predominant hypokinesia and rigor in addition to mild-to-moderate dementia. Low levels of beta-amyloid 1-42 were found in the CSF. Electroencephalography showed left frontotemporal theta waves. Cranial MRI revealed general brain atrophy with a maximum biparietally. In cerebral positron emission tomography, general hypometabolism was found with maxima biparietally and left frontally. The possible differential diagnosis of AD and Parkinson's syndrome is discussed.

  5. Apathy and related executive syndromes in dementia associated with Parkinson's disease and in Alzheimer's disease.

    PubMed

    Grossi, Dario; Santangelo, Gabriella; Barbarulo, Anna Maria; Vitale, Carmine; Castaldo, Giovanna; Proto, Maria Grazia; Siano, Pietro; Barone, Paolo; Trojano, Luigi

    2013-01-01

    Apathy is defined as a lack of motivation and has been reported to be common in Alzheimer's disease (AD) and Parkinson's disease (PD). To explore the neuropsychological correlates of apathy in patients with PD related dementia (PDD) and AD and to identify the specific cognitive profile of apathy in the two forms of neurodegenerative disease, 61 non-depressed patients (29 PDD and 32 AD) were selected. Out of these, 29 patients (47.5%) were detected as apathetic (14 PDD-A+ and 15 AD-A+), and 32 patients as non-apathetic (15 PDD-A- and 17 AD-A-). All patients underwent cognitive tasks tapping memory, visuospatial and executive functions, behavioral rating scales and Clinical Judgment for Apathy Syndrome (CJ-AS), an inventory developed to measure severity of apathy. The four subgroups differed significantly on memory and frontal tasks. The PDD-A+ performed significantly worse than PDD-A- on frontal tasks. The AD-A+ had poorer performance than AD-A- on frontal tasks. Last, PDD-A+ achieved significantly higher scores than AD-A+ on memory tasks. The four groups differed significantly on CJ-AS and behavioral rating scales.The results showed that apathetic patients with both forms of dementia showed a common neuropsychological and behavioral picture, characterized by defects on frontal tasks, thus strongly supporting the existence of an 'apathetic syndrome', characterized by specific cognitive and psychological symptoms.

  6. [Neurological features of decision-making deficit: Korinai syndrome in Parkinson disease and fearlessness in dementia].

    PubMed

    Iwata, Makoto

    2012-10-01

    For patients with Parkinson disease, falls are generally attributed to postural instability. However, closer examination often shows that parkinsonian patients tend to keep falling at the same spot in their home on performing the same actions such as standing up from a chair, starting to walk, or turning at the corner and hitting the same part of the head. Each time the patients fall, their treating physicians explain the reasons for falling and tell them how to avoid falls in daily life. In spite of the repeated advice given by physicians, these patients fall in the same manner and location. When physicians question them regarding the reason for repeating the same risky actions that they had been asked to avoid, the patients answer that they clearly remembered their physician's advice but had thought that they would be able to successfully accomplish the risky actions at that time. Thus, it seems that this type of fall is partly caused by decision-making deficit. Negative-reward motor learning is known to be defective in parkinsonian patients who are being treated with dopamine agonists and are liable to indulge in risky behavior and tend to be involved in pathological gambling. The behavioral abnormalities that are caused by defective decision making and are common to pathological gambling and repeated falling in parkinsonian patients could be termed as "Korinai syndrome, " which means "syndrome involving the inability to learn by experience." In contrast, patients with dementia often show loss of fear reaction, which may result in the life-threatening inability to perceive crises. The present author performed a series of studies just after the Great East Japan Earthquake in March 2011; these studies were based on the behavior of patients with dementia during this large earthquake. The results showed that both intellectually normal elderly people and patients with mild dementia had shown evident fear reactions and could remember their own fearful experience

  7. C9ORF72 intermediate repeat expansion in patients affected by atypical parkinsonian syndromes or Parkinson's disease complicated by psychosis or dementia in a Sardinian population.

    PubMed

    Cannas, Antonino; Solla, Paolo; Borghero, Giuseppe; Floris, Gian Luca; Chio, Adriano; Mascia, Marcello Mario; Modugno, Nicola; Muroni, Antonella; Orofino, Gianni; Di Stefano, Francesca; Calvo, Andrea; Moglia, Cristina; Restagno, Gabriella; Meloni, Mario; Farris, Rita; Ciaccio, Daniela; Puddu, Roberta; Vacca, Melisa Iris; Melis, Rosanna; Murru, Maria Rita; Tranquilli, Stefania; Corongiu, Daniela; Rolesu, Marcella; Cuccu, Stefania; Marrosu, Maria Giovanna; Marrosu, Francesco

    2015-11-01

    The hexanucleotide repeat expansion GGGGCC in the C9ORF72 gene larger than 30 repeats has been identified as a major genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Recent papers investigated the possible pathogenic role and associated clinical phenotypes of intermediate C9ORF72 repeat expansion ranging between 20 and 30 repeats. Some studies suggested its pathogenicity for typical Parkinson's disease (PD), atypical parkinsonian syndromes, FTD with/without parkinsonism, and ALS with/without parkinsonism or with/without dementia. In our study, we aimed to screen patients affected by atypical parkinsonian syndromes or PD complicated by psychosis or dementia for the presence of C9ORF72 repeat expansions, and in unrelated age- and sex-matched healthy controls. Consecutive unrelated patients with atypical parkinsonian syndromes and patients with PD complicated by psychosis or dementia were included in this study. Atypical parkinsonian syndromes were further divided into two groups: one with patients who met the criteria for the classic forms of atypical parkinsonism [multiple system atrophy (MSA), Lewy body disease (LBD), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD)] ;and patients who did not meet the above criteria, named non-classical atypical parkinsonism with or without dementia. Ninety-two unrelated patients (48 men, 44 women) were enrolled. None of the patients was found to be carriers of C9ORF72 repeat expansions with more than 30 repeats. Intermediate 20-30 repeat expansions were detected in four female patients (4.3 %). Three of them presented clinical features of atypical parkinsonian syndromes, two with non-classical atypical parkinsonism and dementia FTD-like, and one with non-classical atypical parkinsonism without dementia. The other patient presented clinical features of typical PD complicated by psychosis. Among 121 control subjects, none presented long or short expansion for the C9ORF

  8. Parkinson's Disease Dementia

    MedlinePlus

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  9. Behavioural assessment of dysexecutive syndrome in Parkinson's disease without dementia: a comparison with other clinical executive tasks.

    PubMed

    Perfetti, Bernardo; Varanese, Sara; Mercuri, Pasqua; Mancino, Elisa; Saggino, Aristide; Onofrj, Marco

    2010-01-01

    The Behavioural Assessment of the Dysexecutive Syndrome (BADS) is a neuropsychological battery developed with the intent of measuring a wide range of executive impairments. Although the psychometric characteristics of BADS have previously been investigated in distinct neurological disorders, data on its validity in Parkinson's Disease (PD) without dementia are still lacking. The principal aim of the study was to address this issue. Twenty-five non-demented PD patients and 24 demographically-matched controls were administered BADS and other commonly used executive tools. Comparisons between groups indicated that two of the six BADS subtests (Temporal Judgement and Action Program) did not have sufficient sensitivity to executive impairments. However, when we explored group-predictive capabilities among the tests, the BADS total score was the most sensitive, followed by the Tower of London (TOL). We obtained similar results when we disentangled the sensitivity of the six BADS subtests. The BADS Six Elements task was the best group predictor followed by the TOL. Our findings showed that BADS is more sensitive to executive dysfunction than some of the tools commonly used to assess this construct in PD. However, we also demonstrated that, to assess executive impairments in PD without dementia adequately, this battery should be administered in combination with the TOL.

  10. Lewy Body Dementias: Dementia With Lewy Bodies and Parkinson Disease Dementia.

    PubMed

    Gomperts, Stephen N

    2016-04-01

    This article provides an overview of the clinical features, neuropathologic findings, diagnostic criteria, and management of dementia with Lewy bodies (DLB) and Parkinson disease dementia (PDD), together known as the Lewy body dementias. DLB and PDD are common, clinically similar syndromes that share characteristic neuropathologic changes, including deposition of α-synuclein in Lewy bodies and neurites and loss of tegmental dopamine cell populations and basal forebrain cholinergic populations, often with a variable degree of coexisting Alzheimer pathology. The clinical constellations of DLB and PDD include progressive cognitive impairment associated with parkinsonism, visual hallucinations, and fluctuations of attention and wakefulness. Current clinical diagnostic criteria emphasize these features and also weigh evidence for dopamine cell loss measured with single-photon emission computed tomography (SPECT) imaging and for rapid eye movement (REM) sleep behavior disorder, a risk factor for the synucleinopathies. The timing of dementia relative to parkinsonism is the major clinical distinction between DLB and PDD, with dementia arising in the setting of well-established idiopathic Parkinson disease (after at least 1 year of motor symptoms) denoting PDD, while earlier cognitive impairment relative to parkinsonism denotes DLB. The distinction between these syndromes continues to be an active research question. Treatment for these illnesses remains symptomatic and relies on both pharmacologic and nonpharmacologic strategies. DLB and PDD are important and common dementia syndromes that overlap in their clinical features, neuropathology, and management. They are believed to exist on a spectrum of Lewy body disease, and some controversy persists in their differentiation. Given the need to optimize cognition, extrapyramidal function, and psychiatric health, management can be complex and should be systematic.

  11. Dementia in Parkinson's disease: the problem of clinicopathological correlation.

    PubMed

    Sudarsky, L; Morris, J; Romero, J; Walshe, T M

    1989-01-01

    Four cases of Parkinson's disease with advanced dementia are described. Postmortem examination revealed cell loss in the substantia nigra, with Lewy bodies present, and loss of cells in the basal nucleus of Meynert. A few tangles were observed in the hippocampus, but no senile plaques or neurofibrillary tangles were found in the neocortex. The authors note that a dramatic dementia syndrome may occur with Parkinson's disease alone, without the associated cytoskeletal markers of Alzheimer's disease. Cases were characterized by disorientation, episodic confusion and hallucinations persisting off medication, disturbed behavior, and the absence of aphasia.

  12. Predictors of dementia in Parkinson disease

    PubMed Central

    Anang, Julius B.M.; Bertrand, Josie-Anne; Romenets, Silvia Rios; Latreille, Veronique; Panisset, Michel; Montplaisir, Jacques

    2014-01-01

    Objective: We investigated an array of possible markers of early dementia in Parkinson disease. Methods: We performed a comprehensive assessment of autonomic, sleep, psychiatric, visual, olfactory, and motor manifestations in 80 patients with Parkinson disease who were dementia-free at baseline. After 4.4 years’ follow-up, patients were evaluated for dementia. Predictive variables were assessed using logistic regression adjusting for disease duration, follow-up duration, age, and sex. Results: Of 80 patients, 27 (34%) developed dementia. Patients destined to develop dementia were older and more often male (odds ratio [OR] = 3.64, p = 0.023). Those with baseline mild cognitive impairment had increased dementia risk (OR = 22.5, p < 0.001). REM sleep behavior disorder at baseline dramatically increased dementia risk (OR = 49.7, p = 0.001); however, neither daytime sleepiness nor insomnia predicted dementia. Higher baseline blood pressure increased dementia risk (OR = 1.37 per 10 mm Hg, p = 0.032). Orthostatic blood pressure drop was strongly associated with dementia risk (OR = 1.84 per 10 mm Hg, p < 0.001); having a systolic drop of >10 mm Hg increased dementia odds 7-fold (OR = 7.3, p = 0.002). Abnormal color vision increased dementia risk (OR = 3.3, p = 0.014), but olfactory dysfunction did not. Among baseline motor variables, proportion of gait involvement (OR = 1.12, p = 0.023), falls (OR = 3.02, p = 0.042), and freezing (OR = 2.63, p = 0.013), as well as the Purdue Pegboard Test (OR = 0.67, p = 0.049) and alternate tap test (OR = 0.97, p = 0.033) predicted dementia. Conclusion: Cardiovascular autonomic dysfunction, REM sleep behavior disorder, color discrimination ability, and gait dysfunction strongly predict development of dementia in Parkinson disease. PMID:25171928

  13. Cholinergic and other neurotransmitter mechanisms in Parkinson's disease, Parkinson's disease dementia, and dementia with Lewy bodies.

    PubMed

    Francis, Paul T; Perry, Elaine K

    2007-09-01

    It is now 30 years since the beginning of intensive efforts to understand the neurotransmitter biochemistry of dementia as exemplified by Alzheimer's disease and such studies have led to the development of rational treatment strategies, which are continuing to benefit patients. However, as studies became more sophisticated and clinicians rediscovered an interest in dementia, because of the potential for symptomatic treatment, it has become clear that there are several different neurodegenerative conditions that gives rise to dementia syndromes and that each has distinct neurochemical pathology. This has important treatment implications since what works for one may not work for another or at the extreme, may make matters worse. Therefore it is clear that a detailed understanding of the neurotransmitter function in each condition is not merely academic but can lead to rationale drug design and treatment strategies appropriate for that group of patients. Dementia with Lewy bodies (DLB) has clinico-pathological features, which overlap with either AD or Parkinson's disease (PD) as well as features that help to distinguish it, such as fluctuations in cognitive impairment and a higher prevalence of visual hallucinations. On this basis, it would be expected that the neurochemistry would have some similarities with both disorders.

  14. Parkinsonian syndrome in familial frontotemporal dementia.

    PubMed

    Siuda, Joanna; Fujioka, Shinsuke; Wszolek, Zbigniew K

    2014-09-01

    Parkinsonism in frontotemporal dementia (FTD) was first described in families with mutations in the microtubule-associated protein tau (MAPT) and progranulin (PRGN) genes. Since then, mutations in several other genes have been identified for FTD with parkinsonism, including chromosome 9 open reading frame 72 (C9ORF72), chromatin modifying protein 2B (CHMP2B), valosin-containing protein (VCP), fused in sarcoma (FUS) and transactive DNA-binding protein (TARDBP). The clinical presentation of patients with familial forms of FTD with parkinsonism is highly variable. The parkinsonism seen in FTD patients is usually characterized by akinetic-rigid syndrome and is mostly associated with the behavioral variant of FTD (bvFTD); however, some cases may present with classical Parkinson's disease. In other cases, atypical parkinsonism resembling progressive supranuclear palsy (PSP) or corticobasal syndrome (CBS) has also been described. Although rare, parkinsonism in FTD may coexist with motor neuron disease. Structural neuroimaging, which is crucial for the diagnosis of FTD, shows characteristic patterns of brain atrophy associated with specific mutations. Structural neuroimaging is not helpful in distinguishing among patients with parkinsonian features. Furthermore, dopaminergic imaging that shows nigrostriatal neurodegeneration in FTD with parkinsonism cannot discriminate parkinsonian syndromes that arise from different mutations. Generally, parkinsonism in FTD is levodopa unresponsive, but there have been cases where a temporary benefit has been reported, so dopaminergic treatment is worth trying, especially, when motor and non-motor manifestations can cause significant problems with daily functioning. In this review, we present an update on the clinical and genetic correlations of FTD with parkinsonism. Copyright © 2014 The Authors. Published by Elsevier Ltd.. All rights reserved.

  15. Parkinsonian Syndrome in Familial Frontotemporal Dementia

    PubMed Central

    Siuda, Joanna; Fujioka, Shinsuke; Wszolek, Zbigniew K.

    2014-01-01

    Parkinsonism in frontotemporal dementia (FTD) was first described in families with mutations in the microtubule-associated protein tau (MAPT) and progranulin (PRGN) genes. Since then, mutations in several other genes have been identified for FTD with Parkinsonism, including chromosome 9 open reading frame 72 (C9ORF72), chromatin modifying protein 2B (CHMP2B), valosin-containing protein (VCP), fused in sarcoma (FUS) and transactive DNA-binding protein (TARDBP). The clinical presentation of patients with familial forms of FTD with Parkinsonism is highly variable. The Parkinsonism seen in FTD patients is usually characterized by akinetic-rigid syndrome and is mostly associated with the behavioral variant of FTD (bvFTD); however, some cases may present with classical Parkinson’s disease. In other cases, atypical Parkinsonism resembling progressive supranuclear palsy (PSP) or corticobasal syndrome (CBS) has also been described. Although rare, Parkinsonism in FTD may coexist with motor neuron disease. Structural neuroimaging, which is crucial for the diagnosis of FTD, shows characteristic patterns of brain atrophy associated with specific mutations. Structural neuroimaging is not helpful in distinguishing among patients with parkinsonian features. Furthermore, dopaminergic imaging that shows nigrostriatal neurodegeneration in FTD with Parkinsonism cannot discriminate parkinsonian syndromes that arise from different mutations. Generally, Parkinsonism in FTD is levodopa unresponsive, but there have been cases where a temporary benefit has been reported, so dopaminergic treatment is worth trying, especially, when motor and non-motor manifestations can cause significant problems with daily functioning. In this review, we present an update on the clinical and genetic correlations of FTD with Parkinsonism. PMID:24998994

  16. Cerebral Microbleeds in Patients with Dementia with Lewy Bodies and Parkinson Disease Dementia.

    PubMed

    Kim, S W; Chung, S J; Oh, Y-S; Yoon, J H; Sunwoo, M K; Hong, J Y; Kim, J-S; Lee, P H

    2015-09-01

    The burden of amyloid β is greater in patients with dementia with Lewy bodies than in those with Parkinson disease dementia, and an increased amyloid β load is closely related to a higher incidence of cerebral microbleeds. Here, we investigated the prevalence and topography of cerebral microbleeds in patients with dementia with Lewy bodies and those with Parkinson disease dementia to examine whether cerebral microbleeds are more prevalent in patients with dementia with Lewy bodies than in those with Parkinson disease dementia. The study population consisted of 42 patients with dementia with Lewy bodies, 88 patients with Parkinson disease dementia, and 35 controls who underwent brain MR imaging with gradient recalled-echo. Cerebral microbleeds were classified as deep, lobar, or infratentorial. The frequency of cerebral microbleeds was significantly greater in patients with dementia with Lewy bodies (45.2%) than in those with Parkinson disease dementia (26.1%) or in healthy controls (17.1%; P = .017). Lobar cerebral microbleeds were observed more frequently in the dementia with Lewy bodies group (40.5%) than in the Parkinson disease dementia (17%; P = .004) or healthy control (8.6%; P = .001) group, whereas the frequencies of deep and infratentorial cerebral microbleeds did not differ among the 3 groups. Logistic regression analyses revealed that, compared with the healthy control group, the dementia with Lewy bodies group was significantly associated with the presence of lobar cerebral microbleeds after adjusting for age, sex, nonlobar cerebral microbleeds, white matter hyperintensities, and other vascular risk factors (odds ratio, 4.39 [95% CI, 1.27-15.25]). However, compared with the healthy control group, the Parkinson disease dementia group was not significantly associated with lobar cerebral microbleeds. This study showed that patients with dementia with Lewy bodies had a greater burden of cerebral microbleeds and exhibited a lobar predominance of cerebral

  17. Parkinsonism, movement disorders and genetics in frontotemporal dementia.

    PubMed

    Baizabal-Carvallo, José Fidel; Jankovic, Joseph

    2016-03-01

    Frontotemporal dementia (FTD) refers to a group of clinically and genetically heterogeneous neurodegenerative disorders that are a common cause of adult-onset behavioural and cognitive impairment. FTD often presents in combination with various hyperkinetic or hypokinetic movement disorders, and evidence suggests that various genetic mutations underlie these different presentations. Here, we review the known syndromatic-genetic correlations in FTD. Although no direct genotype-phenotype correlations have been identified, mutations in multiple genes have been associated with various presentations. Mutations in the genes that encode microtubule-associated protein tau (MAPT) and progranulin (PGRN) can manifest as symmetrical parkinsonism, including the phenotypes of Richardson syndrome and corticobasal syndrome (CBS). Expansions in the C9orf72 gene are most frequently associated with familial FTD, typically combined with motor neuron disease, but other manifestations, such as symmetrical parkinsonism, CBS and multiple system atrophy-like presentations, have been described in patients with these mutations. Less common gene mutations, such as those in TARDBP, CHMP2B, VCP, FUS and TREM2, can also present as atypical parkinsonism. The most common hyperkinetic movement disorders in FTD are motor and vocal stereotypies, which have been observed in up to 78% of patients with autopsy-proven FTD. Other hyperkinetic movements, such as chorea, orofacial dyskinesias, myoclonus and dystonia, are also observed in some patients with FTD.

  18. Biomarkers for dementia and mild cognitive impairment in Parkinson's disease.

    PubMed

    Delgado-Alvarado, Manuel; Gago, Belén; Navalpotro-Gomez, Irene; Jiménez-Urbieta, Haritz; Rodriguez-Oroz, María C

    2016-06-01

    Cognitive decline is one of the most frequent and disabling nonmotor features of Parkinson's disease. Around 30% of patients with Parkinson's disease experience mild cognitive impairment, a well-established risk factor for the development of dementia. However, mild cognitive impairment in patients with Parkinson's disease is a heterogeneous entity that involves different types and extents of cognitive deficits. Because it is not currently known which type of mild cognitive impairment confers a higher risk of progression to dementia, it would be useful to define biomarkers that could identify these patients to better study disease progression and possible interventions. In this sense, the identification among patients with Parkinson's disease and mild cognitive impairment of biomarkers associated with dementia would allow the early detection of this process. This review summarizes studies from the past 25 years that have assessed the potential biomarkers of dementia and mild cognitive impairment in Parkinson's disease patients. Despite the potential importance, no biomarker has as yet been validated. However, features such as low levels of epidermal and insulin-like growth factors or uric acid in plasma/serum and of Aß in CSF, reduction of cerebral cholinergic innervation and metabolism measured by PET mainly in posterior areas, and hippocampal atrophy in MRI might be indicative of distinct deficits with a distinct risk of dementia in subgroups of patients. Longitudinal studies combining the existing techniques and new approaches are needed to identify patients at higher risk of dementia. © 2016 International Parkinson and Movement Disorder Society.

  19. Predictors of dementia in Parkinson disease: a prospective cohort study.

    PubMed

    Anang, Julius B M; Gagnon, Jean-Francois; Bertrand, Josie-Anne; Romenets, Silvia Rios; Latreille, Veronique; Panisset, Michel; Montplaisir, Jacques; Postuma, Ronald B

    2014-09-30

    We investigated an array of possible markers of early dementia in Parkinson disease. We performed a comprehensive assessment of autonomic, sleep, psychiatric, visual, olfactory, and motor manifestations in 80 patients with Parkinson disease who were dementia-free at baseline. After 4.4 years' follow-up, patients were evaluated for dementia. Predictive variables were assessed using logistic regression adjusting for disease duration, follow-up duration, age, and sex. Of 80 patients, 27 (34%) developed dementia. Patients destined to develop dementia were older and more often male (odds ratio [OR] = 3.64, p = 0.023). Those with baseline mild cognitive impairment had increased dementia risk (OR = 22.5, p < 0.001). REM sleep behavior disorder at baseline dramatically increased dementia risk (OR = 49.7, p = 0.001); however, neither daytime sleepiness nor insomnia predicted dementia. Higher baseline blood pressure increased dementia risk (OR = 1.37 per 10 mm Hg, p = 0.032). Orthostatic blood pressure drop was strongly associated with dementia risk (OR = 1.84 per 10 mm Hg, p < 0.001); having a systolic drop of >10 mm Hg increased dementia odds 7-fold (OR = 7.3, p = 0.002). Abnormal color vision increased dementia risk (OR = 3.3, p = 0.014), but olfactory dysfunction did not. Among baseline motor variables, proportion of gait involvement (OR = 1.12, p = 0.023), falls (OR = 3.02, p = 0.042), and freezing (OR = 2.63, p = 0.013), as well as the Purdue Pegboard Test (OR = 0.67, p = 0.049) and alternate tap test (OR = 0.97, p = 0.033) predicted dementia. Cardiovascular autonomic dysfunction, REM sleep behavior disorder, color discrimination ability, and gait dysfunction strongly predict development of dementia in Parkinson disease. © 2014 American Academy of Neurology.

  20. Predictors of survival in dementia with lewy bodies and Parkinson dementia.

    PubMed

    Jellinger, Kurt A; Wenning, Gregor K; Seppi, Klaus

    2007-01-01

    Retrospective analysis of 243 autopsy-confirmed cases of dementia with Lewy bodies (DLB) and Parkinson disease with dementia (PDD) showed an average age at symptom onset of 67 years and a median survival of 5 years from symptom onset. Older age at onset, fluctuating cognition, and hallucinations at onset predicted shorter survival; initial parkinsonism with delayed dementia significantly improved survival. Associated Alzheimer pathology also shortened survival. When adjusted for age, gender, and Alzheimer pathology, fluctuating dementia at symptom onset was identified as best predictor of poor outcome.

  1. Screening and Treatment for Depression, Dementia, and Psychosis with Parkinson Disease

    MedlinePlus

    ... Summary for PATIENTS and THEIR FAMILIES SCREENING AND TREATMENT FOR DEPRESSION, DEMENTIA, AND PSYCHOSIS WITH PARKINSON DISEASE Depression, dementia, ... her judgement to determine use of these drugs. Treatment for depression in people with Parkinson disease can be managed ...

  2. Vascular parkinsonism: Deconstructing a syndrome

    PubMed Central

    Vizcarra, Joaquin A.; Lang, Anthony E.; Sethi, Kapil D; Espay, Alberto J.

    2015-01-01

    Progressive ambulatory impairment and abnormal white matter signal on neuroimaging come together under the diagnostic umbrella of vascular parkinsonism. A critical appraisal of the literature, however, suggests that (1) no abnormal structural imaging pattern is specific to vascular parkinsonism; (2) there is poor correlation between brain magnetic resonance imaging hyperintensities and microangiopathic brain disease and parkinsonism from available clinicopathologic data; (3) pure parkinsonism from vascular injury (“definite” vascular parkinsonism) consistently results from ischemic or hemorrhagic strokes involving the substantia nigra and/or nigrostriatal pathway but sparing the striatum itself, the cortex, and the intervening white matter; and (4) many cases reported as vascular parkinsonism may represent pseudovascular parkinsonism (e.g., Parkinson disease or another neurodegenerative parkinsonism such as progressive supranuclear palsy with non-specific neuroimaging signal abnormalities), vascular pseudoparkinsonism (e.g., akinetic mutism due to bilateral mesial frontal strokes or apathetic depression from bilateral striatal lacunar strokes), or pseudovascular pseudoparkinsonism (e.g., higher-level gait disorders, including normal pressure hydrocephalus with transependimal exudate). These syndromic designations are preferable over vascular parkinsonism until pathology or validated biomarkers confirm the underlying nature and relevance of the leukoaraiosis. PMID:25997420

  3. Parkinson's syndrome and Parkinson's disease in mitochondrial disorders.

    PubMed

    Finsterer, Josef

    2011-04-01

    In the majority of cases, mitochondrial disorders are multisystem conditions that most frequently affect the skeletal muscle, followed by the central nervous system. One of the clinical manifestations of central nervous system involvement is Parkinson's syndrome (PS). Evidence for an association of mitochondrial defects with PS comes from mitochondrial disorder patients who have developed Parkinson's syndrome and from Parkinson's syndrome patients who have developed a mitochondrial disorder. In addition, there are a number of patients with Parkinson's syndrome or Parkinson's disease (PD) who later develop subclinical immunohistological or biochemical indications of mitochondrial defects or accumulates mitochondrial DNA mutations within various cerebral regions. There are also Parkinson's syndrome patients who present with elevated cerebrospinal-fluid lactate by magnetic resonance spectroscopy. Furthermore, it has been shown that mutations in genes causing PD, such as PINK1, parkin, DJ1, alpha-synuclein, and LRRK2, also cause mitochondrial dysfunction, which is one of the reasons why they are called mitochondrial nigropathies. Parkinson's syndrome in patients with a mitochondrial disorder may also result from oxidative stress or exogenous toxins. Treatment of mitochondrial Parkinson's syndrome is not at variance with the treatment of Parkinson's syndrome due to other causes, but because of the multisystem nature of mitochondrial disorders, mitochondrial Parkinson's syndrome requires additional therapeutic support.

  4. Clinical characteristics of parkinsonism in frontotemporal dementia according to subtypes.

    PubMed

    Park, Hee Kyung; Park, Kee Hyung; Yoon, Bora; Lee, Jae-Hong; Choi, Seong Hye; Joung, Jee H; Yoon, Soo Jin; Kim, Byeong C; Kim, Seung Hyun; Kim, Eun-Joo; Na, Duk L; Park, Kyung Won

    2017-01-15

    We investigated the prevalence of parkinsonism in frontotemporal dementia (FTD) subtypes and the cognitive and behavioral differences between FTD with and without parkinsonism in a well-structured, prospective cohort. One hundred and ninety-one FTD patients were enrolled and all patients underwent comprehensive neurological evaluations, neuropsychological tests, and the Unified Parkinson's Disease Rating Scale. The prevalence of parkinsonism was 38.7% (74 patients), and included 33 (46.5%) behavioral variant FTD (bvFTD), 16 (24.2%) semantic dementia (SD), 19 (45.2%) progressive nonfluent aphasia (PNFA), and 6 (50%) FTD associated with motor neuron disease (FTD-MND). SD patients with parkinsonism had higher CDR sum of boxes scores (9.7±4.5 vs 6.2±4.5, p=0.024), frontal behavioral inventory total score (33.7±20.5 vs 24.3±14.5, p=0.045), and executive function score of frontal executive dysfunction, disinhibition, and apathy (28.9±13.7 vs 19.2±12.9, p=0.021) than those without parkinsonism. Seoul Instrumental Activities of Daily Living score (bvFTD: 23.5±11.7 vs 17.3±11.3, p=0.031, SD: 23.1±11.1 vs 11.3±9.3, p=0.005) was higher for bvFTD and SD with parkinsonism than for those without parkinsonism. Parkinsonism is found to be more common in patients with bvFTD, PNFA, and FTD-MND patients than those with SD. Behavioral disturbances were more prominent in SD with parkinsonism than without. Additional studies are needed to determine the pathomechanism and optimal treatment of parkinsonism in different FTD subtypes. Copyright © 2016 Elsevier B.V. All rights reserved.

  5. Diogenes Syndrome in Frontotemporal Dementia.

    PubMed

    Finney, Catherine M; Mendez, Mario F

    2017-01-01

    Diogenes syndrome refers to the combination of extreme self-neglect and excessive collecting with clutter and squalor, which is often present in patients with dementia. Diogenes syndrome may be particularly common in behavioral variant frontotemporal dementia (bvFTD), and the investigation of these patients may help clarify the nature of this syndrome. We describe 5 patients with bvFTD who exhibited a decline in self-care accompanied by hoarding behaviors. These patients, and a review of the literature, suggest a combination of frontal lobe disturbances: loss of insight or self-awareness with a failure to clean up or discard, a general compulsive drive, and an innate impulse to take environmental items. This impulse may be part of the environmental dependency syndrome in frontal disease, with specific involvement of a right frontolimbic-striatal system. Further investigation of the similarities and mechanisms of these symptoms in bvFTD could help in understanding Diogenes syndrome and lead to potential treatment options.

  6. Parkinson's Disease Research Web - Information for Patients and Caregivers

    MedlinePlus

    ... Information Page Parkinson's Disease: Hope Through Research NINDS Deep Brain Stimulation for Parkinson's Disease Strategic Plans NINDS ... Syndrome Information Page Dandy-Walker Syndrome Information Page Deep Brain Stimulation for Parkinson's Disease Information Page Dementia ...

  7. Prevalence and treatment pattern of Parkinson's disease dementia in Korea.

    PubMed

    Oh, Yoon-Sang; Kim, Joong-Seok; Park, In-Seok; Shim, Yong-Soo; Song, In-Uk; Park, Jeong-Wook; Lee, Phil-Hyu; Lyoo, Chul-Hyung; Ahn, Tae-Beom; Ma, Hyo-Il; Kim, Yong-Duk; Koh, Seong-Beom; Lee, Seung-Jae; Lee, Kwang-Soo

    2016-02-01

    The aim of the present study was to investigate the point prevalence of dementia and mild cognitive impairment (MCI) in patients with Parkinson's disease (PD). A total of 1200 patients with PD from 12 hospitals were included in the study. All patients were grouped into normal cognition, MCI and dementia subgroups. General cognitive status and dementia severity were assessed by the Korean version of the Mini-Mental State Examination, Clinical Dementia Rating and Global Deterioration Scale, and parkinsonian motor status was assessed by the Hoehn and Yahr staging score. Associated sleep behaviors and other medical conditions were checked. Prescribing patterns of antidementia medications were analyzed. Cognitive impairment was frequent in patients with PD; MCI was found in 38.9% of patients, whereas dementia was in 38.3% of patients. The prevalence of cognitive impairment increased with increasing age and longer disease duration, and the symptoms of postural instability and symptoms mimicking rapid eye movement sleep behavior disorder were associated with cognitive impairment. Many dementia patients (95.2%) and 23.6% of MCI patients were treated with antidementia drugs, with rivastigmine the most frequently used. The point prevalence of cognitive impairment in patients with PD was 77.2%. Cognitive impairment was associated with age, disease duration and specific parkinsonian motor/non-motor symptoms. Over 90% of the patients with dementia were treated with antidementia medication, and rivastigmine was the most frequently used for the management of dementia. © 2015 Japan Geriatrics Society.

  8. Clinical Epidemiology, Evaluation, and Management of Dementia in Parkinson Disease.

    PubMed

    Safarpour, Delaram; Willis, Allison W

    2016-11-01

    The prevalence of neurodegenerative diseases such as Parkinson disease (PD) will increase substantially, due to the aging of the population and improved treatments leading to better disease-related outcomes. Dementia is the most common nonmotor symptom in PD, and most patients with PD will have cognitive dysfunction and cognitive decline in the course of their disease. The development of cognitive dysfunction in PD greatly limits the ability to participate in activities of daily living and can be a tipping point for nursing home placement or major caregiver stress. Understanding the different causes of dementia and how to reduce the incidence and impact of secondary cognitive dysfunction in PD are necessary skills for primary care physicians and neurologists. In this review, we discuss the clinical epidemiology of dementia in PD with an emphasis on preventable cognitive dysfunction, present tools for outpatient evaluation of cognitive dysfunction, and describe current pharmacological treatments for dementia in PD. © The Author(s) 2016.

  9. Imaging amyloid in Parkinson's disease dementia and dementia with Lewy bodies with positron emission tomography.

    PubMed

    Brooks, David J

    2009-01-01

    Although Parkinson's disease with later dementia (PDD) and dementia with Lewy bodies (DLB) are pathologically characterized by the presence of intraneuronal Lewy inclusion bodies, amyloid deposition is also associated to varying degrees with both these disorders. Fibrillar amyloid load can now be quantitated in vivo with positron emission tomography (PET) using imaging biomarkers. Here the reported findings of 11C-PIB PET studies concerning the amyloid load associated with PD and its influence on dementia are reviewed. It is concluded that the presence of amyloid acts to accelerate the dementia process in Lewy body disorders, though has little influence on its nature. Anti-amyloid strategies could be a relevant approach for slowing dementia in a number of DLB and PDD cases.

  10. Visual symptoms in Parkinson's disease and Parkinson's disease dementia.

    PubMed

    Archibald, Neil K; Clarke, Mike P; Mosimann, Urs P; Burn, David J

    2011-11-01

    Visual symptoms are common in PD and PD dementia and include difficulty reading, double vision, illusions, feelings of presence and passage, and complex visual hallucinations. Despite the established prognostic implications of complex visual hallucinations, the interaction between cognitive decline, visual impairment, and other visual symptoms remains poorly understood. Our aim was to characterize the spectrum of visual symptomatology in PD and examine clinical predictors for their occurrence. Sixty-four subjects with PD, 26 with PD dementia, and 32 age-matched controls were assessed for visual symptoms, cognitive impairment, and ocular pathology. Complex visual hallucinations were common in PD (17%) and PD dementia (89%). Dementia subjects reported illusions (65%) and presence (62%) more frequently than PD or control subjects, but the frequency of passage hallucinations in PD and PD dementia groups was equivalent (48% versus 69%, respectively; P = 0.102). Visual acuity and contrast sensitivity was impaired in parkinsonian subjects, with disease severity and age emerging as the key predictors. Regression analysis identified a variety of factors independently predictive of complex visual hallucinations (e.g., dementia, visual acuity, and depression), illusions (e.g., excessive daytime somnolence and disease severity), and presence (e.g., rapid eye movement sleep behavior disorder and excessive daytime somnolence). Our results demonstrate that different "hallucinatory" experiences in PD do not necessarily share common disease predictors and may, therefore, be driven by different pathophysiological mechanisms. If confirmed, such a finding will have important implications for future studies of visual symptoms and cognitive decline in PD and PD dementia. Copyright © 2011 Movement Disorder Society.

  11. Cognitive Impairment and Dementia in Patients with Parkinson Disease

    PubMed Central

    Leverenz, James B.; Quinn, Joseph F.; Zabetian, Cyrus; Zhang, Jing; Montine, Kathleen S.; Montine, Thomas J.

    2009-01-01

    Parkinson disease (PD) is an already prevalent neurodegenerative disease that is poised to at least double over the next 25 years. Although best known for its characteristic movement disorder, PD is now appreciated commonly to cause cognitive impairment, including dementia, and behavioral changes. Dementia in patients with PD is consequential and has been associated with reduced quality of life, shortened survival, and increased caregiver distress. Here we review clinical presentation and progression, pathological bases, identification of genetic risk factors, development of small molecule biomarkers, and emerging treatments for cognitive impairment in patients with PD. PMID:19754405

  12. Genetic factors influencing frontostriatal dysfunction and the development of dementia in Parkinson's disease

    PubMed Central

    Huertas, Ismael; Jesús, Silvia; García-Gómez, Francisco Javier; Lojo, José Antonio; Bernal-Bernal, Inmaculada; Bonilla-Toribio, Marta; Martín-Rodriguez, Juan Francisco; García-Solís, David; Gómez-Garre, Pilar; Mir, Pablo

    2017-01-01

    The dual syndrome hypothesis for cognitive impairment in Parkinson's disease (PD) establishes a dichotomy between a frontrostriatal dopamine-mediated syndrome, which leads to executive deficits, and a posterior cortical syndrome, which leads to dementia. Certain genes have been linked to these syndromes although the exact contribution is still controversial. The study’s objective was to investigate the role of APOE, MAPT, COMT, SNCA and GBA genes in the dual syndromes. We genotyped APOE (rs429358 and rs7412), MAPT (rs9468), COMT (rs4680) and SNCA (rs356219) risk polymorphisms and sequenced GBA in a cohort of 298 PD patients. The degree of dopaminergic depletion was investigated with [123I]FP-CIT SPECTs and the presence of dementia was ascertained with a long-term review based on established criteria. The association between genetic and imaging parameters was studied with linear regression, and the relationship with dementia onset with Cox regression. We found that APOE2 allele (Pput = 0.002; Pcau = 0.01), the minor allele 'G' in SNCA polymorphism (Pput = 0.02; Pcau = 0.006) and GBA deleterious variants in (Pput = 0.01; Pcau = 0.001) had a detrimental effect on striatal [123I]FP-CIT uptake in PD. Conversely, Met/Met carriers in COMT polymorphism had increased caudate uptake (Pcau = 0.03). The development of dementia was influenced by APOE4 allele (HR = 1.90; P = 0.03) and GBA deleterious variants (HR = 2.44; P = 0.01). Finally, we observed no role of MAPT locus in any of the syndromes. As a conclusion, APOE2, SNCA, COMT and GBA influence frontostriatal dysfunction whereas APOE4 and GBA influence the development of dementia, suggesting a double-edged role of GBA. The dichotomy of the dual syndromes may be driven by a broad dichotomy in these genetic factors. PMID:28399184

  13. ALS-parkinsonism-dementia complex of Kii and other related diseases in Japan.

    PubMed

    Kaji, Ryuji; Izumi, Yishin; Adachi, Yoshiki; Kuzuhara, Shigeki

    2012-01-01

    The ALS/parkinsonism-dementia complex (PDC) of Kii is an endemic disease with a diverse phenotypic expression characteristic of classical ALS, parkinsonism and dementia. Its clinical and neuropathological manifestations are similar to a syndrome found in Guam, sharing classical ALS pathology together with many neurofibrillary tangles in the brain. The incidence rates of ALS declined dramatically between the 1950s and 1980s. In the 1990 s, Kuzuhara found a high incidence of PDC with abundant neurofibrillary tangles, similar to Guamanian PDC. The incidence rates of PDC dramatically rose during the 1980s and 1990 s, and PDC replaced ALS. More than 70% of patients in the endemic region had a family history of ALS or PDC. We recently found a new gene OPTN causing ALS, and have extended its clinical survey in Japan. Two autopsied cases showed involvement of basal ganglia and/or cerebral cortex with neurofibrillary tangles. A few family members also showed dementia and parkinsonism without evidence of motor neuron disease. Moreover the penetrance seems to be incomplete. Despite these similarities, OPTN mutations were not found in the Kii patients. We speculate that the Kii/ALS-PDC could primarily be a genetic disease, and its clinical manifestation is modified by other genes or environmental factors.

  14. Dementia in Parkinson's disease: Is male gender a risk factor?

    PubMed

    Cereda, Emanuele; Cilia, Roberto; Klersy, Catherine; Siri, Chiara; Pozzi, Beatrice; Reali, Elisa; Colombo, Aurora; Zecchinelli, Anna Lena; Mariani, Claudio Bruno; Tesei, Silvana; Canesi, Margherita; Sacilotto, Giorgio; Meucci, Nicoletta; Zini, Michela; Isaias, Ioannis Ugo; Barichella, Michela; Cassani, Erica; Goldwurm, Stefano; Pezzoli, Gianni

    2016-05-01

    The rates of cognitive decline in patients with Parkinson's disease (PD) are higher than in the general population. Age and disease duration have been associated with increasing rates of dementia in PD. However, the role of other factors including gender has been poorly investigated. We investigated the relationship between dementia and gender along with other established risk factors, such as age and disease duration. We conducted a cross-sectional retrospective study including all consecutive patients diagnosed with idiopathic PD attending a single out-patient tertiary clinic over an 18-year period (1995-2013). Dementia was diagnosed according to DSM-IV criteria. Prevalence of dementia was 11.5% (95%CI, 10.8-12.3) and 13.5% (95%CI, 12.7-14.5) in the whole population (N = 6599) and in those aged ≥60 years (N = 5373), respectively. Age and disease duration were independently associated with dementia, and the latter was associated with dementia up to 84 years of age. Male gender was an independent risk factor. In addition, while the rate of dementia increased in males over all age strata, we found that in females prevalence began to increase steadily after the age of 65 years, reaching male estimates only after 80 years of age. Higher rates in male gender were observed between 60 and 80 years of age. Age and PD duration are confirmed risk factors for dementia. However, disease duration appeared to be a less important factor in cognitive decline in patients aged ≥85 years. As opposed to gender-specific estimates in the general population, male gender is likely associated with higher rates of dementia in PD patients. Copyright © 2016 Elsevier Ltd. All rights reserved.

  15. [Dementia and mild cognitive impairment in Parkinson's disease: a review].

    PubMed

    Bocanegra, Yamile; Trujillo-Orrego, Natalia; Pineda, David

    2014-12-16

    INTRODUCTION. The cognitive disorders in Parkinson's disease (PD) have traditionally been associated with the presence of dementia in later stages of the disease. Recent studies, however, consider that cognitive impairment can appear as of early stages. Knowing the cognitive profile of PD furthers our understanding of the clinical phenotype, making it easier to reach a timely diagnosis and favouring intervention on the symptoms from the initial stages. AIM. To present a review of the literature on mild cognitive impairment (MCI) and dementia associated with PD. DEVELOPMENT. Several studies report that patients with PD who have a prolonged time to progression develop dementia. Yet, there have also been reports claiming that, as of the early stages, patients can present subtle cognitive alterations known as MCI. The initial neuropsychological profile is mainly of a non-amnesic type, characterised by executive dysfunction, alterations affecting attention, operative memory deficit and faulty retrieval of information. When patients develop dementia, disorders will arise in the storage of information, in semantic fluency, and in visuospatial and visuoperceptual skills. Currently there are criteria available for diagnosing the MCI and dementia associated with PD, as well as valid reliable instruments for detecting those disorders. CONCLUSIONS. Cognitive symptoms are frequent in PD. From the initial stages of the disease onwards patients may present MCI that is mainly characterised by a fronto-subcortical cognitive profile, whereas dementia usually develops at later stages, when a pattern of posterior cortical cognitive disorder is also observed.

  16. Diogenes syndrome in patients suffering from dementia

    PubMed Central

    Cipriani, Gabriele; Lucetti, Claudio; Vedovello, Marcella; Nuti, Angelo

    2012-01-01

    Diogenes syndrome (DS) is a behavioral disorder of the elderly. Symptoms include living in extreme squalor, a neglected physical state, and unhygienic conditions. This is accompanied by a self-imposed isolation, the refusal of external help, and a tendency to accumulate unusual objects. To explore the phenomenon of DS in dementia we searched for the terms: “Diogenes syndrome, self-neglect, dementia. ” It has long been understood that individuals with dementia often become shut-ins, living in squalor, in the Eastern Baltimore study, dementia was present in 15% of the elderly cases with moderate and severe social breakdown syndrome; twice as many as in the general population of the same age group. Researchers have underlined the frequent presence of DS (36%) in frontotemporal dementia (FTD): different neuropsychological modifications in FTD may contribute to symptoms of DS. The initial treatment should be a behavioral program, but there is not sufficient information regarding pharmacological treatment of the syndrome. PMID:23393422

  17. Diogenes syndrome in patients suffering from dementia.

    PubMed

    Cipriani, Gabriele; Lucetti, Claudio; Vedovello, Marcella; Nuti, Angelo

    2012-12-01

    Diogenes syndrome (DS) is a behavioral disorder of the elderly. Symptoms include living in extreme squalor, a neglected physical state, and unhygienic conditions. This is accompanied by a self-imposed isolation, the refusal of external help, and a tendency to accumulate unusual objects. To explore the phenomenon of DS in dementia we searched for the terms: "Diogenes syndrome, self-neglect, dementia. " It has long been understood that individuals with dementia often become shut-ins, living in squalor, in the Eastern Baltimore study, dementia was present in 15% of the elderly cases with moderate and severe social breakdown syndrome; twice as many as in the general population of the same age group. Researchers have underlined the frequent presence of DS (36%) in frontotemporal dementia (FTD): different neuropsychological modifications in FTD may contribute to symptoms of DS. The initial treatment should be a behavioral program, but there is not sufficient information regarding pharmacological treatment of the syndrome.

  18. CSF amyloid-β-peptides in Alzheimer's disease, dementia with Lewy bodies and Parkinson's disease dementia

    PubMed Central

    Mollenhauer, Brit; Esselmann, Hermann; Lewczuk, Piotr; Klafki, Hans-Wolfgang; Sparbier, Katrin; Smirnov, Alexandr; Cepek, Lukas; Trenkwalder, Claudia; Rüther, Eckart; Kornhuber, Johannes; Otto, Markus; Wiltfang, Jens

    2006-01-01

    Abstract As the differential diagnosis of dementias based on established clinical criteria is often difficult, biomarkers for applicable diagnostic testing are currently under intensive investigation. Amyloid plaques deposited in the brain of patients suffering from Alzheimer's disease, dementia with Lewy bodies (DLB) and Parkinson's disease dementia (PDD) mainly consist of carboxy-terminally elongated forms of amyloid-beta (Aβ) peptides, such as Aβ1–42. Absolute Aβ1–42 levels in CSF have shown diagnostic value for the diagnosis of Alzheimer's disease, but the discrimination among Alzheimer's disease, DLB and PDD was poor. A recently established quantitative urea-based Aβ-sodium-dodecylsulphate–polyacrylamide-gel-electrophoresis with Western immunoblot (Aβ-SDS–PAGE/immunoblot) revealed a highly conserved Aβ peptide pattern of the carboxy-terminally truncated Aβ peptides 1–37, 1–38, 1–39 in addition to 1–40 and 1–42 in human CSF. We used the Aβ-SDS–PAGE/immunoblot to investigate the CSF of 23 patients with Alzheimer's disease, 21 with DLB, 21 with PDD and 23 non-demented disease controls (NDC) for disease-specific alterations of the Aβ peptide patterns in its absolute and relative quantities. The diagnostic groups were matched for age and severity of dementia. The present study is the first attempt to evaluate the meaning of Aβ peptide patterns in CSF for differential diagnosis of the three neurodegenerative diseases—Alzheimer's disease, DLB and PDD. The Aβ peptide patterns displayed disease-specific variations and the ratio of the differentially altered Aβ1–42 to the Aβ1–37 levels subsequently discriminated all diagnostic groups from each other at a highly significant level, except DLB from PDD. Additionally, a novel peptide with Aβ-like immunoreactivity was observed constantly in the CSF of all 88 investigated patients. The pronounced percentage increase of this peptide in DLB allowed a highly significant discrimination from

  19. Ventricular dilatation and brain atrophy in patients with Parkinson's disease with incipient dementia.

    PubMed

    Camicioli, Richard; Sabino, Jennifer; Gee, Myrlene; Bouchard, Thomas; Fisher, Nancy; Hanstock, Chris; Emery, Derek; Martin, W R Wayne

    2011-07-01

    Age-related ventricular enlargement is accelerated in Alzheimer's disease, but its relationship to cognitive decline in Parkinson's disease is less clear, even though dementia is common in Parkinson's disease. Our goals were to determine if greater enlargement of the ventricles and gray or white matter atrophy occurred in Parkinson's disease patients developing cognitive decline. Older nondemented patients with Parkinson's disease (33) and age- and sex-matched controls (39) were recruited and prospectively assessed for the development of significant cognitive decline over 36 months. Magnetic resonance imaging was obtained every 18 months, and ventricular volume and total brain gray and white matter volumes were measured using reliable segmentation of T1-weighted volumetric scans. Subjects with incidental intracranial abnormalities, an atypical course, and stroke as well as dropouts were excluded from a cohort of 52 patients and 50 controls. Among 33 patients and 39 controls, 10 patients and 3 controls developed significant cognitive impairment or dementia. Ventricular change and Parkinson's disease status were significantly associated with dementia. Ventricular change was significantly correlated with change in Mini-Mental Status Examination in the Parkinson's disease with dementia group (r = 0.87, P = .001). Gray matter atrophy was greater in Parkinson's disease with dementia, with similar change over time in both Parkinson's disease and Parkinson's disease with dementia. White matter volumes were not significantly different between Parkinson's disease and Parkinson's disease with dementia; however, the decrease over time might be greater in Parkinson's disease with dementia. Ventricular dilatation occurs early in the course of significant cognitive decline in patients with Parkinson's disease, possibly reflecting both cortical gray and white matter loss.

  20. Treatment of psychosis and dementia in Parkinson's disease.

    PubMed

    Goldman, Jennifer G; Holden, Samantha

    2014-03-01

    Parkinson's disease (PD) has been increasingly recognized as having a multitude of nonmotor symptoms including psychosis, cognitive impairment and dementia, mood disturbances, fatigue, apathy, and sleep disorders. Psychosis and dementia, in particular, greatly affect quality of life for both patients and caregivers and are associated with poor outcomes. Safe and effective treatment options for psychosis and dementia in PD are much needed. Antipsychotics with dopamine-blocking properties can worsen parkinsonian motor features and have been associated with increased morbidity and mortality in elderly, dementia patients. For treating PD psychosis, a first step would be eliminating confounding variables, such as delirium, infections, or toxic-metabolic imbalances, followed by simplifying parkinsonian medications as tolerated. If additional treatment is warranted after such interventions, clozapine or quetiapine can be implemented at the low dose levels typically needed by PD patients. Although quetiapine is easy-to-use in clinical settings, does not require blood count monitoring like clozapine, and is anecdotally beneficial, it remains "investigational" in evidence-based medicine reviews. Though not currently available, the novel 5-HT2a inverse agonist, pimavanserin has shown promise in the treatment of PD psychosis. Current treatments for PD dementia are mostly derived from those utilized in Alzheimer's disease, focusing mainly on cholinesterase inhibitors and memantine, a NMDA receptor antagonist. Rivastigmine, the only Food and Drug Administration approved medication for PD dementia, is a reasonable first choice. Other cholinesterase inhibitors and memantine have not yet achieved recommendation status in evidence-based medicine reviews but are well tolerated in studies of PD dementia patients. At present, there are no approved treatments for mild cognitive impairment in PD, but rasagiline, a selective MAO-B inhibitor, and atomoxetine, a serotonin norepinephrine

  1. 'Hummingbird' Sign in a Patient with Guam Parkinsonism-Dementia Complex.

    PubMed

    Yeo, Tianrong; Tan, Louis Cs

    2017-09-01

    We present a case of a 71-year-old male Chamorro patient from Guam who presented with progressive supranuclear palsy (PSP)-Richardson's syndrome. Considering his strong family history of parkinsonism and a PSP phenotype, he was clinically diagnosed with Guam parkinsonism-dementia complex (PDC). Magnetic resonance imaging (MRI) of the brain revealed prominent midbrain atrophy with preserved pontine volume, forming the 'hummingbird' sign, which has not been described before in Guam PDC. Molecular analysis of the chromosome 9 open reading frame 72 gene (C9orf72) showed only 6 GGGGCC repeats. We discuss the clinico-pathological similarities and differences between PSP and Guam PDC, and highlight the topography of neuropathological changes seen in Guam PDC to explain the appearance of the 'hummingbird' sign on MRI.

  2. Importance of 123I-metaiodobenzylguanidine scintigraphy/single photon emission computed tomography for diagnosis and differential diagnostics of Parkinson syndromes.

    PubMed

    Jost, Wolfgang H; Del Tredici, Kelly; Landvogt, Christian; Braune, Stefan

    2010-01-01

    The goal of Parkinson syndrome diagnostics is twofold: early diagnosis on the one hand, and accurate differentiation among idiopathic and atypical Parkinson syndromes on the other. (123)I-metaiodobenzylguanidine scintigraphy is the only method that can distinguish with a high degree of sensitivity and specificity between atypical Parkinson syndromes and Parkinson's disease or dementia with Lewy bodies. Additional advantages are the method's widespread availability and radioactive exposure dose comparable to that for single photon emission computed tomography imaging with much lower costs. Only a single radiotracer study is necessary. (123)I-metaiodobenzylguanidine scintigraphy is an indispensable tool for purposes of differentiating among the various Parkinson syndromes.

  3. A unique retinal epitheliopathy is associated with amyotrophic lateral sclerosis/Parkinsonism-Dementia complex of Guam.

    PubMed

    Steele, John C; Wresch, Robert; Hanlon, Samuel D; Keystone, Jay; Ben-Shlomo, Yoav

    2015-08-01

    The aim of this work was to examine whether a linear retinal pigment epitheliopathy is associated with the amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam. A total of 918 Guamanian Chamorros, with and without amyotrophic lateral sclerosis/parkinsonism-dementia complex, were examined cross-sectionally for linear retinal pigment epitheliopathy (LRPE). Overall, 239 Guamanians, who were neurologically asymptomatic, were followed for up to 20 years to determine the risk of developing amyotrophic lateral sclerosis/parkinsonism-dementia complex. The epitheliopathy was present in 59.7% (117 of 196) patients with amyotrophic lateral sclerosis/parkinsonism-dementia complex, but in only 24.7% (178 of 722) of subjects who were neurologically asymptomatic (age- and sex-adjusted risk difference: 35.0%; 95% confidence interval [CI]: 27.5-42.6; p < 0.0001). Prospectively, 15 of 50 cases with epitheliopathy developed amyotrophic lateral sclerosis/parkinsonism-dementia complex, compared to 4 of 189 cases without epitheliopathy (age- and sex-adjusted hazard ratio: 13.1; 95% CI: 4.0-43.1; P < 0.0001). Amyotrophic lateral sclerosis/parkinsonism-dementia complex is associated with an LRPE and predicts future neurological disease. Identifying the cause of this retinopathy could provide an understanding about the pathogenesis of amyotrophic lateral sclerosis/parkinsonism-dementia complex and related diseases. © 2015 International Parkinson and Movement Disorder Society.

  4. Motor and cognitive function in Lewy body dementia: comparison with Alzheimer's and Parkinson's diseases.

    PubMed Central

    Gnanalingham, K K; Byrne, E J; Thornton, A; Sambrook, M A; Bannister, P

    1997-01-01

    OBJECTIVE: Motor and cognitive function were compared in patients with Lewy body dementia, Parkinson's disease, or Alzheimer's disease, to identify features that may be clinically useful in differentiating Lewy body dementia from Alzheimer's disease and Parkinson's disease. METHODS: A range of neuropsychological function and extrapyrimidal signs (EPS) was assessed in 16 patients with Lewy body dementia, 15 with Parkinson's disease, 25 with Alzheimer's disease, and 22 control subjects. RESULTS: The severity of total motor disability scores increased in the following order: controls approximately = Alzheimer's disease << Parkinson's disease < Lewy body dementia. Compared with patients with Parkinson's disease, patients with Lewy body dementia had greater scores for rigidity and deficits in the finger tapping test, but rest tremor and left/right asymmetry in EPS were more evident in Parkinson's disease. Patients with Lewy body dementia were also less likely to present with left/right asymmetry in EPS at the onset of their parkinsonism. "Sensitivity" to neuroleptic drugs was noted in 33% of patients with Lewy body dementia. Alzheimer's disease and Lewy body dementia groups had greater severity of dementia compared with the Parkinson's disease group and controls. Neuropsychological evaluation disclosed severe but similar degrees of impaired performances in tests of attention (digit span), frontal lobe function (verbal fluency, category, and Nelson card sort test) and motor sequencing in both Lewy body dementia and Alzheimer's disease groups, than Parkinson's disease and controls. In the clock face test, improved performance was noted in the "copy" compared to "draw" part of the test in controls, patients with Alzheimer's disease, and those with Parkinson's disease, but not in the patients with Lewy body dementia, who achieved equally poor scores in both parts of the test. CONCLUSIONS: EPS in Lewy body dementia resemble those seen in idiopathic Parkinson's disease

  5. Predicting dementia development in Parkinson's disease using Bayesian network classifiers.

    PubMed

    Morales, Dinora A; Vives-Gilabert, Yolanda; Gómez-Ansón, Beatriz; Bengoetxea, Endika; Larrañaga, Pedro; Bielza, Concha; Pagonabarraga, Javier; Kulisevsky, Jaime; Corcuera-Solano, Idoia; Delfino, Manuel

    2013-08-30

    Parkinson's disease (PD) has broadly been associated with mild cognitive impairment (PDMCI) and dementia (PDD). Researchers have studied surrogate, neuroanatomic biomarkers provided by magnetic resonance imaging (MRI) that may help in the early diagnosis of this condition. In this article, four classification models (naïve Bayes, multivariate filter-based naïve Bayes, filter selective naïve Bayes and support vector machines, SVM) have been applied to evaluate their capacity to discriminate between cognitively intact patients with Parkinson's disease (PDCI), PDMCI and PDD. For this purpose, the MRI studies of 45 subjects (16 PDCI, 15 PDMCI and 14 PDD) were acquired and post-processed with Freesurfer, obtaining 112 variables (volumes of subcortical structures and thickness of cortical parcels) per subject. A multivariate filter-based naïve Bayes model was found to be the best classifier, having the highest cross-validated sensitivity, specificity and accuracy. Additionally, the most relevant variables related to dementia in PD, as predicted by our classifiers, were cerebral white matter, and volumes of the lateral ventricles and hippocampi.

  6. Neuropathological aspects of Alzheimer disease, Parkinson disease and frontotemporal dementia.

    PubMed

    Jellinger, Kurt A

    2008-01-01

    Proteinopathies are a heterogenous group of neurodegenerative disorders, characterized by intra- and extracellular accumulation of abnormal filament proteins. To describe the neuropathology of specific forms of tauopathies and synucleinopathies, the overlap of morphologic features and molecular interactions. The study uses currently available morphologic criteria of different proteinopathies. Alzheimer disease (AD) is featured by deposition of beta-amyloid peptides, phosphorylated tau protein (3- and 4-repeat tau) and frequent alpha-synuclein (aSyn) deposits. Lewy body diseases (LBD), such as sporadic Parkinson disease (PD) and dementia with Lewy bodies (DLB), show aSyn-positive deposits in neurons, neurites, glia, and presynaptic terminals, while frontotemporal dementias present tau-positive and tau-negative, ubiquitin- and TDP-43-positive neuronal and glial inclusions. The latter have also been observed in AD, PD, PD dementia and motor neuron disorders. Molecular interactions between major proteins, which may occur within the same brain in various distribution patterns, cause variable phenotypes and mixed pathologies, e.g. AD with aSyn pathology in the brainstem and amygdala, PD and DLB with AD lesions, and frontotemporal dementia with a mixture of various deposits, while others are featured by one principal pathology without other lesions (e.g. tangle-predominant type of dementia, pure PD, brainstem-predominant LBD). Animal models and in vitro studies showing co-occurrence and mutual promotion of fibrillation of these proteins indicate their synergistic interactions in the pathogenesis of these disorders which, at least in part, are genetically influenced. 2008 S. Karger AG, Basel

  7. Quality Care for Down Syndrome and Dementia

    ERIC Educational Resources Information Center

    Tedder, Amanda

    2012-01-01

    This article will give both examples and methods to use when providing services to individuals with a dual diagnosis of Down syndrome and Dementia. This is a prevalent issue that most care facilities are facing as the population with Down syndrome age. Staff training, schedule adjustments, living space adjustments and a new thought process…

  8. Cognitive impairment in 873 patients with idiopathic Parkinson's disease. Results from the German Study on Epidemiology of Parkinson's Disease with Dementia (GEPAD).

    PubMed

    Riedel, Oliver; Klotsche, Jens; Spottke, Annika; Deuschl, Günther; Förstl, Hans; Henn, Fritz; Heuser, Isabella; Oertel, Wolfgang; Reichmann, Heinz; Riederer, Peter; Trenkwalder, Claudia; Dodel, Richard; Wittchen, Hans-Ulrich

    2008-02-01

    Parkinson's disease (PD) is often accompanied by non-motor complications, such as dementia, depression, and psychotic symptoms, which worsen the prognosis and increase the personal and socioeconomic burden of disease. Prevalence estimates of these complications are quite variable and are lacking for the outpatient care sector. As part of a larger, nationwide, cross-sectional epidemiological study in n = 315 neurological outpatient settings in Germany, this paper estimates the frequency of dementia and cognitive impairment in n = 873 outpatients meeting the UK Brain Bank criteria for idiopathic PD. Assessments were based on a clinical interview and neuropsychological assessments, including the Hoehn & Yahr rating and Unified Parkinson's Disease Rating Scale (UPDRS). Cognitive impairment was assessed by the Mini-Mental State Exam (MMSE), Clock Drawing Test (CDT) and the Parkinson Neuropsychometric Dementia Assessment (PANDA) and the clinician's diagnosis of dementia was based on the diagnostic criteria of DSMIV. Using standardized cutoff scores, the prevalence of cognitive impairment in the study sample as measured by various methods was 17.5% by MMSE (< or = 24), 41.8% by CDT (> or = 3), 43.6% by PANDA (< or = 14), and 28.6% met the DSM-IV criteria for dementia. All estimates increased with age and PD severity. Gender was an inconsistent contributor while illness duration had no significant impact on cognition. Multiple regression analyses revealed PD severity to be the strongest predictor of dementia risk (OR = 4.3; 95% CI: 2.1-9.1), while neuropsychiatric syndromes had independent, although modest additional contributions (OR = 2.5, 95% CI: 1.6-3.8). Estimates of cognitive impairment and dementia in PD patients are largely dependent on the diagnostic measure used. Using established clinical diagnostic standards for dementia the overall rate on routine outpatient neurological care is 28.6%, but using more sensitive neuropsychological measures, rates for cognitive

  9. Visual recognition memory differentiates dementia with Lewy bodies and Parkinson's disease dementia.

    PubMed

    Mondon, K; Gochard, A; Marqué, A; Armand, A; Beauchamp, D; Prunier, C; Jacobi, D; de Toffol, B; Autret, A; Camus, V; Hommet, C

    2007-07-01

    To compare cognitive impairments in dementia with Lewy bodies (DLB) and Parkinson's disease dementia (PDD), to discriminate between the two entities. 10 DLB and 12 PDD consecutive patients performed a neuropsychological battery designed to assess several cognitive domains: verbal and visual memory (Delayed Matching to Sample (DMS)-48), language, gnosia, praxia and executive functions. DLB patients had poorer performances in orientation (p<0.05), Trail Making Test A (p<0.05) and reading of names of colours in the Stroop Test (p<0.05). Their scores were also lower in the visual object recognition memory test (DMS-48), in both immediate (p<0.05) and delayed recognition (p<0.05). No differences were observed in the other tests. Despite global similarities in cognitive performances between DLB and PDD patients, we observed important differences: in particular, DMS-48, a test of visual object recognition memory and visual storage capacity, was poorer in DLB patients.

  10. [Neuropsychiatric symptoms in dementia syndrome].

    PubMed

    Artaso Irigoyen, B; Goñi Sarriés, A; Gómez Martínez, A R

    The aim of this study was to describe the neuropsychiatric disorders that present in dementia and the differences they show at each stage as the disease progresses. The study involved a total of 175 patients from a psychogeriatric clinic who had been diagnosed as suffering from dementia at distinct stages of the disease: 66 had mild dementia, 56 were with moderate dementia and 53 were suffering from severe dementia. The following instruments were used to collect both socio demographic and clinical data: the Spanish version of the Mini Mental State Examination (miniexamen cognitivo: MEC) for cognitive impairment, the Barthel index for functional deterioration and the neuropsychiatric inventory (NPI) for the non cognitive symptoms. There were no significant differences in the NPI according to the degree of cognitive impairment and the most frequently seen symptoms were anomalous motor activity, apathy and irritability; the neuropsychiatric disorder that was least often present was euphoria. The presence of disinhibition, irritability, depression, hallucinations and anomalous motor activity varied significantly in the different phases of dementia. Thus, disinhibition, irritability and depression were more frequent in the initial stages of the disease whereas hallucinations and anomalous motor activity were seen more often when the cognitive impairment was severe. Neuropsychiatric disorders appear throughout the whole course of dementia and symptoms vary according to the stage of the disease.

  11. Everyday Cognitive Failures and Memory Problems in Parkinson's Patients without Dementia

    ERIC Educational Resources Information Center

    Poliakoff, Ellen; Smith-Spark, James H.

    2008-01-01

    There is growing evidence that Parkinson's disease patients without dementia exhibit cognitive deficits in some executive, memory and selective attention tasks. However, the impact of these deficits on their everyday cognitive functioning remains largely unknown. This issue was explored using self-report questionnaires. Twenty-four Parkinson's…

  12. Everyday Cognitive Failures and Memory Problems in Parkinson's Patients without Dementia

    ERIC Educational Resources Information Center

    Poliakoff, Ellen; Smith-Spark, James H.

    2008-01-01

    There is growing evidence that Parkinson's disease patients without dementia exhibit cognitive deficits in some executive, memory and selective attention tasks. However, the impact of these deficits on their everyday cognitive functioning remains largely unknown. This issue was explored using self-report questionnaires. Twenty-four Parkinson's…

  13. Electroencephalographic prodromal markers of dementia across conscious states in Parkinson's disease.

    PubMed

    Latreille, Véronique; Carrier, Julie; Gaudet-Fex, Benjamin; Rodrigues-Brazète, Jessica; Panisset, Michel; Chouinard, Sylvain; Postuma, Ronald B; Gagnon, Jean-François

    2016-04-01

    In Parkinson's disease, electroencephalographic abnormalities during wakefulness and non-rapid eye movement sleep (spindles) were found to be predictive biomarkers of dementia. Because rapid eye movement sleep is regulated by the cholinergic system, which shows early degeneration in Parkinson's disease with cognitive impairment, anomalies during this sleep stage might mirror dementia development. In this prospective study, we examined baseline electroencephalographic absolute spectral power across three states of consciousness (non-rapid eye movement sleep, rapid eye movement sleep, and wakefulness) in 68 non-demented patients with Parkinson's disease and 44 healthy controls. All participants underwent baseline polysomnographic recordings and a comprehensive neuropsychological assessment. Power spectral analyses were performed on standard frequency bands. Dominant occipital frequency during wakefulness and ratios of slow-to-fast frequencies during rapid eye movement sleep and wakefulness were also computed. At follow-up (an average 4.5 years after baseline), 18 patients with Parkinson's disease had developed dementia and 50 patients remained dementia-free. In rapid eye movement sleep, patients with Parkinson's disease who later developed dementia showed, at baseline, higher absolute power in delta and theta bands and a higher slowing ratio, especially in temporal, parietal, and occipital regions, compared to patients who remained dementia-free and controls. In non-rapid eye movement sleep, lower baseline sigma power in parietal cortical regions also predicted development of dementia. During wakefulness, patients with Parkinson's disease who later developed dementia showed lower dominant occipital frequency as well as higher delta and slowing ratio compared to patients who remained dementia-free and controls. At baseline, higher slowing ratios in temporo-occipital regions during rapid eye movement sleep were associated with poor performance on visuospatial tests in

  14. Episodic Memory in Alzheimer Disease, Frontotemporal Dementia, and Dementia With Lewy Bodies/Parkinson Disease Dementia: Disentangling Retrieval From Consolidation.

    PubMed

    Economou, Alexandra; Routsis, Christopher; Papageorgiou, Sokratis G

    2016-01-01

    Differences in episodic memory performance in patients with Alzheimer disease (AD), frontotemporal dementia (FTD), dementia with Lewy bodies (DLB)/Parkinson disease with dementia (PDD) are inconsistent and task dependent. The inconsistencies may be attributed to the different tasks drawing on different memory processes. Few studies have examined episodic memory impairment in the above groups using memory tests that facilitate encoding, to distinguish memory deficits due to impairment of specific processes. We examined the memory performance of 106 AD patients, 51 FTD patients, 26 DLB/PDD patients, and 37 controls using the Five-Words Test, a 5-item memory test that facilitates encoding. The patient groups did not differ in modified Mini Mental State Examination scores. AD patients scored lowest on the Five-Words Test overall, and showed the greatest reduction from immediate total recall to delayed free recall relative to the other 2 groups, consistent with a predominantly consolidation deficit. DLB/PDD patients showed the largest improvement from delayed free to delayed total recall relative to the other 2 groups, consistent with a predominantly retrieval deficit. Deficits in both consolidation and retrieval underlie the memory impairment of the patients, to different extents, and contribute to the theoretical understanding of the nature of the memory impairment of the patient groups.

  15. Cognitive screening in Parkinson's disease: Comparison of the Parkinson Neuropsychometric Dementia Assessment (PANDA) with 3 other short scales.

    PubMed

    Gasser, A-I; Calabrese, P; Kalbe, E; Kessler, J; Rossier, P

    2016-02-01

    Cognitive screening is crucial in Parkinson's disease (PD). However, there is still a lack of short tools in French. In this study, we aimed to compare the Parkinson Neuropsychometric Dementia Assessment (PANDA) with the Mini Mental Parkinson (MMP), the Mini Mental State Examination (MMSE) and the Clock Test in French-speaking patients. We also aimed to propose cut-off scores for cognitive impairment and dementia for the French language version of the PANDA. Fifty-one patients with PD took the PANDA, the MMSE, the MMP, and the Clock Test. They also underwent extensive neuropsychological testing by a neuropsychologist who was blinded to the above-mentioned screening test results. Patients were classified as either having normal cognition (n=15), mild cognitive impairment (n=20) or dementia (n=16). When compared with the three other screening tools, the PANDA exhibited the highest area under the curve (AUC) for both cognitive disorders and dementia. Using the cut-off scores proposed for the German version, the PANDA had 94% specificity and 100% sensitivity for dementia and 100% and 72%, respectively for cognitive disorders. In our study, the PANDA exhibited a higher discriminative power than the three other tests in detecting cognitive disorders and dementia. In PD patients, the PANDA should thus be considered for the detection of cognitive impairment in routine clinical practice. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  16. Depression and synaptic zinc regulation in Alzheimer disease, dementia with lewy bodies, and Parkinson disease dementia.

    PubMed

    Whitfield, David R; Vallortigara, Julie; Alghamdi, Amani; Hortobágyi, Tibor; Ballard, Clive; Thomas, Alan J; O'Brien, John T; Aarsland, Dag; Francis, Paul T

    2015-02-01

    Depression is a common symptom in dementia with Lewy bodies (DLB), Parkinson disease dementia (PDD), and Alzheimer disease (AD), yet its molecular basis remains unclear and current antidepressants do not appear to be effective. Cerebral zinc has been implicated in depression and synaptic dysfunction. We investigated the relationship between synaptic zinc regulation (for which zinc transporter 3 [ZnT3] is responsible) and depression in a large clinicopathologic study. We examined brains from people with PDD (N = 29), DLB (N = 27), and AD (N = 15) and comparison subjects without depression or dementia (N = 24). Individuals were categorized according to the presence and severity of depression (on a scale of 0-3) based on standardized assessments during life (principally Neuropsychiatric Inventory). Western blotting was used to determine ZnT3 levels in Brodmann area 9 (BA9), and regression analysis was used to determine the relationship between ZnT3 and depression. Reductions in ZnT3 in BA9 were significantly associated with elevated depression scores in the study cohort (β = -0.351, df = 93, t = -3.318 p = 0.0004). This association remained when only individuals with DLB, PDD, and no dementia or depression were examined (β = -0.347, df = 78, t = -3.271, p = 0.002) or only individuals with AD and no dementia or depression were examined (β = -0.433, df = 37, t = -2.924, p = 0.006). Although decreased zinc levels have been implicated in the genesis of depression in animal models and in major depressive disorder in humans, this study provides the first evidence of a role for zinc in depression in people with dementia and highlights zinc metabolism as a therapeutic target. Copyright © 2015 American Association for Geriatric Psychiatry. Published by Elsevier Inc. All rights reserved.

  17. Contrasts Between Patients With Lewy Body Dementia Syndromes and APOE-ε3/ε3 Patients With Late-onset Alzheimer Disease Dementia.

    PubMed

    Oliveira, Fabricio F; Machado, Fernando C; Sampaio, Gustavo; Marin, Sheilla M C; Chen, Elizabeth S; Smith, Marilia C; Bertolucci, Paulo H F

    2015-08-01

    Neuropsychiatric and epidemiological patterns may compensate for insufficient specificity of diagnostic criteria of Lewy body dementia (LBD) syndromes in differential analysis with Alzheimer disease (AD) dementia. We aimed to compare and distinguish demographic and neuropsychiatric features between LBD and APOE-ε3/ε3 late-onset AD. A total of 39 consecutive patients with Parkinson disease dementia or dementia with Lewy bodies were matched with 39 APOE-ε3/ε3 patients with late-onset AD according to sex and Mini-Mental State Examination scores, and evaluated for education, age at disease onset, lifetime sanitary conditions, anthropometric measures, alcohol use, smoking, history of head trauma or bacterial infections, family history of neurodegenerative diseases, caregiver burden, functional independence, cognitive decline, neuropsychiatric symptoms, and pharmacological treatment. Family history of parkinsonism and worse motor performance were more prevalent in Parkinson disease dementia, also impacting sleep satisfaction and physical self-maintenance. Patients with AD had higher systolic blood pressure, were more independent, and had better performance in visuospatial tasks and calculations, whereas patients with LBD were more oriented and previously lived longer in rural areas without sanitation. Among neuropsychiatric symptoms, hallucinations, apathy, dysphoria, anxiety, and aberrant motor behavior were the most significant for discrimination amidst dementia diagnoses. Functional performance, visuospatial skills, and behavioral symptoms are helpful for differential diagnoses between LBD and AD. Cerebrovascular risk might be more important for AD pathogenesis, whereas environmental factors might impact development of LBD.

  18. James Parkinson's Chimera: syndrome or disease?

    PubMed

    Kempster, P A; Hurwitz, B; Lees, A J

    2017-06-01

    It is 200 years since James Parkinson published An Essay on the Shaking Palsy. While his monograph continues to be acclaimed for its precedence and clarity of description, what is often overlooked is the originality of Parkinson's ideas. Here we show that he appreciated the weakness of the systematic 18th century nosologies, which presupposed that medical species, the building blocks of these Linnaean taxonomic schemes, were as distinct as plant and animal species; and that Parkinson made a conceptual leap about combinations of clinical phenomena in recurring patterns, now recognised to be one of the germs of neurological thinking about syndromes. The Essay's written style underpins another aspect of significance to contemporary neurological practice - an inherent intellectual humility. In this commemorative year we locate the continuing importance of the related notions of syndrome and disease in successive frameworks of knowledge about the shaking palsy. Syndrome and disease are interpreted as dual character concepts, one clinically-based and the other restricted to pathophysiological causation. They both remain fundamental to understanding Parkinson's syndrome-disease today.

  19. Visual recognition memory differentiates dementia with Lewy bodies and Parkinson's disease dementia

    PubMed Central

    Mondon, K; Gochard, A; Marqué, A; Armand, A; Beauchamp, D; Prunier, C; Jacobi, D; de Toffol, B; Autret, A; Camus, V; Hommet, C

    2007-01-01

    Objective To compare cognitive impairments in dementia with Lewy bodies (DLB) and Parkinson's disease dementia (PDD), to discriminate between the two entities. Methods 10 DLB and 12 PDD consecutive patients performed a neuropsychological battery designed to assess several cognitive domains: verbal and visual memory (Delayed Matching to Sample (DMS)‐48), language, gnosia, praxia and executive functions. Results DLB patients had poorer performances in orientation (p<0.05), Trail Making Test A (p<0.05) and reading of names of colours in the Stroop Test (p<0.05). Their scores were also lower in the visual object recognition memory test (DMS‐48), in both immediate (p<0.05) and delayed recognition (p<0.05). No differences were observed in the other tests. Conclusion Despite global similarities in cognitive performances between DLB and PDD patients, we observed important differences: in particular, DMS‐48, a test of visual object recognition memory and visual storage capacity, was poorer in DLB patients. PMID:17287240

  20. PET radioligands reveal the basis of dementia in Parkinson disease and dementia with Lewy bodies

    PubMed Central

    Gomperts, Stephen N.; Marquie, Marta; Locascio, Joseph J.; Bayer, Stephen; Johnson, Keith A.; Growdon, John H.

    2015-01-01

    Background Effective therapies for dementia with Lewy bodies (DLB) and Parkinson disease dementia (PDD) will require accurate diagnosis and an understanding of the contribution of distinct molecular pathologies to these diseases. We seek to use imaging biomarkers to improve diagnostic accuracy and to clarify the contribution of molecular species to cognitive impairment in DLB and PD. Summary We have performed cross-sectional and prospective cohort studies in subjects with DLB, PD with normal cognition (PD-nl), PD with mild cognitive impairment (PD-MCI), and PD with dementia (PDD), contrasted with Alzheimer's disease (AD) and healthy control subjects (HCS). Subjects underwent formal neurologic examination, detailed neuropsychological assessments, MRI, and PET scans with radioligands altropane (DAT: dopamine transporter) and PiB (Aβ amyloid). Putamen DAT concentrations were similar in DLB and PD and differentiated them from HCS and AD. Decreased caudate DAT concentration related to functional impairment in DLB but not PD. PiB uptake was greatest in DLB. However, cortical PiB retention was common in PD and predicted cognitive decline. PET imaging of tau aggregates holds promise both to clarify the contribution of tau to cognitive decline in these diseases and to differentiate DLB and PD from the parkinsonian tauopathies. Key messages Together, DAT and amyloid PET imaging discriminate DLB from PD and from other disease groups and identify pathologic processes that contribute to their course. Multimodal PET imaging has potential to increase diagnostic accuracy of DLB and PD in the clinic, improve cohort uniformity for clinical trials, and serve as biomarkers for targeted molecular therapies. PMID:26655867

  1. Vestibular Impairment in Frontotemporal Dementia Syndrome

    PubMed Central

    Nakamagoe, Kiyotaka; Kadono, Kotarou; Koganezawa, Tadachika; Takiguchi, Mao; Terada, Makoto; Yamamoto, Fumiko; Moriyama, Tetsuya; Yanagiha, Kumi; Nohara, Seitaro; Tozaka, Naoki; Miyake, Zenshi; Aizawa, Satoshi; Furusho, Kentaro; Tamaoka, Akira

    2016-01-01

    Background No studies to date have attempted to evaluate frontotemporal lobar degeneration from the perspective of the vestibular system. Objective The present study examined vestibular function in patients with frontotemporal dementia (FTD) clinical syndrome and evaluated whether vestibular disorders are involved in the clinical symptoms due to FTD. Methods Fourteen patients with FTD syndrome, as well as healthy elderly controls without dementia, were included in the present study. All subjects underwent vestibular function tests using electronystagmography, such as caloric tests and visual suppression (VS) tests, in which the induced caloric nystagmus was suppressed by visual stimuli. The association between clinical symptoms and vestibular function in the FTD syndrome group was further examined. Results In the FTD syndrome group, caloric nystagmus was not necessarily suppressed during VS tests. Furthermore, VS was observed to be significantly impaired in FTD syndrome patients with gait disturbance as compared to those without such disturbance. Conclusion The present study revealed that impairment of VS in patients with FTD results in an inability to regulate vestibular function by means of visual perception, regardless of multiple presumed neuropathological backgrounds. This could also be associated with gait disturbance in patients with FTD syndrome. PMID:27350780

  2. Biomarkers of Parkinson's disease and Dementia with Lewy bodies.

    PubMed

    Henchcliffe, Claire; Dodel, Richard; Beal, M Flint

    2011-12-01

    Parkinson's disease (PD) and Dementia with Lewy bodies (DLB) are progressive and disabling neurodegenerative disorders, in which signs and symptoms overlap with each other and with other neurodegenerative conditions. Currently, diagnosis, measurement of progression, and response to therapeutic intervention rely upon clinical observation. However, there remains a critical need for validated biomarkers in each of these areas. A definitive diagnostic test would improve clinical management and enrollment into clinical trials. An objective measure of progression is vitally important in identifying neuroprotective interventions. Biomarkers may also provide insight into pathogenesis, and might therefore suggest possible novel targets for therapeutic intervention. In addition, certain biomarkers might be of use in monitoring the biochemical and physiological effects of therapeutic interventions. Development of diagnostic biomarkers has focused until recently upon imaging techniques based upon measuring loss of dopamine neurons. Additionally, advances in understanding the genetic contribution to neurodegenerative disorders, in particular in PD, have identified multiple causative genes and risk factors that in some cases may help estimate PD risk. However, recent availability of increasingly sophisticated bioinformatics technology has rendered development of fluid biomarkers feasible, opening the possibility of generally accessible blood or cerebrospinal fluid (CSF) tests that could impact upon diagnosis, management, and research in PD, PDD, and DLB.

  3. Neuroimaging correlates of cognitive impairment and dementia in Parkinson's disease.

    PubMed

    Mak, Elijah; Su, Li; Williams, Guy B; O'Brien, John T

    2015-08-01

    There has been a gradual shift in the definition of Parkinson's disease, from a movement disorder to a neurodegenerative condition affecting multiple cognitive domains. Mild cognitive impairment (PD-MCI) is a frequent comorbidity in PD that is associated with progression to dementia (PDD) and debilitating consequences for patients and caregivers. At present, the pathophysiology underpinning cognitive impairment in PD is not established, although emerging evidence has suggested that multi-modal imaging biomarkers could be useful in the early diagnosis of PD-MCI and PDD, thereby identifying at-risk patients to enable treatment at the earliest stage possible. Structural MRI studies have revealed prominent grey matter atrophy and disruptions of white matter tracts in PDD, although findings in non-demented PD have been more variable. There is a need for further longitudinal studies to clarify the spatial and temporal progression of morphological changes in PD, as well as to assess their underlying involvement in the evolution of cognitive deficits. In this review, we discuss the aetiology and neuropsychological profiles of PD-MCI and PDD, summarize the putative imaging substrates in light of evidence from multi-modal neuroimaging studies, highlight limitations in the present literature, and suggest recommendations for future research.

  4. Current Treatment Options for Alzheimer's Disease and Parkinson's Disease Dementia

    PubMed Central

    Szeto, Jennifer Y.Y.; Lewis, Simon J.G.

    2016-01-01

    Alzheimer's disease (AD) and Parkinson's disease (PD) are the two most common neurodegenerative disorders encountered in clinical practice. Whilst dementia has long been synonymous with AD, it is becoming more widely accepted as part of the clinical spectrum in PD (PDD). Neuropsychiatric complications, including psychosis, mood and anxiety disorders, and sleep disorders also frequently co-exist with cognitive dysfunctions in AD and PDD patients. The incidence of such symptoms is often a significant source of disability, and may aggravate pre-existing cognitive deficits. Management of AD and PDD involves both pharmacological and non-pharmacological measures. Although research on pharmacological therapies for AD and PDD has so far had some success in terms of developing symptomatic treatments, the benefits are often marginal and non-sustained. These shortcomings have led to the investigation of non-pharmacological and novel treatments for both AD and PD. Furthermore, in light of the diverse constellation of other neuropsychiatric, physical, and behavioural symptoms that often occur in AD and PD, consideration needs to be given to the potential side effects of pharmacological treatments where improving one symptom may lead to the worsening of another, rendering the clinical management of these patients challenging. Therefore, the present article will critically review the evidence for both pharmacological and non-pharmacological treatments for cognitive impairment in AD and PD patients. Treatment options for other concomitant neuropsychiatric and behavioural symptoms, as well as novel treatment strategies will also be discussed. PMID:26644155

  5. Downward finger displacement distinguishes Parkinson disease dementia from Alzheimer disease.

    PubMed

    Lieberman, Abraham; Deep, Aman; Shi, Jiong; Dhall, Rohit; Shafer, Saulena; Moguel-Cobos, Guillermo; Dhillon, Ravneet; Frames, Christopher W; McCauley, Margaret

    2017-09-15

    Purpose/Aim of the study: To study finger displacement in patients with Parkinson disease dementia (PDD) and in patients with Alzheimer disease (AD). We examined 56 patients with PDD and 35 with AD. Patients were examined during their regular outpatient clinic visit. Finger displacement was measured by observers not actively involved in the study using a creative grid ruler for all PDD and AD patients. Finger displacement was examined by asking patients to point their index fingers toward the grid ruler with the nails facing upward. Patients were asked to maintain the pointing position for 15 s. After 15 s, patients were asked to close their eyes for another 15 s while maintaining the same position. A positive result was downward index finger displacement of ≥5 cm within the 15-second time window with eyes closed. Of the 56 PDD patients, 53 had bilateral finger displacement of > 5 cm. In comparison, of the 35 AD patients, only 1 patient had minimal displacement. Results of the non-invasive finger displacement test may provide insight, on an outpatient basis, of the integrity of subcortical-cortical circuits. Downward finger displacement, especially bilateral downward displacement, may signal the extensive disruption of subcortical-cortical circuits that occurs in PDD patients.

  6. Use of the Rey Auditory Verbal Learning Test in Differentiating Normal Aging from Alzheimer's and Parkinson's Dementia.

    ERIC Educational Resources Information Center

    Tierney, Mary C.; And Others

    1994-01-01

    Thirty-eight elderly control subjects performed better than did 18 patients with moderate Alzheimer's disease (AD), 33 with severe AD, and 12 with Parkinson's dementia on all measures of the Rey Auditory Verbal Learning Test. Results indicate that the test is useful in distinguishing AD from Parkinson's dementia. (SLD)

  7. Use of the Rey Auditory Verbal Learning Test in Differentiating Normal Aging from Alzheimer's and Parkinson's Dementia.

    ERIC Educational Resources Information Center

    Tierney, Mary C.; And Others

    1994-01-01

    Thirty-eight elderly control subjects performed better than did 18 patients with moderate Alzheimer's disease (AD), 33 with severe AD, and 12 with Parkinson's dementia on all measures of the Rey Auditory Verbal Learning Test. Results indicate that the test is useful in distinguishing AD from Parkinson's dementia. (SLD)

  8. Changes in motor subtype and risk for incident dementia in Parkinson's disease.

    PubMed

    Alves, Guido; Larsen, Jan Petter; Emre, Murat; Wentzel-Larsen, Tore; Aarsland, Dag

    2006-08-01

    The objective of this study was to assess the temporal relationship between changes in predominant motor symptoms and incident dementia in Parkinson's disease (PD). A community-based sample of 171 nondemented patients with PD was followed prospectively and examined at baseline and after 4 and 8 years. The motor subtype of Parkinsonism was classified into tremor-dominant (TD), indeterminate, or postural instability gait difficulty (PIGD) subtype at each visit, based on defined items in the Unified Parkinson's Disease Rating Scale, subscales II and III. Dementia was diagnosed according to DSM-III-R criteria, based on clinical interview, cognitive rating scales, and neuropsychological examination. Logistic regression was used to analyze the relationship between subtype of Parkinsonism and dementia. Transition from TD to PIGD subtype was associated with a more than threefold increase in the rate of Mini-Mental State Examination decline. Compared to patients with persistent TD or indeterminate subtype, the odds ratio for dementia was 56.7 (95% CI: 4.0-808.4; P = 0.003) for patients changing from TD or indeterminate subtype to PIGD subtype, and 80.0 (95% CI: 4.6-1400.1; P = 0.003) for patients with persistent PIGD subtype. Patients with TD subtype at baseline did not become demented until they developed PIGD subtype, and dementia did not occur among patients with persistent TD subtype of Parkinsonism. In a substantial proportion of PD patients who develop postural instability and gait disorder during the course of the disease, this transition is associated with accelerated cognitive decline and highly increased risk for subsequent dementia. These findings raise the question whether PIGD and dementia share common or parallel neuropathology.

  9. Quality of life of patients with Parkinson's disease and neurodegenerative dementia: A nationally representative study.

    PubMed

    Chekani, Farid; Bali, Vishal; Aparasu, Rajender R

    2016-01-01

    The disability inherent to Parkinson's disease and dementia would suggest poor health-related quality of life for patients with these neurodegenerative conditions; however, the extent of disability from a nationally representative data has not been previously available. This study examined factors associated with the health-related quality of life in patients with Parkinson's disease and dementia using nationally representative samples. The study used data from 2002 to 2011 Medical Expenditure Panel Survey (MEPS), a nationally representative survey of households in the United States. The quality of life of patients was captured based on Physical Component Summary (PCS), Mental Component Summary (MCS), Activities of Daily Living (ADL) and Instrumental Activities of Daily Living (IADL). Multivariate regression models were used to compare PCS, MCS, ADL and IADL across the two neurodegenerative conditions after controlling for various sociodemographic and clinical characteristics. The weighted study population included 0.80 million (95% Confidence Interval, CI: 0.75-0.85) patients; those with Parkinson's disease accounted for 40.23% and remaining 59.77% were diagnosed with dementia. Mean age of the study population was 74.32 years (Standard Deviation, SD = 11.36). Most of the Parkinson's patients were male (57.70%), whereas most of the dementia patients were females (58.10%). The unadjusted mean PCS was 33.66 and 35.31 in Parkinson's and dementia patients, respectively (P < 0.01). Patients with Parkinson's disease were less likely to seek help for IADL than neurodegenerative dementia (Odds Ratio, OR = 0.68, P = 0.02). Various other individual, biological and environmental factors were also associated with quality of life in patients with Parkinson's disease and neurodegenerative dementia. This study found that patients with Parkinson's disease had lower PCS and were less likely to seek help for IADL when compared to the patients with neurodegenerative dementia

  10. Discrimination of dementia with lewy bodies from Alzheimer disease and Parkinson disease using the clock drawing test.

    PubMed

    Cahn-Weiner, Deborah A; Williams, Karren; Grace, Janet; Tremont, Geoffrey; Westervelt, Holly; Stern, Robert A

    2003-06-01

    The authors' objective was to examine the ability of the Clock Drawing Test to discriminate Dementia with Lewy bodies from Alzheimer disease and Parkinson disease. Recent advances in medical treatments for dementia underscore the importance of differentiating among dementia subtypes. Clinically, Dementia with Lewy bodies can often be difficult to discriminate from Alzheimer disease and Parkinson disease because of similar and overlapping cognitive and motor features. While the Clock Drawing Test has been shown to discriminate dementia from normal aging fairly accurately, less is known about its ability to discriminate between various dementia groups. Patients with Alzheimer disease (n = 22), patients with cognitively impaired Parkinson disease (n = 17), and patients with Dementia with Lewy bodies (n = 20), matched for age, education, and dementia severity, were compared on the Clock Drawing Test, scored for overall accuracy as well as the presence of specific error types. There were no significant group differences on a global quantitative measure of Clock Drawing Test performance. With regard to differences on the error types evaluated, the patients with Dementia with Lewy bodies were more likely to make conceptual errors than the patients with Alzheimer disease and Parkinson disease, and the patients with Parkinson disease and Dementia with Lewy bodies made more planning errors than the patients with Alzheimer disease. Classification accuracy was fair, with 69% overall classification for Alzheimer disease versus Dementia with Lewy bodies, and 68% overall classification for Parkinson disease versus Dementia with Lewy bodies. Although some differences may exist in the qualitative features of clock drawing performance between these patient groups, overall clock drawing performance is relatively similar, and as a single instrument, the Clock Drawing Test provides limited discrimination of Dementia with Lewy bodies from Alzheimer disease and Parkinson disease.

  11. Temporal lobe atrophy on MRI in Parkinson disease with dementia: a comparison with Alzheimer disease and dementia with Lewy bodies.

    PubMed

    Tam, C W C; Burton, E J; McKeith, I G; Burn, D J; O'Brien, J T

    2005-03-08

    To investigate the extent of medial temporal lobe atrophy (MTA) on MRI in Parkinson disease (PD) with and without dementia compared with Alzheimer disease (AD) and dementia with Lewy bodies (DLB) and to determine whether MTA correlates with cognitive impairment in PD and PD dementia (PDD). Coronal T1-weighted MRI scans were acquired from control subjects (n = 39) and patients with PD (n = 33), PDD (n = 31), DLB (n = 25), and AD (n = 31), diagnosed according to standardized clinical diagnostic criteria. Cognitive function was assessed using the Cambridge Cognitive Examination (CAMCOG), and MTA was rated visually using a standardized (Scheltens) scale. More severe MTA was seen in PDD (p = 0.007), DLB (p < 0.001), and AD (p < 0.001) vs control subjects. PD subjects had greater hippocampal atrophy than control subjects (p = 0.015) but less than subjects with DLB and AD, though not with PDD. MTA correlated with CAMCOG score and memory scores in the DLB group and with age in control, PDD, and AD groups. There were no correlations between MTA and cognitive impairment in PD, PDD, and AD. PDD and DLB had a similar profile of cognitive impairment and MTA. Medial temporal lobe atrophy (MTA) was seen in cognitively intact older subjects with Parkinson disease (PD) and was not more pronounced in Parkinson disease dementia (PDD). Alzheimer disease (AD) and, to a lesser extent, dementia with Lewy bodies (DLB) showed more pronounced MTA. Results suggest early hippocampal involvement in PD and that when dementia develops in PD, anatomic structures apart from the hippocampus are predominantly implicated. Greater hippocampal involvement in AD vs PDD and DLB is consistent with clinical, cognitive, and pathologic differences between the disorders.

  12. Cholinergic and perfusion brain networks in Parkinson disease dementia

    PubMed Central

    McKeith, Ian G.; Burn, David J.; Wyper, David J.; O'Brien, John T.; Taylor, John-Paul

    2016-01-01

    Objective: To investigate muscarinic M1/M4 cholinergic networks in Parkinson disease dementia (PDD) and their association with changes in Mini-Mental State Examination (MMSE) after 12 weeks of treatment with donepezil. Methods: Forty-nine participants (25 PDD and 24 elderly controls) underwent 123I-QNB and 99mTc-exametazime SPECT scanning. We implemented voxel principal components (PC) analysis, producing a series of PC images of patterns of interrelated voxels across individuals. Linear regression analyses derived specific M1/M4 and perfusion spatial covariance patterns (SCPs). Results: We found an M1/M4 SCP of relative decreased binding in basal forebrain, temporal, striatum, insula, and anterior cingulate (F1,47 = 31.9, p < 0.001) in cholinesterase inhibitor–naive patients with PDD, implicating limbic-paralimbic and salience cholinergic networks. The corresponding regional cerebral blood flow SCP showed relative decreased uptake in temporoparietal and prefrontal areas (F1,47 = 177.5, p < 0.001) and nodes of the frontoparietal and default mode networks (DMN). The M1/M4 pattern that correlated with an improvement in MMSE (r = 0.58, p = 0.005) revealed relatively preserved/increased pre/medial/orbitofrontal, parietal, and posterior cingulate areas coinciding with the DMN and frontoparietal networks. Conclusion: Dysfunctional limbic-paralimbic and salience cholinergic networks were associated with PDD. Established cholinergic maintenance of the DMN and frontoparietal networks may be prerequisite for cognitive remediation following cholinergic treatment in this condition. PMID:27306636

  13. Effects of carbonated liquid on swallowing dysfunction in dementia with Lewy bodies and Parkinson's disease dementia.

    PubMed

    Larsson, Victoria; Torisson, Gustav; Bülow, Margareta; Londos, Elisabet

    2017-01-01

    Swallowing dysfunction is an increasingly recognized problem in patients with dementia with Lewy bodies (DLB) and Parkinson's disease dementia (PDD), which can result in aspiration pneumonia and death. Few studies have examined potential ways of improving swallowing function in this fragile patient group. The aim of this study was to evaluate swallowing dysfunction and carbonated liquid using videofluoroscopy in DLB and PDD patients. A total of 48 patients with DLB and PDD were referred for a clinical examination with videofluoroscopy. Descriptive overall assessments were provided at the time of the examination regarding swallowing function and the effects of different modifications, including carbonated thin liquid (CTL). Additionally, a repeated measures quantitative retrospective analysis has been performed comparing 1) thin liquids; 2) thickened liquids and 3) CTLs, with regard to the quantitative variables 1) pharyngeal transit time (PTT); 2) pharyngeal retention and 3) tracheal penetration. In all, 40/48 (83%) of the patients had a swallowing dysfunction, which was confirmed on videofluoroscopy, with 34/40 (85%) patients having a pharyngeal-type dysfunction. A total of 14/40 (35%) patients with an objective swallowing impairment did not have any subjective swallowing symptoms. Out of the patients with swallowing dysfunction, 87% had an overall improved swallowing function with carbonated liquid. PTT for carbonated liquid (median 633 ms, interquartile range [IQR] 516-786 ms) was quicker than for thin liquid (760 ms, IQR 613-940 ms, P=0.014) and thickened liquid (880.0 ms, IQR 600-1,500 ms, P<0.001). No significant effect was seen in residue or penetration. The majority of patients with DLB or PDD had a swallowing dysfunction, sometimes without subjective swallowing symptoms, which improved with carbonated liquid. This highlights the importance of investigating patients with videofluoroscopy and to carry out a prospective interventional study to further

  14. Genetic Characterization of Movement Disorders and Dementias

    ClinicalTrials.gov

    2017-09-28

    Ataxia; Dystonia; Parkinson's Disease; Amyotrophic Lateral Sclerosis; Corticobasal Degeneration; Multiple System Atrophy; Alzheimer's Disease; Lewy Body Dementia; Parkinson Disease-Dementia; Dentatorubral-pallidoluysian Atrophy; Creutzfeldt-Jakob Disease and Fatal Familial Insomnia; Fragile X-associated Tremor/Ataxia Syndrome; Krabbe's Disease; Niemann-Pick Disease, Type C; Neuronal Ceroid Lipofuscinosis

  15. Dopamine agonist withdrawal syndrome in Parkinson disease.

    PubMed

    Rabinak, Christina A; Nirenberg, Melissa J

    2010-01-01

    To report and characterize a dopamine agonist (DA) withdrawal syndrome (DAWS) in Parkinson disease. Retrospective cohort study. Outpatient tertiary movement disorders clinic. Patients A cohort of 93 nondemented patients with Parkinson disease enrolled in a prospective study of nonmotor and motor disease manifestations. Main Outcome Measure The presence of DAWS, defined as a severe, stereotyped cluster of physical and psychological symptoms that correlate with DA withdrawal in a dose-dependent manner, cause clinically significant distress or social/occupational dysfunction, are refractory to levodopa and other Parkinson disease medications, and cannot be accounted for by other clinical factors. Of 40 subjects treated with a DA, 26 underwent subsequent DA taper. Of these 26 subjects, 5 (19%) developed DAWS and 21 (81%) did not. All subjects with DAWS had baseline DA-related impulse control disorders. Symptoms of DAWS resembled those of other drug withdrawal syndromes and included anxiety, panic attacks, agoraphobia, depression, dysphoria, diaphoresis, fatigue, pain, orthostatic hypotension, and drug cravings. Subjects with DAWS as compared with those without DAWS had higher baseline DA use (mean [SD], 420 [170] vs 230 [180] DA levodopa equivalent daily doses [DA-LEDD], respectively; P = .04) and higher cumulative DA exposure (mean [SD], 1800 [1200] vs 700 [900] DA-LEDD-years, respectively; P = .03). Subjects with DAWS also had considerably lower Unified Parkinson's Disease Rating Scale motor scores than those without DAWS (mean [SD], 21 [5] vs 31 [10], respectively; P = .007), despite comparable disease duration (mean [SD], 7.3 [7] vs 6.3 [4] years, respectively; P = .77) and similar total dopaminergic medication use (mean [SD], 830 [450] vs 640 [610] total LEDD, respectively; P = .52) in the 2 groups. Dopamine agonists have a stereotyped withdrawal syndrome that can lead to profound disability in a subset of patients. Physicians should monitor patients closely when

  16. Brain Connectivity Alterations Are Associated with the Development of Dementia in Parkinson's Disease.

    PubMed

    Bertrand, Josie-Anne; McIntosh, Anthony R; Postuma, Ronald B; Kovacevic, Natasha; Latreille, Véronique; Panisset, Michel; Chouinard, Sylvain; Gagnon, Jean-François

    2016-04-01

    Dementia affects a high proportion of Parkinson's disease (PD) patients and poses a burden on caregivers and healthcare services. Electroencephalography (EEG) is a common nonevasive and nonexpensive technique that can easily be used in clinical settings to identify brain functional abnormalities. Only few studies had identified EEG abnormalities that can predict PD patients at higher risk for dementia. Brain connectivity EEG measures, such as multiscale entropy (MSE) and phase-locking value (PLV) analyses, may be more informative and sensitive to brain alterations leading to dementia than previously used methods. This study followed 62 dementia-free PD patients for a mean of 3.4 years to identify cerebral alterations that are associated with dementia. Baseline resting state EEG of patients who developed dementia (N = 18) was compared to those of patients who remained dementia-free (N = 44) and of 37 healthy subjects. MSE and PLV analyses were performed. Partial least squares statistical analysis revealed group differences associated with the development of dementia. Patients who developed dementia showed higher signal complexity and lower PLVs in low frequencies (mainly in delta frequency) than patients who remained dementia-free and controls. Conversely, both patient groups showed lower signal variability and higher PLVs in high frequencies (mainly in gamma frequency) compared to controls, with the strongest effect in patients who developed dementia. These findings suggest that specific disruptions of brain communication can be measured before PD patients develop dementia, providing a new potential marker to identify patients at highest risk of developing dementia and who are the best candidates for neuroprotective trials.

  17. Goal Setting for Cognitive Rehabilitation in Mild to Moderate Parkinson's Disease Dementia and Dementia with Lewy Bodies.

    PubMed

    Watermeyer, Tamlyn J; Hindle, John V; Roberts, Julie; Lawrence, Catherine L; Martyr, Anthony; Lloyd-Williams, Huw; Brand, Andrew; Gutting, Petra; Hoare, Zoe; Edwards, Rhiannon Tudor; Clare, Linda

    2016-01-01

    Alongside the physical symptoms associated with Parkinson's disease dementia and dementia with Lewy bodies, health services must also address the cognitive impairments that accompany these conditions. There is growing interest in the use of nonpharmacological approaches to managing the consequences of cognitive disorder. Cognitive rehabilitation is a goal-orientated behavioural intervention which aims to enhance functional independence through the use of strategies specific to the individual's needs and abilities. Fundamental to this therapy is a person's capacity to set goals for rehabilitation. To date, no studies have assessed goal setting in early-stage Parkinson's disease dementia or dementia with Lewy bodies. Semistructured interviews were carried out with 29 participants from an ongoing trial of cognitive rehabilitation for people with these conditions. Here, we examined the goal statements provided by these participants using qualitative content analysis, exploring the types and nature of the goals set. Participants' goals reflected their motivations to learn new skills or improve performance in areas such as technology-use, self-management and orientation, medication management, and social and leisure activities. These results suggest that goal setting is achievable for these participants, provide insight into the everyday cognitive difficulties that they experience, and highlight possible domains as targets for intervention. The trial is registered with ISRCTN16584442 (DOI 10.1186/ISRCTN16584442 13/04/2015).

  18. Dementia in Parkinson's Disease Correlates with α-Synuclein Pathology but Not with Cortical Astrogliosis

    PubMed Central

    van den Berge, Simone A.; Kevenaar, Josta T.; Sluijs, Jacqueline A.; Hol, Elly M.

    2012-01-01

    Dementia is a common feature in Parkinson's disease (PD) and is considered to be the result of limbic and cortical Lewy bodies and/or Alzheimer changes. Astrogliosis may also affect the development of dementia, since it correlates well with declining cognition in Alzheimer patients. Thus, we determined whether cortical astrogliosis occurs in PD, whether it is related to dementia, and whether this is reflected by the presence of glial fibrillary acidic protein (GFAP) and vimentin in cerebrospinal fluid (CSF). We have examined these proteins by immunohistochemistry in the frontal cortex and by Western blot in CSF of cases with PD, PD with dementia (PDD), dementia with Lewy bodies (DLB) and nondemented controls. We were neither able to detect an increase in cortical astrogliosis in PD, PDD, or DLB nor could we observe a correlation between the extent of astrogliosis and the degree of dementia. The levels of GFAP and vimentin in CSF did not correlate to the extent of astrogliosis or dementia. We did confirm the previously identified positive correlation between the presence of cortical Lewy bodies and dementia in PD. In conclusion, we have shown that cortical astrogliosis is not associated with the cognitive decline in Lewy body-related dementia. PMID:22577599

  19. Parkinson disease with dementia: comparing patients with and without Alzheimer pathology.

    PubMed

    Sabbagh, Marwan N; Adler, Charles H; Lahti, Tyson J; Connor, Donald J; Vedders, Linda; Peterson, Lars K; Caviness, John N; Shill, Holly A; Sue, Lucia I; Ziabreva, Iryna; Perry, Elaine; Ballard, Clive G; Aarsland, Dag; Walker, Douglas G; Beach, Thomas G

    2009-01-01

    Subjects with Parkinson disease (PD) frequently develop dementia with greater than one-third meeting neuropathologic diagnostic criteria for Alzheimer disease (AD). The objective is to identify clinical and neuropathologic differences between Parkinson disease with dementia (PDD) subjects, with and without coexistent AD pathology. Neuropathologic examination was available on subjects diagnosed by clinicopathologic criteria with PDD-AD (N=23) and PDD+AD (N=28). A small subset of subjects with PDD-AD and PDD+AD had received at least 1 standardized neuropsychologic assessment. PDD+AD subjects were significantly older at age of PD onset and death, progressed to onset of dementia in less time, and had a shorter duration of PD symptoms before the onset of dementia. Education, responsiveness of L-dopa and dopaminergic medications, presence of cognitive fluctuations and hallucinations, and mean Mini-Mental State Examination, Global Deterioration Scale, Functional Assessment Staging, and Unified Parkinson Disease Rating Scale scores did not differ significantly between the 2 groups. The PDD+AD group had significantly greater total plaques, neuritic plaques, total tangles, and Braak stages compared with PDD-AD. This study suggests that it is difficult to distinguish PDD+AD and PDD-AD on the basis of movement, clinical, and neuropsychologic assessment. PDD-AD and PDD+AD have similar degrees of dementia and approximately half of PDD subjects have enough AD pathology to attain a neuropathologic diagnosis of AD. PDD can develop in the absence of significant Alzheimer pathology.

  20. Association between environmental tobacco smoke exposure and dementia syndromes

    PubMed Central

    Chen, Ruoling; Wilson, Kenneth; Chen, Yang; Zhang, Dongmei; Qin, Xia; He, Meizi; Hu, Zhi; Ma, Ying; Copeland, John R

    2013-01-01

    Objectives Environmental tobacco smoke (ETS) has a range of adverse health effects, but its association with dementia remains unclear and with dementia syndromes unknown. We examined the dose–response relationship between ETS exposure and dementia syndromes. Methods Using a standard method of GMS, we interviewed 5921 people aged ≥60 years in five provinces in China in 2007–2009 and characterised their ETS exposure. Five levels of dementia syndrome were diagnosed using the Automated Geriatric Examination for Computer Assisted Taxonomy instrument. The relative risk (RR) of moderate (levels 1–2) and severe (levels 3–5) dementia syndromes among participants exposed to ETS was calculated in multivariate adjusted regression models. Results 626 participants (10.6%) had severe dementia syndromes and 869 (14.7%) moderate syndromes. Participants exposed to ETS had a significantly increased risk of severe syndromes (adjusted RR 1.29, 95% CI 1.05 to 1.59). This was dose-dependently related to exposure level and duration. The cumulative exposure dose data showed an adjusted RR of 0.99 (95% CI 0.76 to 1.28) for >0–24 level years of exposure, 1.15 (95% CI 0.93 to 1.42) for 25–49 level years, 1.18 (95% CI 0.87 to 1.59) for 59–74 level years, 1.39 (95% CI 1.03 to 1.84) for 75–99 level years and 1.95 (95% CI 1.34 to 2.83) for ≥100 level years. Significant associations with severe syndromes were found in never smokers and in former/current smokers. There were no positive associations between ETS and moderate dementia syndromes. Conclusions ETS should be considered an important risk factor for severe dementia syndromes. Avoidance of ETS may reduce the rates of severe dementia syndromes worldwide. PMID:23104731

  1. Next-generation profiling to identify the molecular etiology of Parkinson dementia.

    PubMed

    Henderson-Smith, Adrienne; Corneveaux, Jason J; De Both, Matthew; Cuyugan, Lori; Liang, Winnie S; Huentelman, Matthew; Adler, Charles; Driver-Dunckley, Erika; Beach, Thomas G; Dunckley, Travis L

    2016-06-01

    We sought to determine the underlying cortical gene expression changes associated with Parkinson dementia using a next-generation RNA sequencing approach. In this study, we used RNA sequencing to evaluate differential gene expression and alternative splicing in the posterior cingulate cortex from neurologically normal control patients, patients with Parkinson disease, and patients with Parkinson disease with dementia. Genes overexpressed in both disease states were involved with an immune response, whereas shared underexpressed genes functioned in signal transduction or as components of the cytoskeleton. Alternative splicing analysis produced a pattern of immune and RNA-processing disturbances. Genes with the greatest degree of differential expression did not overlap with genes exhibiting significant alternative splicing activity. Such variation indicates the importance of broadening expression studies to include exon-level changes because there can be significant differential splicing activity with potential structural consequences, a subtlety that is not detected when examining differential gene expression alone, or is underrepresented with probe-limited array technology.

  2. Progressive supranuclear palsy presenting as primary lateral sclerosis but lacking parkinsonism, gaze palsy, aphasia, or dementia.

    PubMed

    Nagao, Shigeto; Yokota, Osamu; Nanba, Reiko; Takata, Hiroshi; Haraguchi, Takashi; Ishizu, Hideki; Ikeda, Chikako; Takeda, Naoya; Oshima, Etsuko; Sakane, Katsuaki; Terada, Seishi; Ihara, Yuetsu; Uchitomi, Yosuke

    2012-12-15

    We report an autopsy case of progressive supranuclear palsy (PSP) that clinically showed only slowly progressive and symmetric upper motor neuron syndrome over a disease course of 12 years. A female patient initially exhibited dysarthria at the age of 65, followed by gait disturbance and dysphagia. Neurological examination at age 67 disclosed pseudobulbar palsy, spastic gait, hyperreflexia, and presence of bilateral Hoffmann and Babinski signs. However, muscle atrophy, weakness, evidence of denervation on electromyography, vertical gaze palsy, parkinsonism, gait freezing, aphasia, speech apraxia, or dementia was not noted throughout the course. She was clinically diagnosed as having motor neuron disease consistent with so-called primary lateral sclerosis. Pathological examination disclosed histopathological features of PSP, including argyrophilic and tau-positive tufted astrocytes, neurofibrillary tangles, coiled bodies, and thread-like processes in the motor cortex and superior frontal gyrus, and to a lesser degree, in the basal ganglia and brain stem nuclei. In addition, severe fibrillary gliosis was noted in the precentral gyrus and corticospinal tract, being consistent with upper motor neuron syndrome observed in this case. No TAR-DNA binding protein 43-positive lesion, FUS pathology, Bunina body, or Lewy body-like hyaline inclusion was noted in the motor cortex or lower motor neurons. These findings suggest that when tau pathology is prominent in the motor cortex but is minimal in the basal ganglia and brain stem nuclei, a PSP case can lack all classic clinical features of PSP and show only slowly progressive upper motor syndrome, consistent with clinical picture of primary lateral sclerosis.

  3. [Mild dementia syndrome in middle and old age].

    PubMed

    Zharikov, G A

    1998-01-01

    95 elderly and senile patients with mild dementia were followed up: 20 patients with Alzheimer's disease, 25 patients with senile dementia of Alzheimer's type (DAT), 25 patients with vascular dementia (VD), 25 patients with combined vascular and Alzheimer's type of dementia (DAT/VD). Follow-up of patients for 1 and 3 years demonstrated that the diagnosis of mild dementia according to CDR and ICD-10 criteria had a differential-diagnostic specificity and reliability. It was also noticed that the nosologic qualification of mild dimentia syndrome was difficult or even impossible in patients with DAT/VD in conditions of a single examination. Only follow-up studies (during 3 years as a rule) may give a chance to define the diagnostic belonging of mild dementia syndrome more precisely.

  4. A romantic delusion: de Clerambault's syndrome in dementia.

    PubMed

    Cipriani, Gabriele; Logi, Chiara; Di Fiorino, Andrea

    2012-07-01

    Erotromania (also known as de Clerambault's syndrome) is a rare disorder in which an individual has a delusional belief that a person of a socially higher standing falls in love with her/him. It has rarely been described in older people, but many cases have been reported in conjunction with psychiatric and neurological disorders. The purpose of this paper was to examine the phenomenon of erotomania in people with dementia. We carried out a search of electronic databases for literature on this subject. The search terms used were: erotomania, de Clerambault's syndrome, erotomanic delusion and dementia. The literature on erotomania in the course of dementia consists mostly of case reports and small samples of patients. Misinterpretation of events is common in brain disease, especially with diffuse or multifocal disorders, but erotomania has rarely been reported in dementia. The relationship between dementia and de Clerambault's syndrome remains uncertain. Erotic delusion arising late in life should be thoroughly investigated to rule out organicity.

  5. [Dopamine dysregulation syndrome in Parkinson's disease and restless legs syndrome].

    PubMed

    Bayard, Sophie; Cochen De Cock, Valérie; Dauvillers, Yves

    2011-06-01

    Dopamine replacement therapy in Parkinson's disease (PD) improves the motor symptoms. However, it has recently been shown that a small sub-group of patients suffers from motor and behavioral disturbances associated with the use of dopamine agonists (DAs). The behavioral disorders are incentive- or reward-based repetitive symptoms regrouped under the term « dopamine dysregulation syndrome » (DDS). They include pathological gambling, hypersexuality, compulsive shopping, compulsive eating, punding, and compulsive medication use. Whether these behaviors are related to the dopaminergic medications interacting with an underlying individual vulnerability or whether the primary pathological features of Parkinson's disease play a role is not entirely understood. This review is devoted to the phenomenology of the DDS and factors influencing its susceptibility. We further review the literature studies that investigated the decision-making profile using the Iowa Gambling Task in Parkinson's disease, and the recent literature devoted to these abnormal behaviors in the restless legs syndrome (RLS). Given the potential substantial impact of the DDS on personal, familial, social, and financial well-being, patients with PD or RLS should be informed that DAs use may lead to the development of impulsive and compulsive disorders, and clinicians should include the investigation of these disorders as part of routine clinical care. The refinement of clinical strategies to predict, identify and manage DDS will help the future care of motor and non-motor symptoms of Parkinson's disease.

  6. Capgras syndrome in Dementia with Lewy Bodies

    PubMed Central

    Thaipisutikul, Papan; Lobach, Iryna; Zweig, Yael; Gurnani, Ashita; Galvin, James E.

    2014-01-01

    Background Capgras syndrome is characterized by the recurrent, transient belief that a person has been replaced by an identical imposter. We reviewed clinical characteristics of Dementia with Lewy Bodies (DLB) patients with Capgras syndrome compared to those without Capgras. Methods We identified 55 consecutive DLB patients (11 cases with Capgras syndrome (DLB-C) and 44 cases without evidence of Capgras (DLB). Semi-structured interviews with the patient and an informant, neurological exams, and neuropsychological testing were performed. Caregivers were assessed for caregiver burden and depression. Primary comparisons were made between DLB-C and DLB. Exploratory analyses using stepwise logistic regression and bootstrap analyses were performed to determine clinical features associated with Capgras. Results DLB-C patients experienced more visual hallucinations and self-reported anxiety, had higher scores on the Neuropsychiatric Inventory, and were less likely to be treated with cholinesterase inhibitors at time of initial evaluation. Extrapyramidal symptoms and depression were not associated with Capgras. Caregivers of DLB-C patients had higher caregiver burden. DLB-C was associated with self-reported anxiety (OR 10.9; 95% CI 2.6-47.6). In a bootstrap analysis, clinical findings that were predictors of Capgras included visual hallucinations (log(OR) 18.3; 95% CI 17.9-19.3) and anxiety (log(OR) 2.9; 95% CI (0.31-20.2). Conclusions Our study suggests that Capgras syndrome is common in DLB and usually occurs in the presence of anxiety and visual hallucinations, suggesting related etiopathogenesis. Early appreciation of Capgras syndrome may afford the opportunity to alleviate caregiver burden and improve patient and caregiver outcomes. PMID:23211760

  7. Combined dementia-risk biomarkers in Parkinson's disease: a prospective longitudinal study.

    PubMed

    Compta, Yaroslau; Pereira, Joana B; Ríos, Jose; Ibarretxe-Bilbao, Naroa; Junqué, Carme; Bargalló, Núria; Cámara, Ana; Buongiorno, Mariateresa; Fernández, Manel; Pont-Sunyer, Claustre; Martí, Maria J

    2013-08-01

    Neuropsychological (mostly posterior-cortical) deficits, quantitative magnetic resonance imaging (MRI) atrophy patterns, and low cerebrospinal fluid (CSF) levels of amyloid-β have been separately related to worsening cognition in Parkinson's disease (PD). However, these biomarkers have not been longitudinally assessed in combination as PD-dementia predictors. In this prospective longitudinal study, 27 non-demented PD patients underwent CSF, neuropsychological and 3-T brain-MRI studies at baseline and were re-assessed 18 months later in terms of progression to dementia (primary outcome) and longitudinal neuropsychological and cortical thickness changes (secondary outcomes). At follow-up 11 patients (41%) had progressed to dementia. Lower CSF amyloid-β, worse verbal learning, semantic fluency and visuoperceptual scores, and thinner superior-frontal/anterior cingulate and precentral regions were significant baseline dementia predictors in binary logistic regressions as quantitative and/or dichotomised traits. All participants without baseline biomarker abnormalities remained non-demented whereas all with abnormalities in each biomarker type progressed to dementia, with intermediate risk for those showing abnormalities in a single to two biomarker types (p = 0.006). Both the dementia-outcome and low baseline CSF amyloid-β were prospectively associated with limbic and posterior-cortical neuropsychological decline and frontal, limbic and posterior-cortical thinning from baseline to follow-up. These findings suggest that the combination of CSF amyloid-β, neuropsychological and cortical thickness biomarkers might provide a basis for dementia-risk stratification and progression monitoring in PD.

  8. The applause sign in Parkinson's disease patients is related to dysexecutive syndrome.

    PubMed

    Tomic, Svetlana; Vladetic, Mirjana; Solic, Kresimir; Misevic, Sanja; Soldo, Silva Butkovic

    2013-12-01

    Recent publications report that a positive applause sign is not only present in patients with neurodegenerative diseases where the subcortical structures are affected but also in patients with cortical dementia. The nature of this sign remains unknown. This study aimed to determine the frequency of the applause sign and its correlation with cognitive impairment in patients with idiopathic Parkinson's disease. The study included 30 non-depressed patients diagnosed with idiopathic Parkinson's disease. Study patients underwent the Unified Parkinson Disease Rating Scale part III, Dementia Rating Scale (DRS), Raven's Colored Progressive Matrices, and Mill Hill Vocabulary tests. Statistical analysis was performed by use of the parametric Student's t-test, nonparametric Mann-Whitney U test and Fisher's exact test, with the level of significance set at p<0.05. Negative applause sign was recorded in 66.7% and positive applause sign in 33.3% of patients. There were no between-group differences according to age, disease duration, or severity of motor symptoms. The positive applause sign group had significantly lower scores on the initiation/perseveration subscale of the DRS and a significantly higher frequency of scores below the cut-off score on the conceptualization and construction subscales of the DRS. The applause sign appears to be part of a broader dysexecutive syndrome in idiopathic Parkinson's disease.

  9. Dropped head syndrome in Parkinson's disease.

    PubMed

    Kashihara, Kenichi; Ohno, Manabu; Tomita, Susumu

    2006-08-01

    We determined the frequency of dropped head syndrome in Parkinson's disease (PD) in Japan and evaluated its clinical correlates. A total of 252 consecutive patients with PD who visited our hospital were studied. Dropped head syndrome was found in 15 patients (6.0%) (3 men, 12 women; mean age at onset of PD, 62.8 +/- 11.5 years). The interval before emergence of dropped head after disease onset was 5.4 +/- 4.3 years (-0.5 to 15 years). The Hoehn-Yahr score at the on stage was 3.2 +/- 0.7; at the off stage 3.5 +/- 0.8. Of those 15 patients, 8 had major symptoms of rigidity and akinesia. In 2 patients, administration of a dopamine agonist appeared to evoke dropped head syndrome. An increase in and/or the addition of antiparkinsonian drugs alleviated head drop in 4 patients and reduced head drop in 7 patients. Any medication was not effective for 4 patients. Dropped head syndrome in PD is not rare in Japan. It is more often observed in women and is associated with patients who primarily suffer rigidity and akinesia. Dropped head syndrome in these patients appears to be produced by disproportionate tonus of the neck muscles. It is modulated by antiparkinsonian drugs and is considered to be a type of dystonia.

  10. Capgras Syndrome in a Patient with Parkinson's Disease after Bilateral Subthalamic Nucleus Deep Brain Stimulation: A Case Report

    PubMed Central

    Kyrtsos, Christina Rose; Stahl, Mark C.; Eslinger, Paul; Subramanian, Thyagarajan; Lucassen, Elisabeth B.

    2015-01-01

    Capgras syndrome is a delusional misidentification syndrome (DMS) which can be seen in neurodegenerative diseases such as Lewy body dementia and, to a lesser extent, in Parkinson's disease (PD). Here, we report the case of a 78-year-old man with a history of idiopathic PD who developed Capgras syndrome following bilateral subthalamic nucleus deep brain stimulation (DBS) implantation. As the risk of DMS has been related to deficits in executive, memory, and visuospatial function preoperatively, this case highlights the importance of continuing to improve patient selection for DBS surgery. Capgras syndrome is a rare potential complication of DBS surgery in PD patients with preexisting cognitive decline. PMID:26078747

  11. Localization of disinhibition-dementia-parkinsonism-amyotrophy complex to 17q21-22

    SciTech Connect

    Wilhelmsen, K.C.; Lynch, T.; Pavlou, E.; Higgins, M.; Nygaard, T.G.

    1994-12-01

    Disinhibition-dementia-parkinsonism-amyotrophy complex (DDPAC) is defined by familial adult-onset behavioral disturbance, followed by frontal lobe dementia, parkinsonism, and amyotrophy in variable proportions. A genetic etiology of DDPAC was suspected because of the familial clustering in family Mo, despite their wide geographic distribution. We have mapped the DDPAC locus to a 12-cM (sex averaged) region between D17S800 and D17S787 on chromosome 17q21-22. The basis for the variability of the clinical findings and pathology in DDPAC is unknown but suggests that the DDPAC locus should be screened as a candidate locus in family studies of conditions with behavioral abnormalities and neurological degeneration.

  12. Localization of disinhibition-dementia-parkinsonism-amyotrophy complex to 17q21-22.

    PubMed Central

    Wilhelmsen, K. C.; Lynch, T.; Pavlou, E.; Higgins, M.; Nygaard, T. G.

    1994-01-01

    Disinhibition-dementia-parkinsonism-amyotrophy complex (DDPAC) is defined by familial adult-onset behavioral disturbance, followed by frontal lobe dementia, parkinsonism, and amyotrophy in variable proportions. A genetic etiology of DDPAC was suspected because of the familial clustering in family Mo, despite their wide geographic distribution. We have mapped the DDPAC locus to a 12-cM (sex averaged) region between D17S800 and D17S787 on chromosome 17q21-22. The basis for the variability of the clinical findings and pathology in DDPAC is unknown but suggests that the DDPAC locus should be screened as a candidate locus in family studies of conditions with behavioral abnormalities and neurological degeneration. PMID:7977375

  13. Etiology and Pathophysiology of Frontotemporal Dementia, Parkinson Disease and Alzheimer Disease: Lessons from Genetic Studies

    PubMed Central

    Wider, Christian; Wszolek, Zbigniew K.

    2008-01-01

    Genetic studies have led to major discoveries in the pathogenesis of various neurodegenerative diseases. Ubiquitin-positive familial frontotemporal dementia was recently found to be caused by mutations in the progranulin gene (PGRN), and the major constituent of the inclusions, TDP-43, was subsequently identified. The tau gene (MAPT) causes frontotemporal dementia with parkinsonism linked to chromosome 17. In Parkinson disease, LRRK2 mutations have emerged as a major cause of both familial and sporadic forms, adding to the previously known genes SNCA, PRKN, DJ1 and PINK1. Several genes have been implicated in Alzheimer disease, including the APP gene and the PSEN genes. Recently, variants in the sortilin-related receptor 1 gene, SORL1, were associated with Alzheimer disease. PMID:18322368

  14. Etiology and pathophysiology of frontotemporal dementia, Parkinson disease and Alzheimer disease: lessons from genetic studies.

    PubMed

    Wider, Christian; Wszolek, Zbigniew K

    2008-01-01

    Genetic studies have led to major discoveries in the pathogenesis of various neurodegenerative diseases. Ubiquitin-positive familial frontotemporal dementia was recently found to be caused by mutations in the progranulin gene (PGRN), and the major constituent of the inclusions, TDP-43, was subsequently identified. The tau gene (MAPT) causes frontotemporal dementia with parkinsonism linked to chromosome 17. In Parkinson disease, LRRK2 mutations have emerged as a major cause of both familial and sporadic forms, adding to the previously known genes SNCA,PRKN,DJ1 and PINK1. Several genes have been implicated in Alzheimer disease, including the APP gene and the PSEN genes. Recently, variants in the sortilin-related receptor 1 gene, SORL1, were associated with Alzheimer disease. 2008 S. Karger AG, Basel

  15. History of Wolff-Parkinson-White syndrome.

    PubMed

    Scheinman, Melvin M

    2005-02-01

    While Drs. Wolff, Parkinson, and White fully described the syndrome that bears their names in 1930, prior case reports had already described the essentials. Over the ensuing century this syndrome has captivated the interest of anatomists, clinical cardiologists, and cardiac surgeons. Stanley Kent described lateral muscular connections over the atrioventricular (AV) groove, which he felt were the normal AV connections. The normal AV connections were, however, clearly described by His and Tawara. True right-sided AV connections were initially described by Wood et al., while Ohnell first described left free wall pathways. David Scherf is thought to be the first to describe our current understanding of the pathogenesis of the Wolff-Parkinson-White (WPW) syndrome in terms of a reentrant circuit involving both the AV node--His axis as well as the accessory pathway. This hypothesis was not universally accepted and many theories were applied to explain the clinical findings. The basics of our understandings were established by the brilliant work of Pick, Langendorf, and Katz who by using careful deductive analysis of ECGs were able to define the basic pathophysiological processes. Subsequently, Wellens and Durrer applied invasive electrical stimulation to the heart in order to confirm the pathophysiological processes. Sealy and his colleagues at Duke University Medical Center were the first to successfully surgically divide an accessory pathway and ushered in the modern area for curative therapy for these patients. Morady and Scheinman were the first to successfully ablate an accessory pathway (posteroseptal) using high-energy direct-current shocks. Subsequently, Jackman, Kuck, Morady, and a number of groups proved the remarkable safety and efficiency of catheter ablation for pathways in all locations using radiofrequency energy. More recently, Gallob et al. first described the gene responsible for a familial form of WPW. The current ability to cure patients with WPW is

  16. Olfactory dysfunction and dementia in newly diagnosed patients with Parkinson's disease.

    PubMed

    Domellöf, Magdalena Eriksson; Lundin, Karl-Fredrik; Edström, Mona; Forsgren, Lars

    2017-05-01

    Studies report that up to 90% of patients with idiopathic Parkinson's disease (PD) have olfactory dysfunction (hyposmia). Hyposmia has also been connected to cognitive impairment and dementia in PD, but no studies of newly diagnosed patients followed longer than three years exists. The present study investigates the prevalence of olfactory dysfunction at PD diagnosis, how it evolves over time and whether hyposmia increases the risk of dementia in Parkinson's disease. Olfactory function was assessed with Brief Smell Identification Test (B-SIT) in 125 newly diagnosed patients with PD. They were followed for a maximum of 10 years (median six years) with extensive investigations at baseline, 12, 36, 60 and 96 months. Patients with B-SIT<9 were considered hyposmic. Hyposmia was found in 73% of the patients at diagnosis. During the follow up period of ten years 42 (46%) patients with hyposmia at baseline developed dementia compared to seven (21%) of the normosmic patients. Cox proportional hazards model showed that hyposmia at baseline (controlled for age, gender, UPDRS III and Mild Cognitive Impairment) increased the risk of developing dementia (hazard ratio (95%CI): 3.29 (1.44-7.52), p = 0.005). Only one of 22 patients with normal cognition and normal olfaction at baseline developed dementia. Olfactory dysfunction was common at the time of PD diagnosis and increased the risk of dementia up to ten years after PD diagnosis regardless of baseline cognitive function. Normal olfaction together with normal cognition at baseline predicted a benign cognitive course up to ten years after diagnosis. Copyright © 2017 Elsevier Ltd. All rights reserved.

  17. [Dopamine dysregulation syndrome in Parkinson's disease].

    PubMed

    Borg, M; Bayreuther, C

    2008-04-01

    Dopamine replacement therapy in Parkinson's disease ameliorates motor symptoms. However, it has recently been recognized that a small subgroup of patients suffer motor and behavioral disturbances attributable to taking quantities of medication well beyond the dose required to treat their motor disabilities. Dopamine dysregulation syndrome can be regarded as a pattern of compulsive medication use leading to disabling motor and behavioral features. The major theories of psychostimulant addiction may help explain some of the phenomena seen in the dopamine dysregulation syndrome. In contrast to the predictable pattern of severe degeneration of ventrolateral nigral dopaminergic cells, there is a smaller and more variable loss of dopamine neurons within the ventral tegmental areas. Sensitization of ventral striatal networks to antiparkinsonian therapy and appetitive behaviors may be analogous to the neuroplastic changes in the dorsal striatum thought to contribute to the motor complications of treatment such as dyskinesias. This syndrome greatly affects patients, their families and society. Treatment is difficult; deep brain stimulation of the subthalamic nucleus may therefore prove useful in some cases.

  18. The Views of People Who Care for Adults with Down's Syndrome and Dementia: A Service Evaluation

    ERIC Educational Resources Information Center

    Furniss, Kate Atkins; Loverseed, Annie; Lippold, Tessa; Dodd, Karen

    2012-01-01

    It is well established that people with Down's syndrome are more likely to develop dementia than other people and that onset of dementia is likely to occur earlier at an earlier age. The article reports on a specialist service for people with Down's syndrome and dementia. The service has offered dementia screening and assessment to people with…

  19. The Views of People Who Care for Adults with Down's Syndrome and Dementia: A Service Evaluation

    ERIC Educational Resources Information Center

    Furniss, Kate Atkins; Loverseed, Annie; Lippold, Tessa; Dodd, Karen

    2012-01-01

    It is well established that people with Down's syndrome are more likely to develop dementia than other people and that onset of dementia is likely to occur earlier at an earlier age. The article reports on a specialist service for people with Down's syndrome and dementia. The service has offered dementia screening and assessment to people with…

  20. Italian version of the Parkinson Neuropsychometric Dementia Assessment (PANDA): a useful instrument to detect cognitive impairments in Parkinson's Disease.

    PubMed

    Pignatti, Riccardo; Bertella, Laura; Scarpina, Federica; Mauro, Alessandro; Portolani, Elisa; Calabrese, Pasquale

    2014-01-01

    Parkinson's disease (PD) is frequently characterized by cognitive and affective dysfunctions. The "Parkinson Neuropsychometric Dementia Assessment" (PANDA) is a screening tool designed for the early detection of mild cognitive impairment as well as dementia in PD. The PANDA is already validated in German and in French. The aim of the present work was to provide normative data for the Italian-speaking population, Swiss regions included; moreover, the effectiveness of the PANDA compared to the Mini Mental State Examination (MMSE) was tested. One-hundred and eleven PD patients with and without cognitive impairment and one-hundred and three matched healthy subjects participated at this study; all patients underwent an extensive neuropsychological evaluation. A PANDA total score of 13 appeared to be the most fitting cut-off with a sensitivity of 96.6% and a specificity of 82.2%; with the MMSE, the same value of sensitivity but with a specificity of 72,4% was reached only by adopting a cut-off of 28. Moreover, a PANDA range of 13-17 appeared to be suggestive for possible cognitive disturbance. The present work provides evidence for the effectiveness of the PANDA in evaluating cognitive deficits also in PD Italian-speaking patients, even when their pathological degree is still initial or very mild.

  1. Using the Montreal Cognitive Assessment Scale to screen for dementia in Chinese patients with Parkinson's Disease

    PubMed Central

    CHEN, Ling; YU, Cuiyu; FU, Xiaosu; LIU, Weiguo; HUA, Ping; ZHANG, Ning; KUO, Shenghan

    2013-01-01

    Background Dementia is one of the most distressing and burdensome health problems associated with Parkinson's Disease (PD). The Montreal Cognitive Assessment scale (MoCA) is widely used to screen for dementia in PD patients, but the appropriate diagnostic cutoff score when used with Chinese PD patients is not known. Aim Determine a diagnostic cutoff value of the Chinese version of the MoCA (MoCA-C) for Chinese PD patients and describe the characteristics of PD patients screened positive for dementia using the MoCA-C. Methods The presence of dementia in 616 PD patients and 85 community controls was determined using the Movement Disorder Society Task Force criteria (the gold standard diagnosis). We administered the MoCA-C to these individuals and used a receiver operating characteristic (ROC) curve to identify the cutoff score of the MoCA-C that most efficiently identified dementia in both PD patients and community controls. Demographic and clinical characteristics of PD patients who were screened positive or negative for dementia using the MoCA-C were compared. Results A MoCA-C score of 23 was the optimal cutoff score for dementia in both patients and controls. Using this cutoff score, the sensitivity and specificity of the MoCA-C in PD patients were 0.70 and 0.77, respectively; the positive and negative predictive values were 0.59 and 0.85, respectively; and the overall concordance (kappa [95% confidence interval]) was 0.45 (0.39-0.52). The corresponding kappa value (concordance) in community controls was only 0.25 (0.05-0.45). Compared to PD patients who screened negative for dementia, those who screened positive for dementia were significantly impaired in all cognitive domains, including visuospatial and executive functioning, naming, attention, language, abstraction, delayed recall and orientation (all p<0.001). Among the PD patients, screening positive for dementia was independently associated with old age, low educational attainment, female gender and more

  2. Cholesterol and Alzheimer Type Dementia among Adults with Down Syndrome

    ERIC Educational Resources Information Center

    Buckley, Frank

    2008-01-01

    This article reports a summary of research by Warren Zigman and colleagues investigating the link between cholesterol levels and Alzheimer type dementia among adults with Down syndrome. Warren Zigman and colleagues followed 123 adults with Down syndrome between May 1998 and April 2006. The participants were aged between 41 and 78 years at the…

  3. Cholesterol and Alzheimer Type Dementia among Adults with Down Syndrome

    ERIC Educational Resources Information Center

    Buckley, Frank

    2008-01-01

    This article reports a summary of research by Warren Zigman and colleagues investigating the link between cholesterol levels and Alzheimer type dementia among adults with Down syndrome. Warren Zigman and colleagues followed 123 adults with Down syndrome between May 1998 and April 2006. The participants were aged between 41 and 78 years at the…

  4. The distinct cognitive syndromes of Parkinson's disease: 5 year follow-up of the CamPaIGN cohort.

    PubMed

    Williams-Gray, Caroline H; Evans, Jonathan R; Goris, An; Foltynie, Thomas; Ban, Maria; Robbins, Trevor W; Brayne, Carol; Kolachana, Bhaskar S; Weinberger, Daniel R; Sawcer, Stephen J; Barker, Roger A

    2009-11-01

    Cognitive abnormalities are common in Parkinson's disease, with important social and economic implications. Factors influencing their evolution remain unclear but are crucial to the development of targeted therapeutic strategies. We have investigated the development of cognitive impairment and dementia in Parkinson's disease using a longitudinal approach in a population-representative incident cohort (CamPaIGN study, n = 126) and here present the 5-year follow-up data from this study. Our previous work has implicated two genetic factors in the development of cognitive dysfunction in Parkinson's disease, namely the genes for catechol-O-methyltransferase (COMT Val(158)Met) and microtubule-associated protein tau (MAPT) H1/H2. Here, we have explored the influence of these genes in our incident cohort and an additional cross-sectional prevalent cohort (n = 386), and investigated the effect of MAPT H1/H2 haplotypes on tau transcription in post-mortem brain samples from patients with Lewy body disease and controls. Seventeen percent of incident patients developed dementia over 5 years [incidence 38.7 (23.9-59.3) per 1000 person-years]. We have demonstrated that three baseline measures, namely, age >or=72 years, semantic fluency less than 20 words in 90 s and inability to copy an intersecting pentagons figure, are significant predictors of dementia risk, thus validating our previous findings. In combination, these factors had an odds ratio of 88 for dementia within the first 5 years from diagnosis and may reflect the syndrome of mild cognitive impairment of Parkinson's disease. Phonemic fluency and other frontally based tasks were not associated with dementia risk. MAPT H1/H1 genotype was an independent predictor of dementia risk (odds ratio = 12.1) and the H1 versus H2 haplotype was associated with a 20% increase in transcription of 4-repeat tau in Lewy body disease brains. In contrast, COMT genotype had no effect on dementia, but a significant impact on Tower of London

  5. Dementia in idiopathic Parkinson's disease. A neuropathological study of 32 cases.

    PubMed

    Gaspar, P; Gray, F

    1984-01-01

    Neuronal loss was estimated semiquantitatively in the substantia nigra (SN) and locus coeruleus (LC), and by cell counts in the nucleus basalis of Meynert (NBM), in 32 patients with idiopathic Parkinson's disease (14 non-demented and 18 demented). The number of senile plaques (SP) and neurofibrillary tangles (NFT) was rated in four cortical areas. Neuronal loss in the SN seemed in dependent of mental impairment, while severe lesions of the LC were more frequent in demented patients. In the NBM, neuronal loss and Lewy bodies were observed in most cases (95%) and were associated with significant reductions of choline acetyltransferase (CAT) activity both in the NBM and the cortex (measurements available for 13 cases). This confirms that the cholinergic innominato-cortical pathway is damaged in Parkinson's disease and that the lesion is severer in subjects with dementia. SP and NFT were present in the cortex in 75% of the cases and significantly more numerous in demented patients. However, in 37% of the cases (six cases with dementia), the score for cortical changes was low and could be related to age. Cortical SP and NFT were not correlated to the degree of cell loss in LC and NBM, or to CAT activity in the cortex or NBM. Damage to coeruleo-cortical, innominato-cortical and intra-cortical neurones could each play a role in the appearance of dementia in Parkinsonism. The lesions in the different neuronal systems do not seem to evolve in parallel, but may be additive or potentiate one another in terms of functional expression. Also, the variety in extent and degree of lesions encountered in Parkinson's disease may offer a pathological substrate for the wide variety of mental symptoms described in this illness.

  6. The Adaptive Behaviour Dementia Questionnaire (ABDQ): Screening Questionnaire for Dementia in Alzheimer's Disease in Adults with Down Syndrome

    ERIC Educational Resources Information Center

    Prasher, V.; Farooq, A.; Holder, R.

    2004-01-01

    The diagnosis of dementia in Alzheimer's disease remains at times problematic in adults with intellectual disability. The analysis of 5-year consecutive data developed a researched-based clinical screening tool for dementia in Alzheimer's disease in adults with Down syndrome. The Adaptive Behaviour Dementia Questionnaire (ABDQ) is a 15-item…

  7. The Adaptive Behaviour Dementia Questionnaire (ABDQ): Screening Questionnaire for Dementia in Alzheimer's Disease in Adults with Down Syndrome

    ERIC Educational Resources Information Center

    Prasher, V.; Farooq, A.; Holder, R.

    2004-01-01

    The diagnosis of dementia in Alzheimer's disease remains at times problematic in adults with intellectual disability. The analysis of 5-year consecutive data developed a researched-based clinical screening tool for dementia in Alzheimer's disease in adults with Down syndrome. The Adaptive Behaviour Dementia Questionnaire (ABDQ) is a 15-item…

  8. Pathologic Accumulation of α-Synuclein and Aβ in Parkinson Disease Patients With Dementia

    PubMed Central

    Kotzbauer, Paul T.; Cairns, Nigel J.; Campbell, Meghan C.; Willis, Allison W.; Racette, Brad A.; Tabbal, Samer D.; Perlmutter, Joel S.

    2013-01-01

    Objective To determine the relative contributions of individual pathologic protein deposits associated with parkinson disease (PD). Design Autopsied patients were analyzed from February 24, 2005, through July 25, 2010, to determine the distribution and severity of individual pathologic protein deposits (α-synuclein, Aβ, and tau) using routine protocols for histologic and immunohistochemical analysis and established neuropathologic staging criteria. Clinical data were extracted from an electronic medical record system used for all patients with PD. Patients Thirty-two consecutive autopsied patients treated at the Washington University Movement Disorders Center who had neuropathologic confirmation of PD and a history of dementia, regardless of the timing of the onset of dementia with respect to motor symptoms. Results Three pathologic subgroups of dementia associated with PD were identified: (1) predominant synucleinopathy (Braak Lewy body stages 5–6) (12 [38%]), (2) predominant synucleinopathy with Aβ deposition (Braak amyloid stages B–C) but minimal or no cortical tau deposition (19 [99%]), and (3) synucleinopathy and Aβ deposition with at least moderate neocortical tauopathy (Braak tau stages 5–6; 1 [3%]). Kaplan-Meier and Cox regression analyses revealed that patients with synucleinopathy plus Aβ deposition had significantly shorter survival (years from PD onset until death and years from dementia onset until death) than patients with synucleinopathy only. Conclusions Dementia associated with PD has 2 major pathologic subgroups: neocortical synucleinopathy and neocortical synucleinopathy with Aβ deposition. Alzheimer disease with neocortical Aβ and tau deposition does not commonly cause dementia with PD. Furthermore, accumulation of Aβ is associated with lower survival rates in PD patients with dementia. Additional studies are needed to prospectively determine the association between α-synuclein and Aβ accumulation and the role of Aβ in the

  9. Guidelines for dementia or Parkinson's disease with depression or anxiety: a systematic review.

    PubMed

    Goodarzi, Zahra; Mele, Bria; Guo, Selynne; Hanson, Heather; Jette, Nathalie; Patten, Scott; Pringsheim, Tamara; Holroyd-Leduc, Jayna

    2016-11-25

    Depression and anxiety remain under-diagnosed and under-treated in those with neurologic diseases such as dementia or Parkinson's Disease (PD). Our objectives were to first, to provide a synthesis of high quality guidelines available for the identification and management of depression or anxiety in those with dementia or PD. Second, to identify areas for improvement for future guidelines. We searched MEDLINE, PsycINFO, and EMBASE (2009 to July 24, 2015), grey literature (83 sources; July 24-Sept 6, 2015), and bibliographies of included studies. Included studies were evaluated for quality by four independent reviewers the AGREE II tool. Guideline characteristics, statements and recommendations relevant to depression or anxiety for dementia and PD were then extracted. (PROSPERO CRD: 42016014584) RESULTS: 8121 citations were reviewed with 31 full text articles included for assessment with the AGREE II tool. 17 were of sufficient quality for inclusion. Mean overall quality scores were between 4.25 to 6.5. Domain scores were lowest in the areas of stakeholder involvement, applicability, and editorial independence. Recommendations for the screening and diagnosis of depression were found for PD and dementia. There was little evidence to guide diagnosis or management of anxiety. Non-pharmacologic therapies were recommended for dementia patients. Most advocated pharmacologic treatment for depression, for both PD and dementia, but did not specify an agent due to lack of evidence. The available recent high quality guidelines outline several recommendations for the management of comorbid depression or anxiety in PD or dementia. However there remain significant gaps in the evidence.

  10. Parkinson's disease with mild cognitive impairment: severe cortical thinning antedates dementia.

    PubMed

    Gasca-Salas, Carmen; García-Lorenzo, Daniel; Garcia-Garcia, David; Clavero, Pedro; Obeso, José A; Lehericy, Stephane; Rodríguez-Oroz, María C

    2017-07-14

    Mild cognitive impairment (MCI) in Parkinson's disease (PD) is a risk factor for dementia and thus, it is of interest to elucidate if specific patterns of atrophy in PD-MCI patients are associated with a higher risk of developing dementia. We aim to define pattern(s) of regional atrophy in PD-MCI patients who developed dementia during 31 months of follow-up using cortical thickness analysis Twenty-three PD-MCI patients and 18 controls underwent brain MRI and completed a neuropsychological examination at baseline, PD-MCI patients were followed after a 31 month follow-up in order to assess their progression to dementia. At follow up, 8 PD-MCI patients had converted to dementia (PD-MCI converters) whereas 15 remained as PD-MCI (PD-MCI non-converters). All patients were at least 60 years old and suffered PD ≥ 10 years. There were no baseline differences between the two groups of patients in clinical and neuropsychological variables. The cortex of PD-MCI converters was thinner than that of PD-MCI non-converters, bilaterally in the frontal, insula and the left middle temporal areas, also displaying a more widespread pattern of cortical thinning relative to the controls. This study shows that aged and long-term PD patients with MCI who convert to dementia in the short-mid term suffer a thinning of the cortex in several areas (frontal cortex, and middle temporal lobe and insula), even when their cognitive impairment was similar to that of PD-MCI non-converters. Thus, MRI analysis of cortical thickness may represent a useful measure to identify PD-MCI patients at a higher risk of developing dementia.

  11. Fragile X tremor ataxia syndrome and white matter dementia.

    PubMed

    Filley, Christopher M

    2016-08-01

    Fragile X tremor ataxia syndrome (FXTAS) is an inherited neurodegenerative disease in which dementia is common and disabling. The pathogenesis of dementia in FXTAS is poorly understood, but the salience of executive dysfunction and slowed processing speed, the frequent presence of the middle cerebellar peduncle sign on magnetic resonance imaging (MRI), and striking neuropathological alterations of white matter all suggest that myelinated tracts are significantly involved. This paper considers the role of white matter disease in FXTAS dementia, particularly with regard to the concept of white matter dementia (WMD). A focused review of FXTAS in relation to known white matter disorders is provided to propose that the concept of WMD may illuminate the basis of dementia in FXTAS. The putative pathogenetic contribution of white matter involvement in other neurodegenerative diseases is also considered. Considerable evidence supports the importance of white matter disease in the pathogenesis of dementia in FXTAS. Whereas, gray matter regions are also involved, white matter degeneration is prominent, even early in the disease, and correlates with executive dysfunction and slowed processing speed. Evidence for white matter involvement in other neurodegenerative diseases lends additional support to the relevance of white matter in FXTAS. The dementia of FXTAS is closely related to the profile of WMD, and white matter involvement is also supported by MRI and neuropathological observations. White matter pathology is also relevant to the pathogenesis of other neurodegenerative diseases. Further study of white matter promises to clarify the origin of dementia in FXTAS.

  12. Next-generation profiling to identify the molecular etiology of Parkinson dementia

    PubMed Central

    Henderson-Smith, Adrienne; Corneveaux, Jason J.; De Both, Matthew; Cuyugan, Lori; Liang, Winnie S.; Huentelman, Matthew; Adler, Charles; Driver-Dunckley, Erika; Beach, Thomas G.

    2016-01-01

    Objective: We sought to determine the underlying cortical gene expression changes associated with Parkinson dementia using a next-generation RNA sequencing approach. Methods: In this study, we used RNA sequencing to evaluate differential gene expression and alternative splicing in the posterior cingulate cortex from neurologically normal control patients, patients with Parkinson disease, and patients with Parkinson disease with dementia. Results: Genes overexpressed in both disease states were involved with an immune response, whereas shared underexpressed genes functioned in signal transduction or as components of the cytoskeleton. Alternative splicing analysis produced a pattern of immune and RNA-processing disturbances. Conclusions: Genes with the greatest degree of differential expression did not overlap with genes exhibiting significant alternative splicing activity. Such variation indicates the importance of broadening expression studies to include exon-level changes because there can be significant differential splicing activity with potential structural consequences, a subtlety that is not detected when examining differential gene expression alone, or is underrepresented with probe-limited array technology. PMID:27275011

  13. Lower urinary tract symptoms in dementia with Lewy bodies, Parkinson disease, and Alzheimer disease.

    PubMed

    Ransmayr, G N; Holliger, S; Schletterer, K; Heidler, H; Deibl, M; Poewe, W; Madersbacher, H; Kiss, G

    2008-01-22

    The present study sought to investigate lower urinary tract symptoms and urodynamic and cystometric findings in Parkinson disease (PD), dementia with Lewy bodies (DLB), and Alzheimer disease (AD). Included were patients with frequency, urgency, incontinence, and nocturia, without major bladder outflow obstruction. The protocol comprised physical examination, urine analysis, prostate specific antigen, 24-hours frequency of micturition, mean voided volume (MVV), free flow before instrumentation (Qmax(before)), post-void residual volume (PVR), and cystometry. Fifteen patients with DLB and PD and 16 patients with AD were examined. MVV, PVR, Qmax(before) and with transurethral catheter, cystometric bladder capacity, and detrusor pressor at maximum flow were similar in the three groups and corresponded to values of the general elderly population. Urge episodes and urge incontinence were observed in 93 and 53% of the patients with DLB, 53 and 27% of the patients with PD, and 19 and 12% of the patients with AD, and detrusor overactivity in 92% of the patients with DLB, 46% of the patients with PD, and 40% of the patients with AD. Urgency and urge incontinence suggest detrusor overactivity, which was more prevalent in dementia with Lewy bodies than in Parkinson disease and Alzheimer disease, whereas mean voided volume, free flow, cystometric bladder capacity, and detrusor pressor were similar in the groups. Frequency of micturition could not be reliably assessed in patients with dementia.

  14. Sleep spindles in Parkinson's disease may predict the development of dementia.

    PubMed

    Latreille, Véronique; Carrier, Julie; Lafortune, Marjolaine; Postuma, Ronald B; Bertrand, Josie-Anne; Panisset, Michel; Chouinard, Sylvain; Gagnon, Jean-François

    2015-02-01

    Sleep disturbances and cognitive impairment are common non-motor manifestations of Parkinson's disease (PD). Recent studies suggest that sleep spindles and slow waves play a role in brain plasticity mechanisms and are associated with cognitive performance. However, it remains unknown whether these sleep parameters could serve as markers of cognitive decline in PD. Therefore, we examined whether alterations in sleep spindles and slow waves at baseline visit were associated with increased likelihood of developing dementia at follow-up in PD. Sixty-eight nondemented PD patients (64.9 ± 8.8 years old; 46 men) participated in the study, along with 47 healthy individuals (65.0 ± 10.6 years old; 30 men). All participants underwent baseline polysomnographic recording and a comprehensive neuropsychological assessment. Sleep spindles (12-15 Hz) and slow waves (>75 μV and <4 Hz) were automatically detected on all-night non-rapid eye movement sleep electroencephalography. At follow-up (mean: 4.5 years later), 18 PD patients developed dementia (70.2 ± 7.6 years old; 13 men) and 50 remained dementia-free (63.0 ± 8.5 years old; 33 men). Sleep spindle density and amplitude were lower in PD patients who converted to dementia compared with both patients who remained dementia-free and controls, mostly in posterior cortical regions (p < 0.05). Dementia-free PD patients were intermediate between dementia patients and controls, with lower baseline sleep spindle density in all cortical areas compared with controls (p < 0.01). In demented PD patients, lower sleep spindle amplitude in parietal and occipital areas was associated with poorer visuospatial abilities. Although slow wave amplitude was lower in PD patients compared with controls (p < 0.0001), no difference was observed between those who developed or did not develop dementia. Results demonstrate non-rapid eye movement sleep electroencephalographic abnormalities in PD patients. Sleep spindle activity was particularly impaired

  15. [Wolff-Parkinson-White syndrome and cardiopathies].

    PubMed

    Soria, R; Fernandez, F; Heller, J; Brétille, J; Cherif, F; Barrillon, A; Gerbaux, A; Gay, J

    1984-12-01

    Forty-nine cases of Wolff-Parkinson-White syndrome (WPW) were diagnosed out of 10 750 patients with cardiac disease (0.45 p. 100), 24 cases out of 3 761 congenital malformations and 25 cases in the 6 989 patients with acquired heart disease. Right ventricular pre-excitation was recorded in 31 cases; 13 in the lateral zone, 12 in the posterior paraseptal zone and 6 in the anterior paraseptal zone. Left ventricular pre-excitation was recorded in 18 cases: 8 in the lateral zone, 5 in the anterior paraseptal and 5 in the posterior paraseptal zones. WPW and congenital heart disease: Out of 20 cases of Ebstein's anomaly, 5 cases of WPW were observed: 4 right posterior and 1 right lateral pre-excitations. Out of 218 cases of hypertrophic obstructive cardiomyopathy, 7 cases of WPW were observed, 4 of which were congenital. Three cases of WPW were recorded in 699 patients with ventricular septal defects. Out of 1 348 cases of atrial septal defect, 5 cases of pre-excitation were recorded, including 3 right posterior pre-excitations associated with an ostium primum defect. Pre-excitation was also observed in isolated cases of corrected transposition of the great arteries, supravalvular aortic stenosis, aortic incompetence and patent ductus arteriosus. Pre-excitation and acquired heart disease: Five cases of pre-excitation were recorded out of 305 cases of dilated cardiomyopathy (1.62 p. 100). Eleven cases of pre-excitation were recorded in a total of 3 471 cases of valvular heart disease (0.31 p. 100): 9 in rheumatic valve disease and 2 in mitral valve prolapse. Nine cases of pre-excitation were observed in 2 850 cases of coronary artery disease. Intermittent Wolff-Parkinson-White syndrome: Ventricular pre-excitation masks the ECG changes of complete right bundle branch block in Ebstein's anomaly, complete left bundle branch block in aortic incompetence and dilated cardiomyopathy, and the in-complete right bundle branch block often seen in mitral valve prolapse. The

  16. Multi-infarct dementia, subcortical dementia, and hydrocephalus.

    PubMed

    Nadeau, S E

    1991-05-01

    The diagnostic evaluation of dementia is directed toward the identification of treatable causes. It can be facilitated by classification of the dementia into one of four categories: attentional, amnestic, cognitive, and intentional. Intentional dementia reflects dysfunction of frontal lobe systems, components of which include the frontal cortex, basal ganglia, thalamus, limbic structures, and subcortical white matter. Disorders that affect one or more of these components and produce intentional dementia include Binswanger's disease, Parkinson's disease, Huntington's disease, HIV infection, closed head injury, normal pressure hydrocephalus, neoplasms, syphilis, vitamin B12 deficiency, multiple sclerosis, and a number of uncommon degenerative and acquired syndromes. Depression may resemble intentional dementia. Guidelines for diagnosis and management are discussed.

  17. Dementia, stroke and Parkinson's disease in Spanish populations: a review of door-to-door prevalence surveys.

    PubMed

    del Barrio, José Luis; de Pedro-Cuesta, Jesús; Boix, Raquel; Acosta, Jesús; Bergareche, Alberto; Bermejo-Pareja, Félix; Gabriel, Rafael; García de Yébenes, María Jesús; García, Francisco José; López-Pousa, Secundino; Manubens, José María; Mateos, Raimundo; Matías-Guiu, Jordi; Olivé, Josep María; Reñé, Ramón; Rodríguez, Fernanda; Saz, Pedro

    2005-01-01

    We identified 14 door-to-door prevalence surveys on dementia, parkinsonism or stroke in Spanish populations fulfilling specific criteria and combined selected age- and sex-specific data using logistic regression and taking Pamplona as a reference. The prevalence of dementia and of Alzheimer's disease varied significantly with space. However, the largest variation was seen for vascular dementia: odds ratio (OR) and 95% confidence interval (CI) for Gerona were 6.42 (3.23-12.3) in women and 2.30 (1.10-4.79) in men. Stroke was particularly frequent among Arevalo's women, with OR 2.10 and 95% CI 1.26-3.49. The prevalence of Parkinson's disease was twofold higher in Cantalejo. Although differences in methodology make the interpretation of results problematic, the prevalence of stroke and vascular dementia in Spain seems to vary spatially, indicating a space for prevention.

  18. Motor performance differentiates individuals with Lewy body dementia, Parkinson's and Alzheimer's disease.

    PubMed

    Fritz, Nora E; Kegelmeyer, Deborah A; Kloos, Anne D; Linder, Shannon; Park, Ariane; Kataki, Maria; Adeli, Anahita; Agrawal, Punit; Scharre, Douglas W; Kostyk, Sandra K

    2016-10-01

    Differential diagnosis of dementia with Lewy bodies (DLB), Parkinson's disease with dementia (PDD), Parkinson's disease (PD) and Alzheimer's disease (AD) is challenging. Comparative motor profiles of these neurodegenerative disorders may aid in earlier diagnosis but have not been extensively studied. Groups were rigorously matched by age, education, and sex. DLB/PDD participants were matched by Mini-Mental State Examination Score to individuals with AD and by Unified Parkinson's Disease Rating Scale motor scores to individuals with PD. Gait, balance, dual task walking and hand dexterity measures were compared between a combined group (n=21) of individuals with Lewy body dementia (LBD) consisting of those with DLB (n=11) and PDD (n=10) to individuals with PD (n=21) or AD (n=21). Individuals at the same disease stage with LBD walked significantly slower with shorter stride lengths (p<0.05), demonstrated poorer balance on both the Tinetti and Berg Balance Scale, and poorer performance on dual-task and figure-of-eight walking compared to PD and AD (p<0.05 for all) groups. Upper extremity coordination on the 9-hole peg test differentiated LBD from both PD and AD and was the only motor test in which individuals with AD performed worse than those with PD. Tinetti balance subscores were significantly lower in PDD compared to DLB participants (10.4±2.3 versus 12.8±2.3; p=0.027). Motor features distinguish individuals with LBD from those with AD and PD. Measures of gait, balance and finger dexterity provide an additional means of differentiating individuals with LBD from those with AD and PD. Copyright © 2016 Elsevier B.V. All rights reserved.

  19. Visual Hallucinations and Amyloid Deposition in Parkinson's Disease Dementia: A Case Report.

    PubMed

    Um, Yoo Hyun; Kim, Tae-Won; Jeong, Jong-Hyun; Seo, Ho-Jun; Han, Jin-Hee; Hong, Seung-Chul; Jung, Won-Sang; Choi, Woo Hee; Lee, Chang-Uk; Lim, Hyun Kook

    2016-05-01

    Parkinson's disease dementia (PDD) is notorious for its debilitating clinical course and high mortality rates. Consequently, various attempts to investigate predictors of cognitive decline in Parkinson's disease (PD) have been made. Here we report a case of a 75-year-old female patient with PD who visited the clinic with complaints of recurrent visual hallucinations and cognitive decline, whose symptoms were ameliorated by the titration of rivastigmine. Imaging results showed pronounced diffuse cortical amyloid deposition evidenced by 18F-florbetaben amyloid positron emission tomography (PET) imaging. This observation suggests that pronounced amyloid deposition and visual hallucinations in PD patients could be clinically significant predictors of cognitive decline in PD patients. Future research should concentrate on accumulating more evidence for possible predictors of cognitive decline and their association with PD pathology that can enable an early intervention and standardized treatment in PDD patients.

  20. Everyday cognitive failures and memory problems in Parkinson's patients without dementia.

    PubMed

    Poliakoff, Ellen; Smith-Spark, James H

    2008-08-01

    There is growing evidence that Parkinson's disease patients without dementia exhibit cognitive deficits in some executive, memory and selective attention tasks. However, the impact of these deficits on their everyday cognitive functioning remains largely unknown. This issue was explored using self-report questionnaires. Twenty-four Parkinson's patients and 24 age-matched controls rated how frequently they make particular cognitive errors, such as forgetting what they were about to say. In addition, a partner or significant other also rated each participant's propensity for making cognitive errors. Rather than simply rating themselves as making more of all types of errors, these results indicate that PD patients make more of specific types of error. Further analysis suggests that some of these errors are related to attentional processes (being more distractible) whereas others are related to retrieval processes (being unable to recall important details from the previous day).

  1. Demography, diagnostics, and medication in dementia with Lewy bodies and Parkinson's disease with dementia: data from the Swedish Dementia Quality Registry (SveDem).

    PubMed

    Fereshtehnejad, Seyed-Mohammad; Religa, Dorota; Westman, Eric; Aarsland, Dag; Lökk, Johan; Eriksdotter, Maria

    2013-01-01

    Whether dementia with Lewy bodies (DLB) and Parkinson's disease with dementia (PDD) should be considered as one entity or two distinct conditions is a matter of controversy. The aim of this study was to compare the characteristics of DLB and PDD patients using data from the Swedish Dementia Quality Registry (SveDem). SveDem is a national Web-based quality registry initiated to improve the quality of diagnostic workup, treatment, and care of patients with dementia across Sweden. Patients with newly diagnosed dementia of various types were registered in SveDem during the years 2007-2011. The current cross-sectional report is based on DLB (n = 487) and PDD (n = 297) patients. Demographic characteristics, diagnostic workup, Mini-Mental State Examination (MMSE) score, and medications were compared between DLB and PDD groups. No gender differences were observed between the two study groups (P = 0.706). PDD patients were significantly younger than DLB patients at the time of diagnosis (74.8 versus 76.8 years, respectively; P < 0.001). A significantly higher prevalence of patients with MMSE score ≤24 were found in the PDD group (75.2% versus 67.6%; P = 0.030). The mean number of performed diagnostic modalities was significantly higher in the DLB group (4.9 ± 1.7) than in the PDD group (4.1 ± 1.6; P < 0.001). DLB patients were more likely than PDD patients to be treated with cholinesterase inhibitors (odds ratio = 2.5, 95% confidence interval = 1.8-3.5), whereas the use of memantine, antidepressants, and antipsychotics did not differ between the groups. This study demonstrates several differences in the dementia work-up between DLB and PDD. The onset of dementia was significantly earlier in PDD, while treatment with cholinesterase inhibitors was more common in DLB patients. Severe cognitive impairment (MMSE score ≤24) was more frequent in the PDD group, whereas more diagnostic tests were used to confirm a DLB diagnosis. Some similarities also were found, such as

  2. Intraneuronal aluminum accumulation in amyotrophic lateral sclerosis and Parkinsonism-dementia of Guam

    SciTech Connect

    Perl, D.P.; Gajdusek, D.C.; Garruto, R.M.; Yanagihara, R.T.; Gibbs, C.J.

    1982-09-10

    Scanning electron microscopy with energy-dispersive x-ray spectrometry was used to analyze the elemental content of neurofibrillary tangle (NFT)-bearing and NFT-free neurons within the Sommer's sector (H1 region) of the hippocampus in Guamanian Chamorros with amyotrophic lateral sclerosis and parkinsonism-dementia and in neurologically normal controls. Preliminary data indicate prominent accumulation of aluminum within the nuclear region and perikaryal cytoplasm of NFT-bearing hippocampal neurons, regardless of the underlying neurological diagnosis. These findings further extend the association between intraneuronal aluminum and NFT formation and support the hypothesis that environmental factors are related to the neurodegenerative changes seen in the Chamorro population.

  3. Pareidolia in Parkinson's disease without dementia: A positron emission tomography study.

    PubMed

    Uchiyama, Makoto; Nishio, Yoshiyuki; Yokoi, Kayoko; Hosokai, Yoshiyuki; Takeda, Atsushi; Mori, Etsuro

    2015-06-01

    Pareidolia, which is a particular type of complex visual illusion, has been reported to be a phenomenon analogous to visual hallucinations in patients with dementia with Lewy bodies. However, whether pareidolia is observed in Parkinson's disease (PD) or whether there are common underlying mechanisms of these two types of visual misperceptions remains to be elucidated. A test to evoke pareidolia, the Pareidolia test, was administered to 53 patients with PD without dementia and 24 healthy controls. The regional cerebral metabolic rate of glucose was measured using 18F-fluorodeoxyglucose positron emission tomography in the PD patients. PD patients without dementia produced a greater number of pareidolic illusions compared with the controls. Pareidolia was observed in all of the patients having visual hallucinations as well as a subset of those without visual hallucinations. The number of pareidolic illusions was correlated with hypometabolism in the bilateral temporal, parietal and occipital cortices. The index of visual hallucinations was correlated with hypometabolism in the left parietal cortex. A region associated with both pareidolia and visual hallucinations was found in the left parietal lobe. Our study suggests that PD patients without dementia experience pareidolia more frequently than healthy controls and that posterior cortical dysfunction could be a common neural mechanism of pareidolia and visual hallucinations. Pareidolia could represent subclinical hallucinations or a predisposition to visual hallucinations in Lewy body disease. Copyright © 2015 Elsevier Ltd. All rights reserved.

  4. Impaired financial abilities in Parkinson's disease patients with mild cognitive impairment and dementia.

    PubMed

    Martin, Roy C; Triebel, Kristen L; Kennedy, Richard E; Nicholas, Anthony P; Watts, Ray L; Stover, Natividad P; Brandon, Mariko; Marson, Daniel C

    2013-11-01

    Financial capacity (FC) is an instrumental activity of daily living (IADL) critical to independent functioning and sensitive to cognitive impairment in dementia. Little is known about FC in cognitively impaired patients with Parkinson's disease (PD). The present study investigated FC in PD patients with prodromal and clinical dementia. Participants were 20 older controls and 35 PD patients who met consensus criteria for either mild cognitive impairment (PD-MCI, n = 18) or PD dementia (PDD, n = 17). FC was assessed using a standardized performance based measure consisting of 9 domain and two global scores (Financial Capacity Instrument; FCI) (1). FCI domain and global performance scores were compared across groups. Capacity impairment ratings (no impairment, mild/moderate impairment, severe impairment) were calculated for each PD patient's domain and global scores. Relative to controls, PD-MCI patients were impaired on both FCI global scores and domains of basic monetary skills, financial concepts, and investment decision-making. Relative to both controls and PD-MCI patients, PDD patients were impaired on virtually all FCI variables. With respect to impairment ratings, greater than 50% of PD-MCI patients and greater than 90% of PDD patients were classified as either mild/moderate or severely impaired on the two FCI global scores. Impairment of financial capacity is already present in PD-MCI and is advanced in PDD. Complex cognitively-mediated IADLs such as financial capacity appear to be impaired early in the course of PD dementia. Copyright © 2013 Elsevier Ltd. All rights reserved.

  5. Education-corrected CERAD identifies MCI and dementia in Parkinson's disease.

    PubMed

    Karrasch, M; Laatu, S; Ellfolk, U; Marttila, R; Martikainen, K

    2015-04-01

    This study examined whether controlling for educational background in the CERAD cognitive screening battery would affect the likelihood of patients with Parkinson's disease to fulfill criteria for mild cognitive impairment (PD-MCI) and dementia (PDD). One-hundred seventeen patients with PD were studied. Cognitive impairment was determined as two subtest scores falling below either the standard cutoff scores or education-corrected cutoff scores. The presence of dementia was determined by clinical interview or Clinical Dementia Rating. Patients were then classified as PD-MCI and PDD according to cognitive test performance and presence/absence of dementia. The number of cognitively impaired patients (PD-MCI or PDD) was significantly higher when education-controlled cutoff scores were used (62.5% vs 38%). Correspondingly, the number of false negatives (demented PD patients performing normally in CERAD) was significantly lower when education-corrected cutoff scores were used (4% vs 10%). Controlling for education increases the sensitivity of the CERAD for PD-MCI and PDD. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  6. [Impulsive-compulsive syndrome in Parkinson's disease].

    PubMed

    Nikitina, A V; Fedorova, N V

    2013-01-01

    Dopaminergic replacement therapy (DRT) is effective in treatment the motor symptoms of Parkinson's disease (PD) but can lead to impulse control disorders (ICD) in some patients. ICD include pathological gambling, hypersexuality, compulsive shopping, binge eating, punding, dopamine dysregulation syndrome (DDS). Authors studied the prevalence of ICD and its impact on the quality of life and daily activities of PD patients and their relatives. Among 246 patients studied, 55 patients (23%) (28 men, mean age 66.5±9.4 years) were diagnosed with ICD. DDS was noted in 36.4%, punding in 36.4%, binge eating in 23.6%, hypersexuality in 14.5%, compulsive shopping in 14.5% and pathological gambling in 1.8%. Of these 55 patients, 10 (18.1%) had symptoms of 2 of the ICDs: 3 (5.45%) had 3 of the ICDs and 2 (3.63%) patients had 5 of the ICDs. Quality of life ranged from 25% to 89%. Treatment approaches including the adjustment of doses of levodopa and dopamine receptor agonists in PD patients with ICD are presented.

  7. Fahr's syndrome presenting with pure and progressive presenile dementia.

    PubMed

    Modrego, P J; Mojonero, J; Serrano, M; Fayed, N

    2005-12-01

    Fahr's syndrome involves calcification of basal ganglia and dentate nuclei of the cerebellum. Clinically it may present with an array of movement disorders, dementia and other behavioural disturbances. Sporadic and familial cases have been reported with or without calcium/phosphorus metabolism. A rare form of frontotemporal dementia with neurofibrillary tangles and Fahr-type calcifications (DNTC) has been observed mainly in Japan. We report the singular case of a 50-year-old woman with progressive dementia but neither extrapyramidal symptoms nor a metabolic disorder. Brain CT showed Fahr-type calcifications in the basal ganglia, cerebellum and centrum semiovale as well as temporal atrophy; MRI showed diffuse atrophy predominantly in parietotemporal regions. The clinical and radiological features of our patient point to this uncommon form of dementia.

  8. An FP-CIT PET comparison of the differences in dopaminergic neuronal loss between idiopathic Parkinson disease with dementia and without dementia.

    PubMed

    Song, In-Uk; Kim, Young-Do; Cho, Hyun-Ji; Chung, Sung-Woo; Chung, Yong-An

    2013-01-01

    Previous studies have demonstrated a decreased density of dopamine transporters (DAT) in basal ganglia in patients with idiopathic Parkinson disease (IPD) using I-n-fluoropropyl-2b-carbomethoxy-3b-(4-iodophenyl) nortropane (FP-CIT), and the reductions in striatal DAT levels were inversely correlated with the severity of motor dysfunction in IPD. However, there has been no study on the correlation of DAT levels between IPD patients with and without cognitive dysfunction. Thus, we evaluated the differences in regional DAT density in the brain of patients with IPD without dementia and those with dementia using FP-CIT positron emission tomography. We recruited 24 consecutive patients with IPD, including 7 with IPD without dementia and 17 with IPD with dementia, and 18 healthy controls. FP-CIT positron emission tomography scans were acquired 90 and 210 minutes after the FP-CIT injection. The DAT density did not differ in the caudate nucleus or the putamen between patients with IPD without dementia and those with dementia. However, the DAT density between the 2 groups with IPD demonstrated a significantly decreased density compared with that of healthy controls in the putamen. We cautiously suggest that there is no relationship between DAT density and cognitive severity because there were no significant differences in the DAT density between IPD with dementia and those without dementia.

  9. Dementia and Mortality In Persons with Down's Syndrome

    ERIC Educational Resources Information Center

    Coppus, A.; Evenhuis, H.; Verberne, G.-J.; Visser, F.; van Gool, P.; Eikelenboom, P.; van Duijin, C.

    2006-01-01

    Background: Numerous studies have documented that persons with Downs syndrome (DS) are at an increased risk of Alzheimers disease (AD). However, at present it is still not clear whether or not all persons with DS will develop dementia as they reach old age. Methods: We studied 506 people with DS, aged 45 years and above. A standardized assessment…

  10. Diagnosing Alzheimer's Dementia in Down Syndrome: Problems and Possible Solutions

    ERIC Educational Resources Information Center

    Nieuwenhuis-Mark, Ruth E.

    2009-01-01

    It is widely accepted that people with Down syndrome are more likely than the general population to develop Alzheimer's dementia as they age. However, the diagnosis can be problematic in this population for a number of reasons. These include: the large intra-individual variability in cognitive functioning, the different diagnostic and…

  11. Dementia and Mortality In Persons with Down's Syndrome

    ERIC Educational Resources Information Center

    Coppus, A.; Evenhuis, H.; Verberne, G.-J.; Visser, F.; van Gool, P.; Eikelenboom, P.; van Duijin, C.

    2006-01-01

    Background: Numerous studies have documented that persons with Downs syndrome (DS) are at an increased risk of Alzheimers disease (AD). However, at present it is still not clear whether or not all persons with DS will develop dementia as they reach old age. Methods: We studied 506 people with DS, aged 45 years and above. A standardized assessment…

  12. Diagnosing Alzheimer's Dementia in Down Syndrome: Problems and Possible Solutions

    ERIC Educational Resources Information Center

    Nieuwenhuis-Mark, Ruth E.

    2009-01-01

    It is widely accepted that people with Down syndrome are more likely than the general population to develop Alzheimer's dementia as they age. However, the diagnosis can be problematic in this population for a number of reasons. These include: the large intra-individual variability in cognitive functioning, the different diagnostic and…

  13. CBF tomograms with (/sup 99m/Tc-HM-PAO in patients with dementia (Alzheimer type and HIV) and Parkinson's disease--initial results

    SciTech Connect

    Costa, D.C.; Ell, P.J.; Burns, A.; Philpot, M.; Levy, R.

    1988-12-01

    We present preliminary data on the utility of functional brain imaging with (99mTc)-d,l-HM-PAO and single photon emission computed tomography (SPECT) in the study of patients with dementia of the Alzheimer type (DAT), HIV-related dementia syndrome, and the on-off syndrome of Parkinson's disease. In comparison with a group of age-matched controls, the DAT patients revealed distinctive bilateral temporal and posterior parietal deficits, which correlate with detailed psychometric evaluation. Patients with amnesia as the main symptom (group A) showed bilateral mesial temporal lobe perfusion deficits (p less than 0.02). More severely affected patients (group B) with significant apraxia, aphasia, or agnosia exhibited patterns compatible with bilateral reduced perfusion in the posterior parietal cortex, as well as reduced perfusion to both temporal lobes, different from the patients of the control group (p less than 0.05). SPECT studies of HIV patients with no evidence of intracraneal space occupying pathology showed marked perfusion deficits. Patients with Parkinson's disease and the on-off syndrome studied during an on phase (under levodopa therapy) and on another occasion after withdrawal of levodopa (off) demonstrated a significant change in the uptake of (99mTc)-d,l-HM-PAO in the caudate nucleus (lower on off) and thalamus (higher on off). These findings justify the present interest in the functional evaluation of the brain of patients with dementia. (99mTc)-d,l-HM-PAO and regional cerebral blood flow (rCBF)/SPECT appear useful and highlight individual disorders of flow in a variety of neuropsychiatric conditions.

  14. Hallucinations and signs of parkinsonism help distinguish patients with dementia and cortical Lewy bodies from patients with Alzheimer's disease at presentation: a clinicopathological study.

    PubMed Central

    Ala, T A; Yang, K H; Sung, J H; Frey, W H

    1997-01-01

    OBJECTIVES: To compare, in a retrospective clinicopathological study, the presentation features of patients with dementia and cortical Lewy bodies (Lewy body dementia) with those of patients with Alzheimer's disease. METHODS: From a population of 426 cases from the dementia brain bank, 39 cases of Lewy body dementia and 61 cases of Alzheimer's disease with presentation details were identified. RESULTS: The Lewy body dementia group had significantly more frequent hallucinations (23% v 3%, P = 0.006) and signs of parkinsonism (41% v 5%, P < 0.0001) than the Alzheimer's disease group. The Lewy body dementia group also had a greater proportion of men (62% v 34%, P = 0.013). CONCLUSION: Hallucinations and signs of parkinsonism help distinguish Lewy body dementia from Alzheimer's disease at presentation. These indicators may not be very sensitive, because they were reported for less than half of the patients with Lewy body dementia. PMID:9010394

  15. Heterogeneous pathologies associated with dementia in Parkinsonism share a prion-like spreading mechanism.

    PubMed

    Candela, Serena; Giubilei, Franco; Orzi, Francesco

    2013-12-01

    Cognitive alterations accompany or follow motor disorders in subjects with Parkinsonism. The canonical phenotypeof the Parkinson's disease Dementia (PD-D) or Lewy Body Dementia (LBD) includes deficit of attention, executiveand visuospatial functions, and presents often with apathy, hallucinations, delusions, excessive daytime sleepiness,or sleep disorders. However, the clinical expression may overlap with other neurodegenerative diseases associatedwith cognitive disorders. Thus, while clinicians rely on phenomenological patterns to infer the disease causing thecognitive impairment, the inference is weakened by the heterogeneous clinical expression of the disease. In addition,recent post-mortem studies seem to undermine the supposed pathology-phenotype coherence, making it moreand more unreliable the diagnosis based on symptoms. The lack of coherence between phenotype and pathologymay support the speculation about a common mechanism underlying the progression of the disease. While it is verylikely that a distinct, specific causal event determines the disease itself, the progression might well follow commonpatterns. A number of observations suggest that progressive diseases, which cause cognitive impairment, share aprion-like mechanism. A seeding process is supposed to account for the spreading of the lesion.

  16. Rivastigmine in Alzheimer's disease and Parkinson's disease dementia: an ADAS-cog factor analysis.

    PubMed

    Weintraub, Daniel; Somogyi, Monique; Meng, Xiangyi

    2011-09-01

    Rivastigmine treatment is associated with significant improvements on the cognitive subscale of the Alzheimer's Disease Assessment Scale (ADAS-cog) in patients with mild-to-moderate Alzheimer's disease (AD) and Parkinson's disease dementia (PDD). Both AD and PDD are purported to have different profiles of cognitive impairment, which may respond differentially to rivastigmine treatment. This was a retrospective analysis of 3 randomized, double-blind, rivastigmine trial databases (Investigation of transDermal Exelon in ALzheimer's disease [IDEAL; AD], EXelon in PaRkinson's disEaSe dementia Study [EXPRESS; PDD], and Alzheimer's Disease with ENA 713 [ADENA; AD]). Factor analyses of the 11 baseline ADAS-cog items derived the same factors in the 2 diseases, that is, "memory" and "language". Rivastigmine-treated AD and PDD patients showed significant improvements (P < .0001 versus placebo) on both factors. For both AD and PDD, rivastigmine had a numerically greater effect on memory than language. Treatment effect sizes were numerically greater in PDD compared with AD. Rivastigmine treatment is associated with improvement in memory and language in AD and PDD. The numerically greater response in PDD is consistent with greater cholinergic deficits in this disease state.

  17. [Muro disease or ALS-parkinsonism-dementia complex of the Kii peninsula of Japan].

    PubMed

    Kuzuhara, Shigeki

    2007-11-01

    "Muro disease" is an endemic ALS in the Muro district that includes the southern coastal mountainous areas of the Kii peninsula of Japan. Epidemiological survey in 1960s disclosed extremely high incidence of ALS in two villages, Hohara and Kozagawa, and disappearance of high incidence by early 1980s was reported with its etiology unsolved. We resurveyed for neurodegenerative diseases in Hohara and found continuous high ALS incidence. We also found parkinsonism-dementia complex (PDC) verified neuropathologically. ALS and PDC frequently occurred in one individual simultaneously and affected many members in the same family, and neuropathological findings of ALS and PDC were similar to each other, showing a combination of upper and lower motor neuron involvements and many neurofibrillar tangles (NFTs) in the brainstem and cerebral cortex, resembling those of ALS/PDC on Guam. TDP-43 positive inclusions were found in the dentate gyrus of the hippocampus and spinal motor neurons in all cases examined. Age-adjusted incidence rates during 1950 and 2000 have showed that incidence of ALS was gradually declining for 50 years while that of PDC rose up steeply in 1990s. No particular environmental factors were confirmed and gene analyses of candidate genes of ALS, parkinsonism and dementia failed to reveal any mutations. Continuing high incidence and high rates of familial occurrence suggest that primary cause of Kii ALS/PDC may be genetic rather than environmental.

  18. The First Confirmed Case of Down Syndrome with Dementia with Lewy Bodies

    ERIC Educational Resources Information Center

    Prasher, V. P.; Airuehia, E.; Carey, M.

    2010-01-01

    Dementia with Lewy bodies (DLB) is the second commonest cause of dementia in the general population. Several researches have established an association between Down syndrome (DS) and Alzheimer's disease. Very few studies have however showed such an association between dementia with Lewy bodies and Down syndrome. The occurrence of DLB in persons…

  19. The First Confirmed Case of Down Syndrome with Dementia with Lewy Bodies

    ERIC Educational Resources Information Center

    Prasher, V. P.; Airuehia, E.; Carey, M.

    2010-01-01

    Dementia with Lewy bodies (DLB) is the second commonest cause of dementia in the general population. Several researches have established an association between Down syndrome (DS) and Alzheimer's disease. Very few studies have however showed such an association between dementia with Lewy bodies and Down syndrome. The occurrence of DLB in persons…

  20. A Randomized Study of Three Interventions for Aspiration of Thin Liquids in Patients with Dementia or Parkinson's Disease

    ERIC Educational Resources Information Center

    Logemann, Jeri A.; Gensler, Gary; Robbins, JoAnne; Lindblad, Anne S.; Brandt, Diane; Hind, Jacqueline A.; Kosek, Steven; Dikeman, Karen; Kazandjian, Marta; Gramigna, Gary D.; Lundy, Donna; McGarvey-Toler, Susan; Miller Gardner, Patricia J.

    2008-01-01

    Purpose: This study was designed to identify which of 3 treatments for aspiration on thin liquids--chin-down posture, nectar-thickened liquids, or honey-thickened liquids--results in the most successful immediate elimination of aspiration on thin liquids during the videofluorographic swallow study in patients with dementia and/or Parkinson's…

  1. A Randomized Study of Three Interventions for Aspiration of Thin Liquids in Patients with Dementia or Parkinson's Disease

    ERIC Educational Resources Information Center

    Logemann, Jeri A.; Gensler, Gary; Robbins, JoAnne; Lindblad, Anne S.; Brandt, Diane; Hind, Jacqueline A.; Kosek, Steven; Dikeman, Karen; Kazandjian, Marta; Gramigna, Gary D.; Lundy, Donna; McGarvey-Toler, Susan; Miller Gardner, Patricia J.

    2008-01-01

    Purpose: This study was designed to identify which of 3 treatments for aspiration on thin liquids--chin-down posture, nectar-thickened liquids, or honey-thickened liquids--results in the most successful immediate elimination of aspiration on thin liquids during the videofluorographic swallow study in patients with dementia and/or Parkinson's…

  2. Motoric cognitive risk syndrome: multicountry prevalence and dementia risk.

    PubMed

    Verghese, Joe; Annweiler, Cedric; Ayers, Emmeline; Barzilai, Nir; Beauchet, Olivier; Bennett, David A; Bridenbaugh, Stephanie A; Buchman, Aron S; Callisaya, Michele L; Camicioli, Richard; Capistrant, Benjamin; Chatterji, Somnath; De Cock, Anne-Marie; Ferrucci, Luigi; Giladi, Nir; Guralnik, Jack M; Hausdorff, Jeffrey M; Holtzer, Roee; Kim, Ki Woong; Kowal, Paul; Kressig, Reto W; Lim, Jae-Young; Lord, Susan; Meguro, Kenichi; Montero-Odasso, Manuel; Muir-Hunter, Susan W; Noone, Mohan L; Rochester, Lynn; Srikanth, Velandai; Wang, Cuiling

    2014-08-19

    Our objective is to report prevalence of motoric cognitive risk syndrome (MCR), a newly described predementia syndrome characterized by slow gait and cognitive complaints, in multiple countries, and its association with dementia risk. Pooled MCR prevalence analysis of individual data from 26,802 adults without dementia and disability aged 60 years and older from 22 cohorts from 17 countries. We also examined risk of incident cognitive impairment (Mini-Mental State Examination decline ≥4 points) and dementia associated with MCR in 4,812 individuals without dementia with baseline Mini-Mental State Examination scores ≥25 from 4 prospective cohort studies using Cox models adjusted for potential confounders. At baseline, 2,808 of the 26,802 participants met MCR criteria. Pooled MCR prevalence was 9.7% (95% confidence interval [CI] 8.2%-11.2%). MCR prevalence was higher with older age but there were no sex differences. MCR predicted risk of developing incident cognitive impairment in the pooled sample (adjusted hazard ratio [aHR] 2.0, 95% CI 1.7-2.4); aHRs were 1.5 to 2.7 in the individual cohorts. MCR also predicted dementia in the pooled sample (aHR 1.9, 95% CI 1.5-2.3). The results persisted even after excluding participants with possible cognitive impairment, accounting for early dementia, and diagnostic overlap with other predementia syndromes. MCR is common in older adults, and is a strong and early risk factor for cognitive decline. This clinical approach can be easily applied to identify high-risk seniors in a wide variety of settings. © 2014 American Academy of Neurology.

  3. Caregiver burden in Parkinson disease with dementia compared to Alzheimer disease in Korea.

    PubMed

    Shin, Hyeeun; Youn, Jinyoung; Kim, Ji Sun; Lee, Jun-Young; Cho, Jin Whan

    2012-12-01

    We compared caregiver burden in Parkinson disease with dementia (PDD) to that in Alzheimer disease (AD) and examined the factors contributing to the burden in PDD. Totally, 42 patients with PDD and 109 patients with AD and their caregivers participated in this study. The caregiver burden was measured using the Burden Interview (BI). Scores of Barthel activities of daily living (BADLs), Mini-Mental State Examination, Clinical Dementia Rating of patients, and score of Center for Epidemiologic Studies Depression scale, and Euro-quality of life of the caregivers were examined. The Unified Parkinson's Disease Rating Scale (UPDRS) and Hoehn and Yahr stage of the patients were administered to assess burden relating to parkinsonism on PDD. We used multiple linear regression to assess the predictors. The BI of caregivers was higher in PDD (47.9, Standard deviation [SD]: 3.8) than in AD (36.3, SD:2.1). In the AD group, the BI was predicted by cognitive function ((β±SE: -0.8±0.4, P value=04) and basic ADL status of patients (β±SE: -1.3±0.1, P<.001), depressive symptoms (β±SE: 1.1±0.1, P<.001), and poor quality of life (β±SE: -0.2±0.1, P=.017) in caregivers. In PDD group, BI was predicted only by scores of Part 1 on the UPDRS (β±SE: 2.9±1.3, P=.03) of patients and depressive symptoms (β±SE: 1.1±0.2, P<.001) of the caregivers. We concluded the caregiver burden is higher in PDD than in AD and factors predicting burden are different in AD and PDD. In patients with PDD, the neuropsychiatric problems are the major contributor to caregiver burden.

  4. Microglia, amyloid, and glucose metabolism in Parkinson's disease with and without dementia.

    PubMed

    Edison, Paul; Ahmed, Imtiaz; Fan, Zhen; Hinz, Rainer; Gelosa, Giorgio; Ray Chaudhuri, K; Walker, Zuzana; Turkheimer, Federico E; Brooks, David J

    2013-05-01

    [(11)C](R)PK11195-PET measures upregulation of translocator protein, which is associated with microglial activation, [(11)C]PIB-PET is a marker of amyloid, while [(18)F]FDG-PET measures cerebral glucose metabolism (rCMRGlc). We hypothesize that microglial activation is an early event in the Parkinson's disease (PD) spectrum and is independent of the amyloid pathology. The aim of this study is to evaluate in vivo the relationship between microglial activation, amyloid deposition, and glucose metabolism in Parkinson's disease dementia (PDD) and PD subjects without dementia. Here, we evaluated 11 PDD subjects, 8 PD subjects without dementia, and 24 control subjects. Subjects underwent T1 and T2 MRI, [(11)C](R)PK11195, [(18)F]FDG, and [(11)C]PIB PET scans. Parametric maps of [(11)C](R)PK11195 binding potential, rCMRGlc, and [(11)C]PIB uptake were interrogated using region of interest and SPM (statistical parametric mapping) analysis. The PDD patients showed a significant increase of microglial activation in anterior and posterior cingulate, striatum, frontal, temporal, parietal, and occipital cortical regions compared with the controls. The PD subjects also showed a statistically significant increase in microglial activation in temporal, parietal, and occipital regions. [(11)C]PIB uptake was marginally increased in PDD and PD. There was a significant reduction in glucose metabolism in PDD and PD. We have also demonstrated pixel-by-pixel correlation between mini-mental state examination (MMSE) score and microglial activation, and MMSE score and rCMRGlc. In conclusion, we have demonstrated that cortical microglial activation and reduced glucose metabolism can be detected early on in this disease spectrum. Significant microglial activation may be a factor in driving the disease process in PDD. Given this, agents that affect microglial activation could have an influence on disease progression.

  5. Microglia, Amyloid, and Glucose Metabolism in Parkinson's Disease with and without Dementia

    PubMed Central

    Edison, Paul; Ahmed, Imtiaz; Fan, Zhen; Hinz, Rainer; Gelosa, Giorgio; Ray Chaudhuri, K; Walker, Zuzana; Turkheimer, Federico E; Brooks, David J

    2013-01-01

    [11C](R)PK11195-PET measures upregulation of translocator protein, which is associated with microglial activation, [11C]PIB-PET is a marker of amyloid, while [18F]FDG-PET measures cerebral glucose metabolism (rCMRGlc). We hypothesize that microglial activation is an early event in the Parkinson's disease (PD) spectrum and is independent of the amyloid pathology. The aim of this study is to evaluate in vivo the relationship between microglial activation, amyloid deposition, and glucose metabolism in Parkinson's disease dementia (PDD) and PD subjects without dementia. Here, we evaluated 11 PDD subjects, 8 PD subjects without dementia, and 24 control subjects. Subjects underwent T1 and T2 MRI, [11C](R)PK11195, [18F]FDG, and [11C]PIB PET scans. Parametric maps of [11C](R)PK11195 binding potential, rCMRGlc, and [11C]PIB uptake were interrogated using region of interest and SPM (statistical parametric mapping) analysis. The PDD patients showed a significant increase of microglial activation in anterior and posterior cingulate, striatum, frontal, temporal, parietal, and occipital cortical regions compared with the controls. The PD subjects also showed a statistically significant increase in microglial activation in temporal, parietal, and occipital regions. [11C]PIB uptake was marginally increased in PDD and PD. There was a significant reduction in glucose metabolism in PDD and PD. We have also demonstrated pixel-by-pixel correlation between mini-mental state examination (MMSE) score and microglial activation, and MMSE score and rCMRGlc. In conclusion, we have demonstrated that cortical microglial activation and reduced glucose metabolism can be detected early on in this disease spectrum. Significant microglial activation may be a factor in driving the disease process in PDD. Given this, agents that affect microglial activation could have an influence on disease progression. PMID:23303049

  6. Sexuality in patients with Parkinson's disease, Alzheimer's disease, and other dementias.

    PubMed

    Bronner, Gila; Aharon-Peretz, Judith; Hassin-Baer, Sharon

    2015-01-01

    Sexual dysfunction (SD) is common among patients with Parkinson's disease (PD), Alzheimer's disease (AD), and other dementias. Sexual functioning and well-being of patients with PD and their partners are affected by many factors, including motor disabilities, non-motor symptoms (e.g., autonomic dysfunction, sleep disturbances, mood disorders, cognitive abnormalities, pain, and sensory disorders), medication effects, and relationship issues. The common sexual problems are decreased desire, erectile dysfunction, difficulties in reaching orgasm, and sexual dissatisfaction. Hypersexuality is one of a broad range of impulse control disorders reported in PD, attributed to antiparkinsonian therapy, mainly dopamine agonists. Involvement of a multidisciplinary team may enable a significant management of hypersexuality. Data on SD in demented patients are scarce, mainly reporting reduced frequency of sex and erectile dysfunction. Treatment of SD is advised at an early stage. Behavioral problems, including inappropriate sexual behavior (ISB), are distressing for patients and their caregivers and may reflect the prevailing behavior accompanying dementia (disinhibition or apathy associated with hyposexuality). The neurobiologic basis of ISB is still only vaguely understood but assessment and intervention are recommended as soon as ISB is suspected. Management of ISB in dementia demands a thorough evaluation and understanding of the behavior, and can be treated by non-pharmacologic and pharmacologic interventions.

  7. Juvenile frontotemporal dementia with parkinsonism associated with tau mutation G389R.

    PubMed

    Chaunu, Marie-Pierre; Deramecourt, Vincent; Buée-Scherrer, Valérie; Le Ber, Isabelle; Brice, Alexis; Ehrle, Nathalie; El Hachimi, Khalid; Pluot, Michel; Maurage, Claude-Alain; Bakchine, Serge; Buée, Luc

    2013-01-01

    Frontotemporal lobe degeneration includes a large spectrum of neurodegenerative disorders. Patients with frontotemporal dementia with parkinsonism linked to chromosome 17 exhibit heterogeneity in both clinical and neuropathological features. Here, we report the case of a young patient with a G389R mutation. This teenager girl was 17 years old when she progressively developed severe behavioral disturbances. First, she was considered to be suffering from atypical depression. After 2 years, she was referred to the department of neurology. By this time, the patient exhibited typical frontotemporal dementia with mild extrapyramidal disorders. The main behavioral features included apathy and reduced speech output. MRI and SPECT showed a frontotemporal atrophy and hypofixation, respectively. She died 7 years after onset. Three relatives on her father side had also died after early onset dementia. Genetic testing revealed a heterozygous guanine to cytosine mutation at the first base of codon 389 (Exon 13) of MAPT, the tau gene, resulting in a glycine to arginine substitution, in the patient and her non-affected father. Postmortem neuropathological and biochemical data indicate a Pick-like tau pathology but with phosphoserine 262-positive immunoreactivity. This case is remarkable because of the extremely early onset of the disease.

  8. Dropped head syndrome preceding the onset of dementia with Lewy bodies.

    PubMed

    Tanaka, Kanta; Wada, Ikko; Okunomiya, Taro; Shima, Atsushi; Kambe, Daisuke; Shinde, Akiyo; Kageyama, Takashi; Suenaga, Toshihiko

    2014-01-01

    A 67-year-old woman developed dropped head. Her neck was severely flexed, with prominent cervical paraspinal muscles, although no parkinsonism was observed. Brain MRI showed no significant findings. We considered dystonia as the cause of the dropped head and administered trihexyphenidyl, an anticholinergic. After 10 years of follow-up, remarkable psychotic symptoms, including hallucinations regarding insects, appeared. Following the discontinuation of trihexyphenidyl, the psychotic symptoms decreased but still remained. (123)I-N-isopropyl-p-iodoamphetamine single-photon emission computed tomography ((123)I-IMP SPECT) revealed hypoperfusion in the bilateral occipital lobes. We diagnosed the patient with dementia with Lewy bodies (DLB). This case suggests that dropped head syndrome may precede the onset of DLB.

  9. Caring for the Student with Wolff-Parkinson-White Syndrome

    ERIC Educational Resources Information Center

    Prenni, Patricia G.

    2009-01-01

    Wolff-Parkinson-White syndrome is a cardiac condition in which an extra electrical pathway within the heart causes an abnormal increase in heart rate. It affects one to three people of every 1,000 people worldwide, occurring more often in males. Diagnosis usually occurs during young adulthood, so it is important for school nurses to be familiar…

  10. Caring for the Student with Wolff-Parkinson-White Syndrome

    ERIC Educational Resources Information Center

    Prenni, Patricia G.

    2009-01-01

    Wolff-Parkinson-White syndrome is a cardiac condition in which an extra electrical pathway within the heart causes an abnormal increase in heart rate. It affects one to three people of every 1,000 people worldwide, occurring more often in males. Diagnosis usually occurs during young adulthood, so it is important for school nurses to be familiar…

  11. Vitamin D in the Parkinson Associated Risk Syndrome (PARS) study.

    PubMed

    Fullard, Michelle E; Xie, Sharon X; Marek, Ken; Stern, Matthew; Jennings, Danna; Siderowf, Andrew; Willis, Allison W; Chen-Plotkin, Alice S

    2017-09-14

    Lower vitamin D levels have been associated with manifest Parkinson's disease, prompting the hypothesis that vitamin D insufficiency or deficiency may increase risk for PD. To evaluate vitamin D levels in a population at risk for developing PD. Plasma vitamin D levels were measured in the Parkinson Associated Risk Syndrome Study, a cohort of asymptomatic individuals, some of whom are at high risk for PD. Vitamin D levels were compared between subjects at high risk for PD (hyposmia and dopamine transporter scan deficit) versus all others and examined for correlations with dopaminergic system integrity. Mean vitamin D levels did not differ between groups, with a level of 27.8 ng/mL (standard deviation = 12.0) in the high-risk group versus 24.7 ng/mL (standard deviation = 9.0) in all others (P = 0.09). Vitamin D levels did not associate with putaminal dopamine transporter uptake. Our data from the asymptomatic Parkinson Associated Risk Syndrome cohort do not support the hypothesis that chronic vitamin D insufficiency threatens dopaminergic system integrity, contributing to PD pathogenesis. © 2017 International Parkinson and Movement Disorder Society. © 2017 International Parkinson and Movement Disorder Society.

  12. Progression of white matter hyperintensities in Alzheimer disease, dementia with lewy bodies, and Parkinson disease dementia: a comparison with normal aging.

    PubMed

    Burton, Emma J; McKeith, Ian G; Burn, David J; Firbank, Michael J; O'Brien, John T

    2006-10-01

    The objective of this study was to investigate cross-sectional and longitudinal white matter hyperintensity (WMH) changes in older subjects with clinically diagnosed dementia. Fluid-attenuated inversion recovery images were acquired one year apart in subjects with dementia with Lewy bodies (DLB), Parkinson disease dementia (PDD), Alzheimer disease (AD), and also healthy elderly comparison subjects. WMH volume was quantified using an automated technique. Baseline WMH (as a percent of brain volume) was significantly greater compared with healthy subjects (N=33, geometric mean WMH: 0.4%) in subjects with AD (N=23 [1.3%], analysis of variance post hoc p <0.001) but not PDD (N=13 [0.6%]) or DLB (N=14 [0.4%]). Increase in WMH volume (as a percent of brain volume) was not significantly different (Kruskal-Wallis p=0.4) between groups (AD median change: 0.08%; DLB: 0.025%; PDD: 0.07%, healthy: 0.02%). Severity of baseline WMH, rather than diagnosis or severity of dementia, was a significant predictor of lesion progression. Rate of change of WMH had no association with change in global cognitive performance. Significant WMH progression occurs in degenerative dementias with rates influenced by severity of lesions at baseline rather than dementia type or cognitive decline.

  13. Can loss of the swallow tail sign help distinguish between Parkinson Disease and the Parkinson-Plus syndromes?

    PubMed

    Oustwani, Christopher Sami; Korutz, Alexander William; Lester, Malisa Siri; Kianirad, Yasaman; Simuni, Tanya; Hijaz, Tarek Aref

    To determine if loss of the swallow tail sign (STS) can distinguish Parkinson Disease (PD) from the Parkinson-Plus syndromes. Twenty-five patients with PD, 21 with Parkinson-Plus syndromes, and 14 control patients were included. Presence of the STS was assessed. The STS was present in 79% of controls, statistically greater than the PD/Parkinson-Plus patients. There was no difference in the presence of the STS between the PD/Parkinson-Plus subgroups or when scanning at 1.5 T or 3 T. Loss of the STS could not distinguish between PD and Parkinson-Plus patients. The STS can be identified at both 1.5 T and 3 T. Copyright © 2017 Elsevier Inc. All rights reserved.

  14. Sundowning syndrome in aging and dementia: research in mouse models.

    PubMed

    Bedrosian, Tracy A; Nelson, Randy J

    2013-05-01

    Both normal aging and dementia are associated with altered circadian regulation of physiology and behavior. Elderly individuals and dementia patients commonly experience disrupted sleep-wake cycles, which may lead to psychomotor agitation, confusion, and wandering. These behaviors are disruptive to both patients and caregivers. Sundowning syndrome, which encompasses many of these behaviors, is characterized by a temporal pattern in the severity of symptoms, usually expressed as worse during the late afternoon or evening. Other than antipsychotic medications, off-label medications, and restraint, few treatment options are available. The aim of this paper is to review mouse studies of circadian behavioral disturbances relevant to sundowning, in order to determine potential models for studying the mechanisms of sundowning syndrome. The emergence of a useful mouse model should facilitate the development of novel therapeutic approaches. Copyright © 2012 Elsevier Inc. All rights reserved.

  15. Genome-wide analysis of genetic correlation in dementia with Lewy bodies, Parkinson's and Alzheimer's diseases.

    PubMed

    Guerreiro, Rita; Escott-Price, Valentina; Darwent, Lee; Parkkinen, Laura; Ansorge, Olaf; Hernandez, Dena G; Nalls, Michael A; Clark, Lorraine; Honig, Lawrence; Marder, Karen; van der Flier, Wiesje; Holstege, Henne; Louwersheimer, Eva; Lemstra, Afina; Scheltens, Philip; Rogaeva, Ekaterina; St George-Hyslop, Peter; Londos, Elisabet; Zetterberg, Henrik; Ortega-Cubero, Sara; Pastor, Pau; Ferman, Tanis J; Graff-Radford, Neill R; Ross, Owen A; Barber, Imelda; Braae, Anne; Brown, Kristelle; Morgan, Kevin; Maetzler, Walter; Berg, Daniela; Troakes, Claire; Al-Sarraj, Safa; Lashley, Tammaryn; Compta, Yaroslau; Revesz, Tamas; Lees, Andrew; Cairns, Nigel J; Halliday, Glenda M; Mann, David; Pickering-Brown, Stuart; Powell, John; Lunnon, Katie; Lupton, Michelle K; Dickson, Dennis; Hardy, John; Singleton, Andrew; Bras, Jose

    2016-02-01

    The similarities between dementia with Lewy bodies (DLB) and both Parkinson's disease (PD) and Alzheimer's disease (AD) are many and range from clinical presentation, to neuropathological characteristics, to more recently identified, genetic determinants of risk. Because of these overlapping features, diagnosing DLB is challenging and has clinical implications since some therapeutic agents that are applicable in other diseases have adverse effects in DLB. Having shown that DLB shares some genetic risk with PD and AD, we have now quantified the amount of sharing through the application of genetic correlation estimates, and show that, from a purely genetic perspective, and excluding the strong association at the APOE locus, DLB is equally correlated to AD and PD.

  16. [Diagnostic Criteria for Amyotrophic Lateral Sclerosis/Parkinsonism-Dementia Complex in the Kii Peninsula, Japan].

    PubMed

    Kokubo, Yasumasa

    2015-07-01

    The diagnostic criteria for amyotrophic lateral sclerosis/parkinsonism-dementia complex in the Kii peninsula of Japan (Kii ALS/PDC) have been proposed. Muro disease has been considered an endemic neurodegenerative disease in the southern part of the Kii peninsula. Recent intensive and comprehensive research has revealed it to be a complex and genetically heterogeneous disease. At present, there are four subtypes of Muro disease: sporadic amyotrophic lateral sclerosis (ALS), Kii ALS/PDC with tauopathy, ALS with C9orf72 gene mutation, and ALS with optineurin gene mutation. The present criteria are applicable to only one of these subtypes, Kii ALS/PDC with tauopathy, which is quite similar to ALS/PDC in Guam.

  17. Genome-wide analysis of genetic correlation in dementia with Lewy bodies, Parkinson's and Alzheimer's diseases

    PubMed Central

    Guerreiro, Rita; Escott-Price, Valentina; Darwent, Lee; Parkkinen, Laura; Ansorge, Olaf; Hernandez, Dena G.; Nalls, Michael A.; Clark, Lorraine; Honig, Lawrence; Marder, Karen; van der Flier, Wiesje; Holstege, Henne; Louwersheimer, Eva; Lemstra, Afina; Scheltens, Philip; Rogaeva, Ekaterina; St George-Hyslop, Peter; Londos, Elisabet; Zetterberg, Henrik; Ortega-Cubero, Sara; Pastor, Pau; Ferman, Tanis J.; Graff-Radford, Neill R.; Ross, Owen A.; Barber, Imelda; Braae, Anne; Brown, Kristelle; Morgan, Kevin; Maetzler, Walter; Berg, Daniela; Troakes, Claire; Al-Sarraj, Safa; Lashley, Tammaryn; Compta, Yaroslau; Revesz, Tamas; Lees, Andrew; Cairns, Nigel J.; Halliday, Glenda M.; Mann, David; Pickering-Brown, Stuart; Powell, John; Lunnon, Katie; Lupton, Michelle K.; Dickson, Dennis; Hardy, John; Singleton, Andrew; Bras, Jose

    2016-01-01

    The similarities between dementia with Lewy bodies (DLB) and both Parkinson's disease (PD) and Alzheimer's disease (AD) are many and range from clinical presentation, to neuropathological characteristics, to more recently identified, genetic determinants of risk. Because of these overlapping features, diagnosing DLB is challenging and has clinical implications since some therapeutic agents that are applicable in other diseases have adverse effects in DLB. Having shown that DLB shares some genetic risk with PD and AD, we have now quantified the amount of sharing through the application of genetic correlation estimates, and show that, from a purely genetic perspective, and excluding the strong association at the APOE locus, DLB is equally correlated to AD and PD. PMID:26643944

  18. Imaging prodromal Parkinson disease: the Parkinson Associated Risk Syndrome Study.

    PubMed

    Jennings, Danna; Siderowf, Andrew; Stern, Matthew; Seibyl, John; Eberly, Shirley; Oakes, David; Marek, Kenneth

    2014-11-04

    The purpose of this study is to evaluate the relative risk of abnormal dopamine transporter (DAT) imaging for subjects with and without hyposmia and the feasibility of acquiring a large, community-based, 2-tiered biomarker assessment strategy to detect prodromal Parkinson disease (PD). In this observational study, individuals without a diagnosis of PD, recruited through 16 movement disorder clinics, underwent tier 1 assessments (olfactory testing, questionnaires). Tier 2 assessments (neurologic examination, DAT imaging, and other biomarker assessments) were completed by 303 subjects. The main outcome of the study is to compare age-expected [(123)I]β-CIT striatal binding ratio in hyposmic and normosmic subjects. Tier 1 assessments were mailed to 9,398 eligible subjects and returned by 4,999; 669 were hyposmic. Three hundred three subjects (203 hyposmic, 100 normosmic) completed baseline evaluations. DAT deficit was present in 11% of hyposmic subjects compared with 1% of normosmic subjects. Multiple logistic regression demonstrates hyposmia (odds ratio [OR] 12.4; 95% confidence interval [CI] 1.6, 96.1), male sex (OR 5.5; 95% CI 1.7, 17.2), and constipation (OR 4.3; 95% CI 1.6, 11.6) as factors predictive of DAT deficit. Combining multiple factors (hyposmia, male sex, and constipation) increased the percentage of subjects with a DAT deficit to >40%. Subjects with DAT deficit who do not meet criteria for a diagnosis of PD can be identified by olfactory testing. Sequential biomarker assessment may identify those at risk of PD. Selecting hyposmic individuals enriches the population for DAT deficit, and combining hyposmia with other potential risk factors (male sex, constipation) increases the percentage of subjects with a DAT deficit compatible with prodromal PD. © 2014 American Academy of Neurology.

  19. Tau pathology involves protein phosphatase 2A in Parkinsonism-dementia of Guam

    PubMed Central

    Arif, Mohammad; Kazim, Syed Faraz; Grundke-Iqbal, Inge; Garruto, Ralph M.; Iqbal, Khalid

    2014-01-01

    Parkinsonism-dementia (PD) of Guam is a neurodegenerative disease with parkinsonism and early-onset Alzheimer-like dementia associated with neurofibrillary tangles composed of hyperphosphorylated microtubule-associated protein, tau. β-N-methylamino-l-alanine (BMAA) has been suspected of being involved in the etiology of PD, but the mechanism by which BMAA leads to tau hyperphosphorylation is not known. We found a decrease in protein phosphatase 2A (PP2A) activity associated with an increase in inhibitory phosphorylation of its catalytic subunit PP2Ac at Tyr307 and abnormal hyperphosphorylation of tau in brains of patients who had Guam PD. To test the possible involvement of BMAA in the etiopathogenesis of PD, we studied the effect of this environmental neurotoxin on PP2A activity and tau hyperphosphorylation in mouse primary neuronal cultures and metabolically active rat brain slices. BMAA treatment significantly decreased PP2A activity, with a concomitant increase in tau kinase activity resulting in elevated tau hyperphosphorylation at PP2A favorable sites. Moreover, we found an increase in the phosphorylation of PP2Ac at Tyr307 in BMAA-treated rat brains. Pretreatment with metabotropic glutamate receptor 5 (mGluR5) and Src antagonists blocked the BMAA-induced inhibition of PP2A and the abnormal hyperphosphorylation of tau, indicating the involvement of an Src-dependent PP2A pathway. Coimmunoprecipitation experiments showed that BMAA treatment dissociated PP2Ac from mGluR5, making it available for phosphorylation at Tyr307. These findings suggest a scenario in which BMAA can lead to tau pathology by inhibiting PP2A through the activation of mGluR5, the consequent release of PP2Ac from the mGluR5–PP2A complex, and its phosphorylation at Tyr307 by Src. PMID:24395787

  20. Hippocampal head atrophy predominance in Parkinson's disease with hallucinations and with dementia.

    PubMed

    Ibarretxe-Bilbao, Naroa; Ramírez-Ruiz, Blanca; Tolosa, Eduardo; Martí, María Jose; Valldeoriola, Francesc; Bargalló, Nuria; Junqué, Carme

    2008-09-01

    We studied regional gray matter density in the hippocampus in Parkinson's disease (PD) patients. We obtained magnetic resonance scans in 44 PD patients (PD patients with dementia (PDD) = 9, non-demented PD patients with visual hallucinations (PD + VH) = 16, and PD patients without dementia and without visual hallucinations (PD - VH) = 19) and 56 controls matched for age and years of education. A region of interest (ROI) of the hippocampus following voxel-based morphometry (VBM) procedures was used to perform group comparisons, single-case individual analysis and correlations with learning scores. Group comparisons showed that PDD patients and PD+VH patients had significant hippocampal gray matter loss compared to controls. In PDD patients, hippocampal gray matter loss involved the entire hippocampus and in PD+VH this reduction was mainly confined to the hippocampal head. 78 % of PDD patients, 31 % of PD+VH patients and 26 % of PD-VH patients had hippocampal head gray matter loss when compared to controls. These results suggest that in PD the neurodegenerative process in the hippocampus starts in the head of this structure and later spreads to the tail and that, in addition, memory impairment assessed by Rey's Auditory Verbal Learning Test (RAVLT) correlates with hippocampal head gray matter loss.

  1. Neuropsychiatric Symptoms in Parkinson's Disease with Mild Cognitive Impairment and Dementia

    PubMed Central

    Leroi, Iracema; Pantula, Hiranmayi; McDonald, Kathryn; Harbishettar, Vijay

    2012-01-01

    Neuropsychiatric symptoms commonly complicate Parkinson's disease (PD), however the presence of such symptoms in mild cognitive impairment (PD-MCI) specifically has not yet been well described. The objective of this study was to examine and compare the prevalence and profile of neuropsychiatric symptoms in patients with PD-MCI (n = 48) to those with PD and no cognitive impairment (PD-NC, n = 54) and to those with dementia in PD (PDD, n = 25). PD-MCI and PDD were defined using specific consensus criteria, and neuropsychiatric symptoms were assessed with the 12-item Neuropsychiatric Inventory (NPI). Self-rated apathy, depression, and anxiety rating scales were also administered. Over 79% of all participants reported at least one neuropsychiatric symptom in the past month. The proportion in each group who had total NPI scores of ≥4 (“clinically significant”) was as follows: PD-NC, 64.8%; PD-MCI, 62%; PDD 76%. Apathy was reported in almost 50% of those with PD-MCI and PDD, and it was an important neuropsychiatric symptom differentiating PD-MCI from PD-NC. Psychosis (hallucinations and delusions) increased from 12.9% in PD-NC group; 16.7% in PD-MCI group; and 48% in PDD group. Identifying neuropsychiatric symptoms in PD-MCI may have implications for ascertaining conversion to dementia in PD. PMID:22970412

  2. Alpha- and beta-synuclein expression in Parkinson disease with and without dementia.

    PubMed

    Beyer, Katrin; Ispierto, Lourdes; Latorre, Pilar; Tolosa, Eduardo; Ariza, Aurelio

    2011-11-15

    Parkinson disease (PD) is the most important movement disorder and about 50% of patients develop dementia over the time. PD belongs to the group of Lewy body disorders. Alpha-synuclein (AS) is the main component of Lewy bodies and its aggregation is a key event in the pathogenesis of PD. Beta-synuclein (BS) inhibits AS aggregation in vitro and in vivo and has been shown to interact directly with AS regulating its functionality and preventing its oligomerization. Recently, we have described a molecular subgroup of DLB characterized by the drastic BS reduction in cortical areas. In this study we have analyzed the expression of two BS transcripts and the main AS transcript SNCA140, in frozen samples of three brain areas, temporal cortex, caudate nucleus and pons, from patients with PD and PDD in comparison with controls. Relative mRNA expression was determined by real-time PCR with SybrGreen, neuron-specific-enolase as housekeeping gene and the deltadeltaCt method. The most important difference in BS and AS mRNA expression between PD and PDD was found in the caudate nucleus, where BS mRNA was overexpressed in PD and AS mRNA diminished in PDD. Our findings provide new insights into the pathogenesis of dementia in PD, indicating that differential BS and AS expression in the caudate nucleus may represent one of the molecular mechanisms involved in these complex diseases.

  3. Prevalence of Amyloid PET Positivity in Dementia Syndromes

    PubMed Central

    Ossenkoppele, Rik; Jansen, Willemijn J.; Rabinovici, Gil D.; Knol, Dirk L.; van der Flier, Wiesje M.; van Berckel, Bart N. M.; Scheltens, Philip; Visser, Pieter Jelle

    2015-01-01

    IMPORTANCE Amyloid-β positron emission tomography (PET) imaging allows in vivo detection of fibrillar plaques, a core neuropathological feature of Alzheimer disease (AD). Its diagnostic utility is still unclear because amyloid plaques also occur in patients with non–AD dementia. OBJECTIVE To use individual participant data meta-analysis to estimate the prevalence of amyloid positivity on PET in a wide variety of dementia syndromes. DATA SOURCES The MEDLINE and Web of Science databases were searched from January 2004 to April 2015 for amyloid PET studies. STUDY SELECTION Case reports and studies on neurological or psychiatric diseases other than dementia were excluded. Corresponding authors of eligible cohorts were invited to provide individual participant data. DATA EXTRACTION AND SYNTHESIS Data were provided for 1359 participants with clinically diagnosed AD and 538 participants with non–AD dementia. The reference groups were 1849 healthy control participants (based on amyloid PET) and an independent sample of 1369 AD participants (based on autopsy). MAIN OUTCOMES AND MEASURES Estimated prevalence of positive amyloid PET scans according to diagnosis, age, and apolipoprotein E (APOE) ε4 status, using the generalized estimating equations method. RESULTS The likelihood of amyloid positivity was associated with age and APOE ε4 status. In AD dementia, the prevalence of amyloid positivity decreased from age 50 to 90 years in APOE ε4 noncarriers(86%[95%CI,73%–94%]at 50 years to 68% [95% CI,57%–77%] at 90 years; n = 377) and to a lesser degree in APOE ε4 carriers (97% [95% CI, 92%–99%] at 50 years to 90% [95% CI, 83%–94%] at 90 years; n = 593; P < .01). Similar associations of age and APOE ε4 with amyloid positivity were observed in participants with AD dementia at autopsy. In most non–AD dementias, amyloid positivity increased with both age (from 60 to 80 years) and APOE ε4 carriership. Total ParticipantsAmyloid Positivity, % (95% CI) Age 60 yAge 80

  4. Symptoms of Dementia among Adults with Down's Syndrome: A Qualitative Study

    ERIC Educational Resources Information Center

    Deb, Shoumitro; Hare, M.; Prior, L.

    2007-01-01

    Background: Dementia is common among adults with Down's syndrome (DS); yet the diagnosis of dementia, particularly in its early stage, can be difficult in this population. One possible reason for this may be the different clinical manifestation of dementia among people with intellectual disabilities. Aims: The aim of this study was to map out the…

  5. Maladaptive Behaviors Related to Dementia Status in Adults with Down Syndrome

    ERIC Educational Resources Information Center

    Urv, Tiina K.; Zigman, Warren B.; Silverman, Wayne

    2008-01-01

    Changes in maladaptive behaviors related to specific stages of dementia were investigated in 251 adults 45 years of age and older with Down syndrome. Findings indicate clear differences in maladaptive behaviors at various stages of dementia. Generally, individuals with no signs or symptoms of dementia displayed fewer and less severe maladaptive…

  6. Prospective Study of the Prevalence of Alzheimer-Type Dementia in Institutionalized Individuals with Down Syndrome.

    ERIC Educational Resources Information Center

    Visser, F. E.; And Others

    1997-01-01

    Institutionalized patients with Down syndrome (N=307) were monitored for 5 to 10 years to determine prevalence of Alzheimer-type dementia. Prevalence increased from 11% between ages 40 and 49 to 77% between 60 and 69. All patients 70 and over had dementia. Mean age of onset of dementia was 56 years. Neuropathological findings were consistent with…

  7. Maladaptive Behaviors Related to Dementia Status in Adults with Down Syndrome

    ERIC Educational Resources Information Center

    Urv, Tiina K.; Zigman, Warren B.; Silverman, Wayne

    2008-01-01

    Changes in maladaptive behaviors related to specific stages of dementia were investigated in 251 adults 45 years of age and older with Down syndrome. Findings indicate clear differences in maladaptive behaviors at various stages of dementia. Generally, individuals with no signs or symptoms of dementia displayed fewer and less severe maladaptive…

  8. Symptoms of Dementia among Adults with Down's Syndrome: A Qualitative Study

    ERIC Educational Resources Information Center

    Deb, Shoumitro; Hare, M.; Prior, L.

    2007-01-01

    Background: Dementia is common among adults with Down's syndrome (DS); yet the diagnosis of dementia, particularly in its early stage, can be difficult in this population. One possible reason for this may be the different clinical manifestation of dementia among people with intellectual disabilities. Aims: The aim of this study was to map out the…

  9. Subcortical grey matter changes in untreated, early stage Parkinson's disease without dementia.

    PubMed

    Lee, Hye Mi; Kwon, Kyum-Yil; Kim, Min-Jik; Jang, Ji-Wan; Suh, Sang-Il; Koh, Seong-Beom; Kim, Ji Hyun

    2014-06-01

    Previous MRI studies have investigated cortical or subcortical grey matter changes in patients with Parkinson's disease (PD), yielding inconsistent findings between the studies. We therefore sought to determine whether focal cortical or subcortical grey matter changes may be present from the early disease stage. We recruited 49 untreated, early stage PD patients without dementia and 53 control subjects. Voxel-based morphometry was used to evaluate cortical grey matter changes, and automated volumetry and shape analysis were used to assess volume changes and shape deformation of the subcortical grey matter structures, respectively. Voxel-based morphometry showed neither reductions nor increases in grey matter volume in patients compared to controls. Compared to controls, PD patients had significant reductions in adjusted volumes of putamen, nucleus accumbens, and hippocampus (corrected p < 0.05). Vertex-based shape analysis showed regionally contracted area on the posterolateral and ventromedial putamen bilaterally in PD patients (corrected p < 0.05). No correlations were found between cortical and subcortical grey matter and clinical variables representing disease duration and severity. Our results suggest that untreated, early stage PD without dementia is associated with volume reduction and shape deformation of subcortical grey matter, but not with cortical grey matter reduction. Our findings of structural changes in the posterolateral putamen and ventromedial putamen/nucleus accumbens could provide neuroanatomical basis for the involvement of motor and limbic striatum, further implicating motor and non-motor symptoms in PD, respectively. Early hippocampal involvement might be related to the risk for developing dementia in PD patients. Copyright © 2014 Elsevier Ltd. All rights reserved.

  10. Burning mouth syndrome in Parkinson's disease: dopamine as cure or cause?

    PubMed

    Coon, Elizabeth A; Laughlin, Ruple S

    2012-04-01

    Burning mouth syndrome has been reported as being more common in Parkinson's disease patients than the general population. While the pathophysiology is unclear, decreased dopamine levels and dopamine dysregulation are hypothesized to play a role. We report a patient with Parkinson's disease who developed burning mouth syndrome with carbidopa/levodopa. Our patient had resolution of burning mouth symptoms when carbidopa/levodopa was replaced with a dopamine agonist. Based on our patient's clinical course, in conjunction with earlier studies assessing the relationship between burning mouth syndrome and Parkinson's disease, we discuss a potential role for dopamine in burning mouth syndrome in Parkinson's disease.

  11. A comparison of caregiver burden in older persons and persons with Parkinson's disease or dementia in sub-Saharan Africa.

    PubMed

    Dotchin, C L; Paddick, S-M; Longdon, A R; Kisoli, A; Gray, W K; Dewhurst, F; Chaote, P; Dewhurst, M; Walker, R W

    2014-04-01

    Caregiver burden includes the many physical, mental and socio-economic problems arising from caring for individuals with chronic and disabling diseases. Being a carer in sub-Saharan Africa (SSA), where little is known about chronic neurological conditions, may be extremely demanding. Conversely, multigenerational living may allow sharing of care among many caregivers. We wished to determine the relative burden of caring for two chronic neurodegenerative conditions (Parkinson's disease (PD) and dementia) in rural Tanzania. All surviving patients from a PD prevalence study, newly identified people with PD from a neurological disorders study and all people with dementia from a dementia prevalence study in Hai, rural Tanzania, were invited to participate. The Zarit Burden Interview (ZBI) was used to determine level of caregiver strain (higher score reflects more strain). Of 25 PD patients ZBI was recorded in 20 (14 male). Five had no identifiable carer as they were largely independent. Three had PD dementia (PDD). Of 75 people with dementia (excluding 3 PDD), 43 (32 female) completed the ZBI. For the other 32, the caregivers felt the care they provided was a normal intergenerational expectation. Median ages were 78.5 and 85 years for PD and dementia, respectively. Median ZBI was 30.5 for PD and 14 for dementia (U = 166.0, z = -3.913, p < 0.001). Disease duration and disease type (PD or dementia) were univariate predictor of ZBI score, although only disease type was predictive by multivariable linear regression. Caring for an individual with PD may be more burdensome than caring for an individual with dementia in SSA. People with more advanced PD had higher caregiver burden.

  12. The Bee Sting Related Wolff-Parkinson-White Syndrome

    PubMed Central

    Santhosh M., Shanmuga Ravi; Viswanathan, Stalin; Kumar, Shanthi

    2012-01-01

    Hymenoptera stings are common reasons for emergency visits. The admissions for the hymenoptera stings occur for systemic or unusual reactions. We are reporting a man with multiple bee stings, who presented with dizziness and palpitations and was found to have ECG findings of the Wolff-Parkinson-White syndrome. He had no worsening of symptoms or new ECG changes during his hospitalization. The hymenoptera related cardiac effects have also been reviewed and summarized. PMID:23285451

  13. Alpha-synuclein in familial Alzheimer disease: epitope mapping parallels dementia with Lewy bodies and Parkinson disease.

    PubMed

    Lippa, C F; Schmidt, M L; Lee, V M; Trojanowski, J Q

    2001-11-01

    Alpha-synuclein is a major component of Lewy bodies (LBs) in Parkinson disease and dementia with LBs and of glial cytoplasmic inclusions in multiple system atrophy. However, epitope mapping for alpha-synuclein is distinctive in different neurodegenerative diseases. The reasons for this are poorly understood but may reflect fundamental differences in disease mechanisms. To investigate the alpha-synuclein epitope mapping properties of LBs in familial Alzheimer disease. We compared LBs in familial Alzheimer disease with those in synucleinopathies by probing 6 brains of persons with familial Alzheimer disease using a panel of antibodies to epitopes spanning the alpha-synuclein protein. Results were compared with data from brains of persons with Parkinson disease, dementia with LBs, and multiple system atrophy. The brains of persons with familial Alzheimer disease showed consistent staining of LBs with all antibodies, similar to Parkinson disease and dementia with LBs but different from alpha-synuclein aggregates that occurred in multiple system atrophy. These data suggest that the epitope profiles of alpha-synuclein in LBs are similar, regardless of whether the biological trigger is related to synuclein or a different genetic pathway. These findings support the hypothesis that the mechanism of alpha-synuclein aggregation is the same within cell types but distinctive between cell types.

  14. Amantadine for executive dysfunction syndrome in patients with dementia.

    PubMed

    Drayton, Shannon J; Davies, Kendra; Steinberg, Martin; Leroi, Iracema; Rosenblatt, Adam; Lyketsos, Constantine G

    2004-01-01

    This article reports the results of an open uncontrolled chart review study of amantadine treatment for executive dysfunction syndrome in patients with dementia. All patients admitted to the neuropsychiatry or geriatric psychiatry inpatient units of Johns Hopkins Hospital in 2000 and 2001 who were treated empirically with amantadine for executive dysfunction syndrome were included in the review. Of the 30 patients whose cases were reviewed, 17 (57%) were at least "much improved," and most patients were discharged taking amantadine, suggesting that their physicians believed that they may have benefited from it. The medication was well tolerated in this frail group of patients. Most patients were taking one or more concurrent psychotropic medications, which may have contributed to the positive outcomes. Despite its limitations, this study offers preliminary data to support a controlled trial of amantadine in patients with executive dysfunction syndrome.

  15. Parkinson's Disease: Is It a Toxic Syndrome?

    PubMed Central

    Gad ELhak, Seham A.; Ghanem, Abdel Aziz A.; AbdelGhaffar, Hassan; El Dakroury, Sahar; Salama, Mohamed M.

    2010-01-01

    Parkinson's disease (PD) is one of the neurodegenerative diseases which we can by certainty identify its pathology, however, this confidence disappeares when talking about the cause. A long history of trials, suggestions, and theories tried linking PD to a specific causation. In this paper, a new suggestion is trying to find its way, could it be toxicology? Can we—in the future—look to PD as an occupational disease, in fact, many clues point to the possible toxic responsibility—either total or partial—in causing this disease. Searching for possible toxic causes for PD would help in designing perfect toxic models in animals. PMID:21152209

  16. Association of restless legs syndrome, pain, and mood disorders in Parkinson's disease.

    PubMed

    Rana, Abdul Qayyum; Qureshi, Abdul Rehman M; Rahman, Labiba; Jesudasan, Ajantha; Hafez, Kevin K; Rana, Mohammad A

    2016-01-01

    The objectives of the study were to analyze the association between Parkinson's disease and restless legs syndrome, and to explore the relationship between mood disorder comorbidity (anxiety and depression), pain, and restless legs syndrome. This study included 123 Parkinson's disease patients and 123 non-Parkinson's disease patients matched for age and gender, and evaluated for anxiety severity, depression severity, pain severity, pain interference, pain disability, and restless legs syndrome prevalence. This was performed using semi-structured interviews and a neurological examination through the restless legs syndrome diagnostic criteria and the following inventories; Hospital Anxiety and Depression Scale, Brief Pain Inventory, and Pain Disability Index. Parkinson's disease patients had significantly greater anxiety severity, depression severity, pain severity, pain interference, pain disability, and restless legs syndrome prevalence in comparison to controls. In addition, Parkinson's disease patients' comorbid for anxiety and depression had significantly greater pain severity, pain interference, and pain disability, but not RLS prevalence, in comparison to Parkinson's disease only, Parkinson's disease anxiety, and Parkinson's disease depression patients. Pain interference, pain severity, and pain disability is greater among Parkinson's disease patients with anxiety and depression, in comparison to Parkinson's disease patients without anxiety and depression. On the contrary, the prevalence of restless legs syndrome was not found to be relevant.

  17. Psychosis in Parkinson's disease without dementia: common and comorbid with other non-motor symptoms.

    PubMed

    Lee, Angela H; Weintraub, Daniel

    2012-06-01

    Psychosis in Parkinson's disease (PD) is common and associated with a range of negative outcomes. Dementia and psychosis are highly correlated in PD, but the frequency and correlates of psychosis in patients without cognitive impairment are not well understood. One hundred and ninety-one non-demented PD patients at two movement disorders centers participated in a study of neuropsychiatric complications in PD and completed a detailed neurological and neuropsychiatric assessment, including the rater-administered Parkinson Psychosis Rating Scale for hallucinations, delusions, and minor symptoms of psychosis (illusions and misidentification of persons). Psychotic symptoms were present in 21.5% of the sample. Visual hallucinations were most common (13.6%), followed by auditory hallucinations (6.8%), illusions or misidentification of people (7.3%), and paranoid ideation (4.7%). Visual hallucinations and illusions or misidentification of people were the most common comorbid symptoms (3.1%). Depression (P = 0.01) and rapid eye movement behavior disorder symptoms (P = 0.03) were associated with psychosis in a multivariable model. The odds of experiencing psychotic symptoms were approximately five times higher in patients with comorbid disorders of depression and sleep-wakefulness. Even in patients without global cognitive impairment, psychosis in PD is common and most highly correlated with other non-motor symptoms. Screening for psychosis should occur at all stages of PD as part of a broad non-motor assessment. In addition, these findings suggest a common neural substrate for disturbances of perception, mood, sleep-wakefulness, and incipient cognitive decline in PD. Copyright © 2012 Movement Disorder Society.

  18. A familial form of parkinsonism, dementia, and motor neuron disease: a longitudinal study

    PubMed Central

    Fujioka, Shinsuke; Boeve, Bradley F.; Parisi, Joseph E.; Tacik, Pawel; Aoki, Naoya; Strongosky, Audrey J.; Baker, Matt; Ross, Owen A.; Rademakers, Rosa; Sossi, Vesna; Dickson, Dennis W.; Stoessl, A. Jon; Wszolek, Zbigniew K.

    2014-01-01

    Objective To describe clinical, positron emission tomography (PET), pathological, and genetic findings of a large kindred with progressive neurodegenerative phenotypes in which the proband had autopsy-confirmed corticobasal degeneration (CBD). Methods Five family members, including the proband, were examined neurologically. Clinical information from the other family members was collected by questionnaires. Three individuals underwent PET with 11C-dihydrotetrabenazine and 18F-fludeoxyglucose. The proband was examined post-mortem. Genetic studies were performed. Results The pedigree contains 64 individuals, including 8 affected patients. The inheritance is likely autosomal dominant with reduced penetrance. The proband developed progressive speech and language difficulties at the age of 64 years. Upon examination at the age of 68 years, she showed non-fluent aphasia, word-finding difficulties, circumlocution, frontal release signs, and right-sided bradykinesia, rigidity, and pyramidal signs. She died 5 years after disease onset. The neuropathology was consistent with CBD, including many cortical and subcortical astrocytic plaques. Other family members had progressive neurodegenerative phenotypes – two were diagnosed with parkinsonism and behavioral problems, two with parkinsonism alone, one with amyotrophic lateral sclerosis alone, one with dementia, and one with progressive gait and speech problems. PET on three potentially affected individuals showed no significant pathology. Genetic sequencing of DNA from the proband excluded mutations in known neurodegenerative-related genes including MAPT, PGRN, LRRK2, and C9ORF72. Conclusions Families with such complex phenotypes rarely occur. They are usually associated with MAPT mutations; however, in this family, MAPT mutations have been excluded, implicating another causative gene or genes. Further genetic studies on this family may eventually disclose the etiology. PMID:25175602

  19. Naturally Occurring Autoantibodies against Tau Protein Are Reduced in Parkinson's Disease Dementia

    PubMed Central

    Kronimus, Yannick; Albus, Alexandra; Balzer-Geldsetzer, Monika; Straub, Sarah; Semler, Elisa; Otto, Markus; Klotsche, Jens; Dodel, Richard; Mengel, David

    2016-01-01

    Background and Objective Altered levels of naturally occurring autoantibodies (nAbs) against disease-associated neuronal proteins have been reported for neurodegenerative diseases, such as Alzheimer's (AD) and Parkinson's disease (PD). Recent histopathologic studies suggest a contribution of both Lewy body- and AD-related pathology to Parkinson's disease dementia (PDD). Therefore, we explored nAbs against alpha-synuclein (αS), tau and β-amyloid (Aβ) in PDD compared to cognitively normal PD patients. Materials and Methods We established three different ELISAs to quantify the nAbs-tau, nAbs-αS, and nAbs-Aβ levels and avidity towards their specific antigen in serum samples of 18 non-demented (PDND) and 18 demented PD patients (PDD), which were taken from an ongoing multi-center cohort study (DEMPARK/LANDSCAPE). Results PDD patients had significantly decreased nAbs-tau serum levels compared to PDND patients (p = 0.007), whereas the serum titers of nAbs-αS and nAbs-Aβ were unchanged. For all three nAbs, no significant differences in avidity were found between PDD and PDND cohorts. However, within both patient groups, nAbs-tau showed lowest avidity to their antigen, followed by nAbs-αS, and nAbs-Aβ. Though, due to a high interassay coefficient of variability and the exclusion of many samples below the limit of detection, conclusions for nAbs-Aβ are only conditionally possible. Conclusion We detected a significantly decreased nAbs-tau serum level in PDD patients, indicating a potential linkage between nAbs-tau serum titer and cognitive deficits in PD. Thus, further investigation in larger samples is justified to confirm our findings. PMID:27802290

  20. PDD-5S: A useful screening tool for Parkinson's disease dementia.

    PubMed

    Lee, Wei-Ju; Wu, Shey-Lin; Li, Jie-Yuan; Chen, Chin-Chung; Lin, Juei-Jueng; Peng, Giia-Sheun; Lin, Tsu-Kung; Wang, Shuu-Jiun; Chen, Rou-Shayn; Yang, Yuan-Han; Tsai, Chon-Haw; Hsu, Jung-Lung; Liao, Yi-Chu; Chen, Shih-Pin; Fuh, Jong-Ling

    2016-04-01

    Parkinson's disease dementia (PDD) contributes to poor quality of life and increases the mortality risk. Early detection and diagnosis of PDD are essential for clinical care. We recruited patients with idiopathic Parkinson's disease (PD), who underwent clinical assessments and neuropsychological tests, at 12 teaching hospitals in Taiwan. Probable PDD was diagnosed according to the Movement Disorder Society Task Force clinical criteria. Using binary logistic regression, we selected significant items from an original 30-item informant questionnaire. We utilized these items, along with a simple cognitive test, to discriminate between PDD and nondemented PD (PD-ND). Among the 265 PD patients (156 men, 109 women, mean age 71.9 ± 9.1 years), 102 and 163 patients were diagnosed with probable PDD and PD-ND, respectively. The mean education of participants was 8.8 ± 5.3 years, and the mean disease duration was 5.5 ± 5.4 years. When the patients fulfilled either of the following criteria: (1) a score ≥ 3 for the five endorsed screening questions, (2) a score of 1-2 for the five above screening questions combined with a score ≤ 10 items for category verbal fluency, the sensitivity and specificity of the PDD screening tool were 80.4% and 80.4%, respectively. The area under the receiver operating characteristic curve (AUC) was 0.804. We tested this screening tool in another 137 unrelated PD patients and the sensitivity, specificity, and AUC were 77.4%, 96.4%, and 0.869, respectively. The "PDD-5S" is a brief and useful screening tool for PDD. Copyright © 2016 Elsevier Ltd. All rights reserved.

  1. Neuropsychiatric Symptoms in Parkinson's Disease Dementia Are More Similar to Alzheimer's Disease than Dementia with Lewy Bodies: A Case-Control Study.

    PubMed

    Chiu, Pai-Yi; Tsai, Chun-Tang; Chen, Ping-Kun; Chen, Whe-Jen; Lai, Te-Jen

    2016-01-01

    Previous studies on the clinical and pathological manifestations of Parkinson's disease dementia (PDD) have reported findings more similar to dementia with Lewy bodies (DLB) than to Alzheimer's disease (AD). The aim of this study was to investigate the neuropsychiatric symptoms of PDD compared to DLB and AD. We conducted a retrospective case-control study on 125 newly diagnosed consecutive PDD patients and age- and dementia stage-matched controls with either DLB (N = 250) or AD (N = 500) who visited the same hospital over the same period. For each case and control, neuropsychiatric symptoms were assessed using the Neuropsychiatric Inventory (NPI). Overall, 513 (58.6%) patients were female and 362 (41.4%) were male. Comparisons of clinical data revealed that the PDD group, similar to the AD group, had a lower NPI total score, NPI caregiver burden score, and rate of antipsychotic use (all p < 0.001) than the DLB group. One or more psychiatric symptoms were reported in 95.2% of the PDD, 99.2% of the DLB, and 96.8% of the AD patients. The PDD group had lower subscores in the items of delusions, hallucinations, agitation, anxiety, irritation, aberrant motor behavior compared to the DLB group. Severe neuropsychiatric symptoms among all dementia patients were associated with younger age, more advanced stage, and a diagnosis of DLB. Neuropsychiatric symptoms in PDD were more like those in AD than in DLB. Severe neuropsychiatric symptoms in degenerative dementia were associated with younger age, more advanced stage of dementia, and a diagnosis of DLB.

  2. Brain volumetry in Parkinson's disease with and without dementia: where are the differences?

    PubMed

    Lee, Seung-Hwan; Kim, Sam Soo; Tae, Woo-Suk; Lee, Seo-Young; Lee, Kang Uk; Jhoo, Jinhyeoung

    2013-06-01

    Cognitive dysfunction is well documented in Parkinson's disease (PD). However, association between regional brain volume change and cognitive decline of PD is uncertain. To compare regional brain volume difference between PD without dementia (PDND) and PD with dementia (PDD). We enrolled 16 normal controls (mean ± SD: 69.5 ± 6.31) and 32 sex-, age-matched patients with PD (16 PDND and 16 PDD patients with Hoehn & Yahr stage II or III). Cognitive function was assessed using mini-mental status examination (MMSE). Intracranial volume (ICV) and the hippocampal volumes were manually measured using magnetic resonance imaging (MRI). Regional gray/white matter volume changes were analyzed using voxel-based morphometry. Age, ICV, volume of gray matter volume (GMV), white matter, and hippocampi did not differ among the three groups. The regional GMV of PDD was significantly decreased in the areas of right middle frontal gyrus, short insular gyri, superior temporal gyri; both precuneus compared to PDND (uncorrected P < 0.001). In the partial correlation analysis (controlled for age, sex, ICV), regional GMV of PD was positively correlated with MMSE score in the areas of short insular gyri, right circular insular sulcus, right calcarine sulcus, left superior temporal gyrus (planum porale), and left inferior precentral sulcus (uncorrected P < 0.001). We suggest that the volume loss of hippocampus may not be a finding in developing of PDD while variation of the regional volume of the frontal, insular cortex, superior temporal gyri, and precuneus lobes may be a phenomenon of PDD. © 2013 The Foundation Acta Radiologica.

  3. Neuropsychological study of amyotrophic lateral sclerosis and parkinsonism-dementia complex in Kii peninsula, Japan

    PubMed Central

    2014-01-01

    Background The Kii peninsula of Japan is one of the foci of amyotrophic lateral sclerosis and parkinsonism-dementia complex (ALS/PDC) in the world. The purpose of this study is to clarify the neuropsychological features of the patients with ALS/PDC of the Kii peninsula (Kii ALS/PDC). Methods The medical interview was done on 13 patients with Kii ALS/PDC, 12 patients with Alzheimer’s disease, 10 patients with progressive supranuclear palsy, 10 patients with frontotemporal lobar degeneration and 10 patients with dementia with Lewy bodies. These patients and their carer/spouse were asked to report any history of abulia-apathy, hallucination, personality change and other variety of symptoms. Patients also underwent brain magnetic resonance imaging (MRI), single photon emission computed tomography (SPECT), and neuropsychological tests comprising the Mini Mental State Examination, Raven’s Colored Progressive Matrices, verbal fluency, and Paired-Associate Word Learning Test and some of them were assessed with the Frontal Assessment Battery (FAB). Results All patients with Kii ALS/PDC had cognitive dysfunction including abulia-apathy, bradyphrenia, hallucination, decrease of extraversion, disorientation, and delayed reaction time. Brain MRI showed atrophy of the frontal and/or temporal lobes, and SPECT revealed a decrease in cerebral blood flow of the frontal and/or temporal lobes in all patients with Kii ALS/PDC. Disorientation, difficulty in word recall, delayed reaction time, and low FAB score were recognized in Kii ALS/PDC patients with cognitive dysfunction. Conclusions The core neuropsychological features of the patients with Kii ALS/PDC were characterized by marked abulia-apathy, bradyphrenia, and hallucination. PMID:25041813

  4. What can the treatment of Parkinson's disease learn from dementia care; applying a bio-psycho-social approach to Parkinson's disease.

    PubMed

    Gibson, Grant

    2017-07-07

    Within contemporary medical practice, Parkinson's disease (PD) is treated using a biomedical, neurological approach, which although bringing numerous benefits can struggle to engage with how people with PD experience the disease. A bio-psycho-social approach has not yet been established in PD; however, bio-psycho-social approaches adopted within dementia care practice could bring significant benefit to PD care. This paper summarises existing bio-psycho-social models of dementia care and explores how these models could also usefully be applied to care for PD. Specifically, this paper adapts the bio-psycho-social model for dementia developed by Spector and Orrell (), to suggest a bio-psycho-social model, which could be used to inform routine care in PD. Drawing on the biopsychosocial model of Dementia put forward by Spector and Orrell (), this paper explores the application of a bio-psycho-social model of PD. This model conceptualises PD as a trajectory, in which several interrelated fixed and tractable factors influence both PD's symptomology and the various biological and psychosocial challenges individuals will face as their disease progresses. Using an individual case study, this paper then illustrates how such a model can assist clinicians in identifying suitable interventions for people living with PD. This model concludes by discussing how a bio-psycho-social model could be used as a tool in PD's routine care. The model also encourages the development of a theoretical and practical framework for the future development of the role of the PD specialist nurse within routine practice. A biopsychosocial approach to Parkinson's Disease provides an opportunity to move towards a holistic model of care practice which addresses a wider range of factors affecting people living with PD. The paper puts forward a framework through which PD care practice can move towards a biopsychosocial perspective. PD specialist nurses are particularly well placed to adopt such a model

  5. The effects of cognitive reserve and lifestyle on cognition and dementia in Parkinson's disease--a longitudinal cohort study.

    PubMed

    Hindle, John V; Hurt, Catherine S; Burn, David J; Brown, Richard G; Samuel, Mike; Wilson, Kenneth C; Clare, Linda

    2016-01-01

    Cognitive reserve theory seeks to explain the observed mismatch between the degree of brain pathology and clinical manifestations. Early-life education, midlife social and occupational activities and later-life cognitive and social interactions are associated with a more favourable cognitive trajectory in older people. Previous studies of Parkinson's disease (PD) have suggested a possible role for the effects of cognitive reserve, but further research into different proxies for cognitive reserve and longitudinal studies is required. This study examined the effects of cognitive lifestyle on cross-sectional and longitudinal measures of cognition and dementia severity in people with PD. Baseline assessments of cognition, and of clinical, social and demographic information, were completed by 525 participants with PD. Cognitive assessments were completed by 323 participants at 4-year follow-up. Cognition was assessed using the measures of global cognition dementia severity. Cross-sectional and longitudinal serial analyses of covariance for cognition and binomial regression for dementia were performed. Higher educational level, socio-economic status and recent social engagement were associated with better cross-sectional global cognition. In those with normal cognition at baseline, higher educational level was associated with better global cognition after 4 years. Increasing age and low levels of a measure of recent social engagement were associated with an increased risk of dementia. Higher cognitive reserve has a beneficial effect on performance on cognitive tests and a limited effect on cognitive decline and dementia risk in PD. Copyright © 2015 John Wiley & Sons, Ltd.

  6. Evaluating rivastigmine in mild-to-moderate Parkinson's disease dementia using ADAS-cog items.

    PubMed

    Schmitt, Frederick A; Aarsland, Dag; Brønnick, Kolbjørn S; Meng, Xiangyi; Tekin, Sibel; Olin, Jason T

    2010-08-01

    Rivastigmine has been shown to improve cognition in patients with Parkinson's disease dementia (PDD). To further explore the impact of anticholinesterase therapy on PDD, Alzheimer's Disease Assessment Scale-cognitive subscale (ADAS-cog) items were assessed in a retrospective analysis of a 24-week, double-blind, placebo-controlled trial of rivastigmine. Mean changes from baseline at week 24 were calculated for ADAS-cog item scores and for 3 cognitive domain scores. A total of 362 patients were randomized to 3 to 12 mg/d rivastigmine capsules and 179 to placebo. Patients with PDD receiving rivastigmine improved versus placebo on items: word recall, following commands, ideational praxis, remembering test instructions, and comprehension of spoken language (P < .05), with standardized mean differences ranging from 0.04 to 0.30. Rivastigmine also showed significant effects versus placebo on all domains: memory, language, and praxis. The ADAS-cog is sensitive to broad cognitive changes in PDD. Overall, rivastigmine was associated with improvements on individual cognitive items and general cognitive domains.

  7. Subjective poor sleep quality in Chinese patients with Parkinson's disease without dementia

    PubMed Central

    Zhang, Li; Dong, Jingde; Liu, Weiguo; Zhang, Yingdong

    2013-01-01

    Parkinson's disease (PD) is a common progressive neurological disorder and is composed of motor and non-motor symptoms. Sleep disturbances are frequent problems for patients with PD. The relationship between sleep disturbances with Hoehn and Yahr (H&Y) staging have been demonstrated. However, the relationship between sleep disorders and H&Y is still unclear in patients with PD without dementia in Chinese PD patients. In this study, we interviewed 487 non-demented PD patients of Chinese Han descents by H&Y classification. We found that night sleep quality was significantly associated with the severity of PD (P = 0.008). Panic disorder severity scale (PDSS) total scores were correlated with PD non-motor symptoms scale (PDNMS) scores (r = -0.528, P < 0.001), the Hamilton depression scale (HAMD) scores (r = -0.545, P < 0.001) and the Hamilton anxiety scale (HAMA) scores (r = -0.498, P < 0.001). Our results indicated that sleep quality deteriorated with the advancing of PD in Chinese non-demented patients with PD. Depression and anxiety may partly explain sleep disturbances in non-demented patients with PD. PMID:23885268

  8. Nonselenium glutathione peroxidase in human brain : elevated levels in Parkinson's disease and dementia with lewy bodies.

    PubMed

    Power, John H T; Shannon, John M; Blumbergs, Peter C; Gai, Wei-Ping

    2002-09-01

    Nonselenium glutathione peroxidase (NSGP) is a new member of the antioxidant family. Using antibodies to recombinant NSGP we have examined the distribution of this enzyme in normal, Parkinson's disease (PD), and dementia with Lewy body disease (DLB) brains. We have also co-localized this enzyme with alpha-synuclein as a marker for Lewy bodies. In normal brains there was a very low level of NSGP staining in astrocytes. In PD and DLB there were increases in the number and staining intensity of NSGP-positive astrocytes in both gray and white matter. Cell counting of NSGP cells in PD and DLB frontal and cingulated cortices indicated there was 10 to 15 times more positive cells in gray matter and three times more positive cells in white matter than in control cortices. Some neurons were positive for both alpha-synuclein and NSGP in PD and DLB, and double staining indicated that NSGP neurons contained either diffuse cytoplasmic alpha-synuclein deposits or Lewy bodies. In concentric Lewy bodies, alpha-synuclein staining was peripheral whereas NSGP staining was confined to the central core. Immunoprecipitation indicated there was direct interaction between alpha-synuclein and NSGP. These results suggest oxidative stress conditions exist in PD and DLB and that certain cells have responded by up-regulating this novel antioxidant enzyme.

  9. Age and dementia-associated atrophy predominates in the hippocampal head and amygdala in Parkinson's disease.

    PubMed

    Bouchard, Thomas P; Malykhin, Nikolai; Martin, W R Wayne; Hanstock, Christopher C; Emery, Derek J; Fisher, Nancy J; Camicioli, Richard M

    2008-07-01

    The hippocampus (HC) and amygdala (AG) decrease in volume with age and in Parkinson's disease (PD) with (PDD) and without dementia. We compared 44 PD to 44 age, sex and education-matched subjects without PD (non-PD) and 13 PDD subjects. T1-weighted MR images were used to manually segment the head, body and tail of the HC and the AG. HC volumes, corrected to intracranial volume, were smaller in PDD than non-PD (p=0.04), reflected predominantly by head atrophy. Right AG volumes were smaller in PD compared to non-PD (p=0.03). HC volumes in older (>70), but not younger, non-demented PD differed from non-PD (HC, p=0.02; head, p=0.03). Age correlated negatively with overall HC (r=-0.43, p=0.004) and head (r=-0.48, p=0.001) in PD, but not in non-PD. In PD, left HC head volumes correlated with recall, but not recognition scores on the CVLT-II (r=0.35, p=0.02) and BVMT-R (r=0.35, p=0.02); AG volumes correlated with CVLT-II recall (r=0.35, p=0.02). No correlations were found in non-PD (p>0.4). In conclusion, functionally meaningful age-associated hippocampal and amygdala atrophy occurs in PD.

  10. Dementia

    MedlinePlus

    ... skills) Dementia usually first appears as forgetfulness. Mild cognitive impairment (MCI) is the stage between normal forgetfulness due to aging and the development of dementia. People with MCI have mild problems ...

  11. Dementia

    MedlinePlus

    ... dementia have serious problems with two or more brain functions, such as memory and language. Although dementia is common in very elderly people, it is not part of normal aging. Many different diseases ... dementia or repair brain damage, they may improve symptoms or slow down ...

  12. GDF15/MIC1 and MMP9 Cerebrospinal Fluid Levels in Parkinson's Disease and Lewy Body Dementia.

    PubMed

    Maetzler, Walter; Deleersnijder, Willy; Hanssens, Valérie; Bernard, Alice; Brockmann, Kathrin; Marquetand, Justus; Wurster, Isabel; Rattay, Tim W; Roncoroni, Lorenzo; Schaeffer, Eva; Lerche, Stefanie; Apel, Anja; Deuschle, Christian; Berg, Daniela

    2016-01-01

    Based on animal and ex-vivo experiments, Growth/Differentiation Factor-15 (GDF15, also called Macrophage Inhibitory Cytokine-1, MIC1), a member of the transforming growth factor-beta family, and Matrix Metalloproteinase-9 (MMP9), a member of the matrix metalloprotease family may be potential markers for Lewy body disorders, i.e. Parkinson's disease with (PDD) and without dementia (PDND) and Lewy body dementia (DLB). GDF15 has a prominent role in development, cell proliferation, differentiation, and repair, whereas MMP9 degrades, as a proteolytic enzyme, components of the extracellular matrix. In this study, cerebrospinal fluid GDF15 and MMP9 levels of 59 PDND, 17 PDD and 23 DLB patients, as well as of 95 controls were determined, and associated with demographic, clinical and biochemical parameters. Our analysis confirmed the already described association of GDF15 levels with age and gender. Corrected GDF15 levels were significantly higher in PDD than in PDND patients, and intermediate in DLB patients. Within Lewy body disorders, GDF15 levels correlated positively with age at onset of Parkinsonism and dementia, Hoehn & Yahr stage and cerebrospinal fluid t-Tau and p-Tau levels, and negatively with the Mini Mental State Examination. Remarkably, it does not relevantly correlate with disease duration. MMP9 was not relevantly associated with any of these parameters. Cerebrospinal GDF15, but not MMP9, may be a potential marker of and in Lewy body disorders.

  13. Cognitive disconnective syndrome by single strategic strokes in vascular dementia.

    PubMed

    Lanna, Maria Elisa de Oliveira; Alves, Carlos Eduardo O; Sudo, Felipe Kenji; Alves, Gilberto; Valente, Letice; Moreira, Denise Madeira; Cavalcanti, José Luiz Sá; Engelhardt, Eliasz

    2012-11-15

    Strategic regions correspond to associative, limbic and paralimbic structures and related circuits, that underpin cognitive/behavioral functions. Strokes in these eloquent sites produce pictures of vascular dementia with syndromic features due to specific site lesion and/or interruption of their interconnections. This study aims at analysing subcortical strategic strokes that express similar cognitive/behavioral elements, by sharing common pathways. Patients (n=6) who attended in specialized ambulatory, were submitted to neuropsychological and neuroimaging assessments through MRI (GE Signa Horizon 1.5T) and brain SPECT (Millennium MG, ECD [TC-99m]). Stroke locations and respective main symptoms were: 1. anteromedian thalamus [L]: anterograde and retrograde amnesia (ARA), expression aphasia (EA), executive dysfunction (ED), apathy, and depression; 2. anterior thalamus [R]: ARA, inattention, apathy, and aggressiveness; 3. dorsomedian thalamus [L]: inattention, ED, anosognosia, and aggressiveness; 4. central paramedian thalamus [R]: EA, visual perception deficits (VPD), ED, infantility, and personality disorder; 5. caudate nucleus (ventral-head) [L]: VPD, ED, delirium, visual hallucinations, and personality disorder; and 6. anterior capsule [L]: VPD, ED, apathy, and depression. Vascular strategic syndromes connote the predominantly impaired cognitive/behavioral symptom of each site. Temporal and frontal disconnection symptoms were produced by disrupted MTT/hippocampal and IML/amygdala circuits expressing amnesic syndrome associated with heterogeneous dysexecutive syndrome, in all the cases, by disrupting frontal-basal ganglia-thalamus-cortical net, in three different levels of their pathway.

  14. Ropinirole-induced Pisa syndrome in Parkinson disease.

    PubMed

    Galati, Salvatore; Möller, Jens Carsten; Städler, Claudio

    2014-01-01

    Pisa syndrome (PS), also known as pleurothotonus, is an abnormal posture characterized by lateral flexion of the trunk that typically disappears in supine position. In Parkinson disease (PD), an abnormal forward flexion of the trunk (defined as camptocormia) is a common observation and has been interpreted as a sign of dystonia. Few reports have described PS mainly related to dopaminergic therapy in this kind of patients.Levodopa/carbidopa, levodopa/benserazide, levodopa/carbidopa/entacapone, pergolide, and pramipexole may cause PS, whereas no reports for ropinirole have been described.Here, we describe a case of a patient with PD who developed severe and reversible PS due to ropinirole intake.

  15. [Guideline-based diagnosis of dementia etiology].

    PubMed

    Hofmann, W

    2012-12-01

    Dementia encompasses a variety of underlying conditions among which Alzheimer's disease represents the most common cause and in addition, vascular and Parkinson's disease dementia, dementia with Lewy bodies and frontotemporal lobar degeneration. All the current guidelines specify a two-step procedure for the diagnostics of dementia. The first step entails performing a comprehensive description, diagnosis and confirmation of the syndrome. The presented article focuses on the second step: diagnosis of the etiology. This review gives an overview of the current diagnostic approaches including the new proposals of the biomarker in cerebrospinal fluid.

  16. Impact of Sjogren's syndrome on Parkinson's disease: A nationwide case-control study.

    PubMed

    Wu, Ming-Chi; Xu, Xun; Chen, Shan-Ming; Tyan, Yeu-Sheng; Chiou, Jeng-Yuan; Wang, Yu-Hsun; Lin, Li-Chi; Chen, Chyong-Mei; Wei, James Cheng-Chung

    2017-01-01

    To investigate whether Sjogren's syndrome would have an influence on the development of Parkinson's disease. A population-based case-control study was conducted. Participants consisted of 7716 subjects with newly diagnosed Parkinson's disease and a population of 75129 matched control subjects between 2000 and 2010. We measured the risk of Parkinson's disease in association with Sjogren's syndrome by using adjusted odds ratios. A total of 143 Parkinson's disease subjects (1.9%) and 893 control subjects (1.2%) suffered from Sjogren's syndrome (p < 0.001). The crude odds ratio for Parkinson's disease among subjects with Sjogren's syndrome was 1.56 (95% CI 1.30-1.86; p < 0.01). After adjustment for potential confounders which have been proposed that would increase the risk of development of Parkinson's disease, Sjogren's syndrome was found to be significantly associated with the risk of Parkinson's disease with an odds ratio of 1.37 (95% CI 1.15-1.65; p < 0.01). This study preliminarily proposed that Sjogren's syndrome was significant associated with an increased risk of Parkinson's disease.

  17. A comparison of gray and white matter density in patients with Parkinson's disease dementia and dementia with Lewy bodies using voxel-based morphometry.

    PubMed

    Lee, Ji E; Park, Bosuk; Song, Sook K; Sohn, Young H; Park, Hae-Jeong; Lee, Phil Hyu

    2010-01-15

    Despite clinical and neuropsychological similarities between Parkinson's disease dementia (PDD) and dementia with Lewy bodies (DLB), recent studies have demonstrated that structural and pathological changes are more severe in DLB than in PDD. We used voxel-based morphometry using a 3-T MRI scanner to compare gray and white matter densities in 20 patients with probable PDD and 18 patients with probable DLB, who had similar overall severity of dementia and similar demographic characteristics. The gray matter density was significantly decreased in the left occipital, parietal, and striatal areas in patients with DLB compared with patients with PDD. The white matter density was significantly decreased in bilateral occipital and left occipito-parietal areas in patients with DLB compared with those with PDD. The degree of white and gray matter atrophy was similar in patients with DLB; in contrast, there was markedly less atrophy in the white matter than in the gray matter in patients with PDD. On analyzing the change of WM density relative to that of GM density in patients with DLB compared to those with PDD, the area of WM atrophy in the occipital areas was more extensive than that of GM atrophy. Our data demonstrate that atrophy of both gray and white matter was more severe in patients with DLB and that white matter atrophy relative to gray matter atrophy was less severe in patients with PDD. These data may reflect a difference in the underlying nature of PDD and DLB.

  18. Validation and attempts of revision of the MDS-recommended tests for the screening of Parkinson's disease dementia.

    PubMed

    Isella, V; Mapelli, C; Siri, C; De Gaspari, D; Pezzoli, G; Antonini, A; Poletti, M; Bonuccelli, U; Vista, M; Appollonio, I M

    2014-01-01

    The Movement Disorders Society (MDS) formulated diagnostic criteria and assessment guidelines for the screening of dementia in Parkinson's disease (PD). We carried out a validation of the cognitive measures suggested in the screening algorithm (i.e. the Mini Mental State Examination - MMSE - total score, serial 7s subtraction, 3-word recall, pentagons copy, and one minute letter fluency) in 86 patients with PD. Thirty-six percent of participants were diagnosed with dementia using the MDS algorithm, but with the Dementia Rating Scale instead of the MMSE. The original MDS procedure misclassified 11 patients (12.8%) as false negatives and 3 (3.5%) as false positives, leading to 65% sensitivity and 95% specificity. The main reason for misdiagnoses was insensitivity of the MMSE total score. Three attempts were made to reach a better screening performance, which warrants high sensitivity more than high specificity: 1. exclusion of the MMSE total score as a diagnostic requirement; 2. determination of a better cut off through Receiver Operating Characteristic curve analysis; 3. replacement of the MMSE with the equally undemanding, but more PD-specific, Mini Mental Parkinson. The first two strategies generally yielded high sensitivity, but poor specificity. The best outcome was achieved using a Mini Mental Parkinson total score <27 as cognitive criterion: sensitivity was 87% and negative predictive value was 90%; however, specificity was only 67%. Our findings seem to suggest that MDS practical guidelines are specific, but might benefit from the use of more PD-oriented tools than the MMSE in terms of sensitivity. Copyright © 2013 Elsevier Ltd. All rights reserved.

  19. Acceleration of ventricular rate by fibrillation associated with the Wolff-Parkinson-White syndrome.

    PubMed

    Sheinman, B D; Evans, T

    1982-10-09

    Amiodarone has proved to be a valuable drug in atrial fibrillation associated with the Wolff-Parkinson-White syndrome. When it was administered to a patient with this syndrome in atrial fibrillation, who had previously suffered an inferior myocardial infarction, the ventricular rate accelerated from 170 to 230 beats/minute.This unusual case emphasises the need for full electrophysiological assessment of patients with the Wolff-Parkinson-White syndrome for whom amiodarone treatment is being considered.

  20. Psychotic symptoms complicate the clinical differentiation of Parkinson's disease with major depressive disorder from dementia with Lewy bodies.

    PubMed

    Miyashita, Mitsuhiro; Sasayama, Daimei; Sugiyama, Nobuhiro; Yasaki, Takehiko; Washizuka, Shinsuke; Amano, Naoji

    2010-06-01

    Dementia with Lewy bodies (DLB) is diagnosed clinically according to the diagnostic criteria in the Third Report of the DLB Consortium. However, psychotic symptoms, such as visual hallucinations, delusions, and stupor, may complicate the clinical diagnosis of DLB. The present study reports on a patient with Parkinson's disease that was difficult to distinguish from DLB because of the presence of various psychotic symptoms. In making a diagnosis of DLB, it is important to assess essential psychiatric features and to observe patients for any changes in these features.

  1. Antihypertensive Agents and Risk of Parkinson's Disease, Essential Tremor and Dementia: A Population-Based Prospective Study (NEDICES)

    PubMed Central

    Louis, Elan D.; Benito-León, Julián; Bermejo-Pareja, Félix

    2009-01-01

    Background Recent interest in antihypertensive agents, especially calcium channel blockers, has been sparked by the notion that these medications may be neuroprotective. A modest literature, with mixed results, has examined whether these medications might lower the odds or risk of Parkinson's disease (PD) or dementia. There are no data for essential tremor (ET). Objective To examine the association between antihypertensive use (defined broadly and by individual subclasses) and ET, PD and dementia. For each disorder, we used cross-sectional data (association with prevalent disease) and prospective data (association with incident disease). Methods Prospective population-based study in Spain enrolling 5,278 participants at baseline. Results Use of antihypertensive medications (aside from β-blockers) was similar in prevalent ET cases and controls. Baseline use of antihypertensive agents was not associated with reduced risk of incident ET. Antihypertensive medication use was not associated with prevalent or incident PD. Calcium channel blocker use was marginally reduced in prevalent dementia cases (ORadjusted = 0.63, p = 0.06) but was not associated with reduced risk of incident dementia (RRadjusted = 1.02, p = 0.95). Conclusions We did not find evidence of a protective effect of antihypertensive medications in these three neurodegenerative disorders. PMID:19696520

  2. Behavioural Characteristics Associated with Dementia Assessment Referrals in Adults with Down Syndrome

    ERIC Educational Resources Information Center

    Adams, D.; Oliver, C.; Kalsy, S.; Peters, S.; Broquard, M.; Basra, T.; Konstandinidi, E.; McQuillan, S.

    2008-01-01

    Background: Behavioural changes associated with dementia in Down syndrome are well documented, yet little is known about the effect of such behaviours on carers and referral. By comparing the behavioural and cognitive profiles of individuals referred for a dementia assessment with those of individuals not referred, some insight can be gained into…

  3. Behavioural Characteristics Associated with Dementia Assessment Referrals in Adults with Down Syndrome

    ERIC Educational Resources Information Center

    Adams, D.; Oliver, C.; Kalsy, S.; Peters, S.; Broquard, M.; Basra, T.; Konstandinidi, E.; McQuillan, S.

    2008-01-01

    Background: Behavioural changes associated with dementia in Down syndrome are well documented, yet little is known about the effect of such behaviours on carers and referral. By comparing the behavioural and cognitive profiles of individuals referred for a dementia assessment with those of individuals not referred, some insight can be gained into…

  4. [Sudden death in intermittent Wolff Parkinson White syndrome].

    PubMed

    Medeiros, A; Iturralde, P; Guevara, M; Mendoza, C; Colín, L

    2001-01-01

    Sudden death is a rare condition in asymptomatic patients with asymptomatic intermittent Wolff Parkinson syndrome (WPW); for this reason it is believed that these patients should not undergo to radiofrequency ablation. We report an asymptomatic 44 year old man who developed ventricular fibrillation with a pre-excited RR interval less than 200 msec during atrial fibrillation, as a first manifestation of WPW syndrome. The Holter monitoring showed intermittent pre-excitation at low heart rate (70 bpm). During the electrophysiological study a successfully radiofrequency catheter ablation of a right posteroseptal accessory pathway was performed. We concluded that intermittent pre-excitation may not be used to identify patients who are at risk of sudden death. Radiofrequency catheter ablation should be recommended in those patients with a very high success rate, and a low incidence of serious complications.

  5. [Dementia with motor and language disorders].

    PubMed

    Frédéric, Assal; Ghika, Joseph

    2016-04-20

    Memory is not the only core diagnostic criteria in Alzheimer's disease and many dementias are characterized by other cognitive deficits. Moreover dementias are often associated with multiple and complex motor signs. The first part of this reviewcovers parkinsonism in diffuse Lewy Body Disease and other neurodegenerative diseases, corticobasal syndrome, or motor deficit in the motoneurone disease-frontotemporal dementia spectrum. In the second part, primary progressive aphasia and its three variants including basic clinical evaluation are described. These complex clinical syndromes involving motor and language systems are important for the clinical practice since they are part of diagnostic criteria of several neurodegenerative diseases and can be considered as phenotypical markers of neurodegeneration.

  6. [Muro disease: amyotrophic lateral sclerosis/parkinsonism-dementia complex in Kii peninsula of Japan].

    PubMed

    Kuzuhara, Shigeki

    2011-02-01

    Muro disease refers to the endemic amyotrophic lateral sclerosis (ALS) and parkinsonism-dementia complex (PDC) in the high incidence ALS focus in the Muro district of the Kii peninsula. Kii paralysis was first described in the 1680s in a folk literature, and as ALS in the medical literature by Kin-no-suke Miura in 1911. Two high-incidence ALS foci were discovered in 1960s by Kimura and Yase, and retro- and anterospective epidemiological surveys were started. Kii ALS was neuropathologically characterized by classical ALS pathology together with many neurofibrillary tangles (NFTs) in the brain, similar to Guamanian ALS. The incidence rates of ALS dramatically declined during the 1950s and 1980s, resulting in the disappearance of the high-incidence foci. In the early 1990s, however, Kuzuhara found existence of high-incidence of ALS in the region, and, in addition, of a high-incidence of PDC with abundant NFTs, similar to Guamanian PDC. The incidence rates of PDC dramatically rose during the 1980s and 1990s, and PDC replaced ALS. Unsuccessful attempts were made to identify cause and pathogenesis of the disease in minerals and environmental factors. More than 70% of patients in the endemic region had a family history of ALS or PDC; therefore, genetic factors were suspected as the cause. The authors analyzed the causative and risk candidate genes in the affected and unaffected family members, but failed to find genes related to ALS/PDC. The changing pattern of Muro disease from ALS with a younger onset and rapid progression to PDC with a later onset and longer survival suggests that some unknown environmental factor(s) might modulate the disease process, which basically might be programmed in the gene(s).

  7. Cognition in individuals at risk for Parkinson's: Parkinson associated risk syndrome (PARS) study findings.

    PubMed

    Chahine, Lama M; Weintraub, Daniel; Hawkins, Keith A; Siderowf, Andrew; Eberly, Shirley; Oakes, David; Seibyl, John; Stern, Matthew B; Marek, Kenneth; Jennings, Danna

    2016-01-01

    The Parkinson Associated Risk Syndrome Study identified a cohort of healthy adults with hyposmia and dopamine transporter binding reduction to characterize individuals at risk for Parkinson's disease (PD). We describe the cognitive profile of this cohort. Individuals older than 50 y without PD were recruited. Two hundred twenty-five completed cognitive testing and were included in the final analysis. A neuropsychological test battery was administered and normative scores created for global cognition, memory, executive function/working memory, processing speed/attention, visuospatial abilities, and language domains. Other non-motor symptoms (constipation, depression, anxiety, and rapid eye movement sleep behavior disorder) were assessed through questionnaires. Individuals with both hyposmia and reduced dopamine transporter binding (n = 38) had lower mean scores for global cognition, executive function/working memory, and memory compared with all other participants (n = 187). In separate multivariate logistic regression models, lower global cognition (odds ratio, 1.97, P = 0.004), and specifically executive function/working memory (odds ratio, 1.84, P = 0.004) scores were associated with membership in the hyposmia with dopamine transporter reduction group. Combining hyposmia with relative impairment on specific cognitive domains increased the odds of dopamine transporter binding reduction compared with hyposmia alone, with the greatest increase in odds for hyposmia plus executive function/working memory relative impairment (68% increase in odds from 4.14 to 6.96). Changes in global cognitive abilities, and specifically executive function/working memory, are present in individuals at risk for PD. Combining non-motor features, including cognition, improves prediction of dopamine transporter binding reduction. © 2015 International Parkinson and Movement Disorder Society.

  8. Restless legs syndrome and its associated risk factors in Parkinson's disease.

    PubMed

    Azmin, Shahrul; Khairul Anuar, Abdul Manaf; Nafisah, Wan Yahya; Tan, Hui Jan; Raymond, Azman Ali; Hanita, Othman; Shah, Shamsul Azhar; Norlinah, Mohamed Ibrahim

    2013-01-01

    Introduction. Restless legs syndrome has been shown to negatively impact the quality of life of patients. Studies have shown an association between restless legs syndrome and Parkinson's disease. We attempted to investigate the prevalence of restless legs syndrome in Parkinson's disease patients and to identify associated risk factors. Method. This was a cross-sectional study among patients with idiopathic Parkinson's disease. Exclusion criterion was a Mini Mental State Examination score of less than 21/30. The International Restless Legs Syndrome Study Group criterion was used to identify patients with restless legs syndrome. Results. A total of 113 patients were recruited. The prevalence rate of restless legs syndrome in our cohort was 9.7% and was significantly associated with a younger onset of Parkinson's disease (P = 0.023), male gender (P = 0.045), higher Mini Mental State Examination score (P = 0.004), and less advanced Hoehn & Yahr stage (P = 0.014). Conclusion. The prevalence rate of restless legs syndrome in our Parkinson's disease population is in keeping with other studies published worldwide. The significance of the association between a younger onset of Parkinson's disease and restless legs syndrome needs to be further investigated.

  9. Wolff-Parkinson-White Syndrome--current views.

    PubMed

    Chung, E K

    1977-02-01

    The Wolff-Parkinson-White (WPW) syndrome is an important clinical entity because of frequent recurrences of very rapid tachyarrhythmias. The electrocardiographic finding of the WPW syndrome often mimicks pseudo diaphragmatic (inferior) myocardial infarction which should not be misinterpreted. The most important diagnostic criterion is recognition of a delta wave; the short P-R interval or broad QRS complex may not be present in every case. The mechanism for the tachycardia is considered to be a reentry phenomenon via anomalous and normal atrioventricllar (A-V) pathways. The drug of choice for the treatment of regular supraventricular (reciprocating) tachycardia with narrow QRS complexes, which is the most common arrhythmia in the WPW syndrome, is propranolol. Digitalis is almost equally effective in this case. For tachyarrhythmias, particularly atrial fibrillation or flutter with anomalous conduction, intravenously-administered lidocaine is considered to be the drug of choice. Procainamide or quinidine is also frequently used under this circumstance with excellent therapeutic result. Many patients with the WPW syndrome require long-term maintenance drug therapy (propranolol, digitalis or quinidine in most cases). In urgent clinical situations, direct current (DC) shock should be applied immediately. In selected patients with refractory tachyarrhythmias, the use of an artificial pacemaker or surgical approach may be considered.

  10. The Distinct Cognitive Syndromes of Parkinson's Disease: 5 Year Follow-Up of the CamPaIGN Cohort

    ERIC Educational Resources Information Center

    Williams-Gray, Caroline H.; Evans, Jonathan R.; Goris, An; Foltynie, Thomas; Ban, Maria; Robbins, Trevor W.; Brayne, Carol; Kolachana, Bhaskar S.; Weinberger, Daniel R.; Sawcer, Stephen J.; Barker, Roger A.

    2009-01-01

    Cognitive abnormalities are common in Parkinson's disease, with important social and economic implications. Factors influencing their evolution remain unclear but are crucial to the development of targeted therapeutic strategies. We have investigated the development of cognitive impairment and dementia in Parkinson's disease using a longitudinal…

  11. The Distinct Cognitive Syndromes of Parkinson's Disease: 5 Year Follow-Up of the CamPaIGN Cohort

    ERIC Educational Resources Information Center

    Williams-Gray, Caroline H.; Evans, Jonathan R.; Goris, An; Foltynie, Thomas; Ban, Maria; Robbins, Trevor W.; Brayne, Carol; Kolachana, Bhaskar S.; Weinberger, Daniel R.; Sawcer, Stephen J.; Barker, Roger A.

    2009-01-01

    Cognitive abnormalities are common in Parkinson's disease, with important social and economic implications. Factors influencing their evolution remain unclear but are crucial to the development of targeted therapeutic strategies. We have investigated the development of cognitive impairment and dementia in Parkinson's disease using a longitudinal…

  12. Evaluation of doctor/patient satisfaction with the use of the Parkinson's Disease Dementia-Short-Screen (PDD-SS): a screening test for dementia in Parkinson's disease (DIFFUSION study).

    PubMed

    Kulisevsky, J; Pagonabarraga, J; Llebaria, G; Hernández, B; Arranz, J

    2011-10-01

    Parkinson disease (PD) has no specific neuropsychological scales for assessing the most significant cognitive impairment in PD, which has determined the use of subjective criteria or instruments designed for other diseases, making difficult the comparison between studies or the follow-up of patients. A screening test for dementia in PD (Parkinson's Disease Dementia-Short Screen [PDD-SS]) has recently been validated. To assess the degree of satisfaction of patients and researchers through the use of PDD-SS in clinical practice. An observational, cross-over, multicentre and national study was conducted on 471 patients with PD. The degree of patient satisfaction was measured using a questionnaire in which the items scored from 0 to 10 on a visual analogue scale (0 = strongly disagree, 10 = completely agree), while the researchers were determined on a 1-5 point Likert scale (1 = strongly disagree, 5 = completely agree). A total of 171 patients (36.3%) patients had dementia associated with PD according to the PDD-SS, of whom 77.3% said they were satisfied with its use. The overall measurement of researcher satisfaction was 3.6±0.6 points. Ninety per cent (n=45) of them reported an overall score >3 points in the satisfaction questionnaire. The mean values of perception of applicability, usability and reliability of PDD-SS among researchers was 3.5±0.7, 3.7±0.6 and 3.1±0.5 points, respectively. PD patients, as well as most of the researchers, were satisfied with the use of PDD-SS in clinical practice. Copyright © 2011 Sociedad Española de Neurología. Published by Elsevier Espana. All rights reserved.

  13. [ALS-parkinsonism-dementia complex of the Kii peninsula of Japan (Muro disease). Historical review, epidemiology and concept].

    PubMed

    Kuzuhara, Shigeki

    2007-11-01

    The Muro district includes the southern coastal mountainous areas of the Kii peninsula of Japan where high incidence foci of ALS and parkinsonism-dementia complex (PDC) exist. "Muro disease" refers to the endemic ALS in Muro, and the oldest description is in a book published in 1689. Miura reported high prevalence of ALS in Muro in 1911, and the first epidemiological survey by Kimura and Yase in 1960s disclosed extremely high prevalence of ALS in Hohara and Kozagawa. The high incidence was, however, reported to have disappeared by early 1980s as in Guam. In 1990s we resurveyed and found not only continuous high ALS incidence but also neuropathologically-verified PDC in Hohara. ALS and PDC here frequently affected one individual simultaneously and members in a family. Neuropathological changes were common to ALS and PDC, showing a combination of ALS changes and many neurofibrillar tangles (NFTs) in the brainstem and cerebral cortex, suggesting ALS/PDC may be a single entity with different clinical manifestations, "ALS-parkinsonism-dementia complex". TDP-43-positive inclusions were confirmed in all cases examined. Age-adjusted incidence rates during 1950 and 2000 have showed that incidence of ALS gradually declined for 50 years while that of PDC rose up steeply in 1990s, suggesting changing pattern of ALS/PDC that had occurred in Guam in 1970s. Continuing high incidence of ALS/PDC and high familial occurrence suggest that primary cause of Kii ALS/PDC may be genetic rather than environmental.

  14. Sundown Syndrome in Persons with Dementia: An Update

    PubMed Central

    Khachiyants, Nina; Trinkle, David; Son, Sang Joon

    2011-01-01

    "Sundowning" in demented individuals, as distinct clinical phenomena, is still open to debate in terms of clear definition, etiology, operationalized parameters, validity of clinical construct, and interventions. In general, sundown syndrome is characterized by the emergence or increment of neuropsychiatric symptoms such as agitation, confusion, anxiety, and aggressiveness in late afternoon, in the evening, or at night. Sundowning is highly prevalent among individuals with dementia. It is thought to be associated with impaired circadian rhythmicity, environmental and social factors, and impaired cognition. Neurophysiologically, it appears to be mediated by degeneration of the suprachiasmatic nucleus of the hypothalamus and decreased production of melatonin. A variety of treatment options have been found to be helpful to ameliorate the neuropsychiatric symptoms associated with this phenomenon: bright light therapy, melatonin, acetylcholinesterase inhibitors, N-methyl-d-aspartate receptor antagonists, antipsychotics, and behavioral modifications. To decrease the morbidity from this specific condition, improve patient's well being, lessen caregiver burden, and delay institutionalization, further attention needs to be given to development of clinically operational definition of sundown syndrome and investigations on etiology, risk factors, and effective treatment options. PMID:22216036

  15. Sundown syndrome in persons with dementia: an update.

    PubMed

    Khachiyants, Nina; Trinkle, David; Son, Sang Joon; Kim, Kye Y

    2011-12-01

    "Sundowning" in demented individuals, as distinct clinical phenomena, is still open to debate in terms of clear definition, etiology, operationalized parameters, validity of clinical construct, and interventions. In general, sundown syndrome is characterized by the emergence or increment of neuropsychiatric symptoms such as agitation, confusion, anxiety, and aggressiveness in late afternoon, in the evening, or at night. Sundowning is highly prevalent among individuals with dementia. It is thought to be associated with impaired circadian rhythmicity, environmental and social factors, and impaired cognition. Neurophysiologically, it appears to be mediated by degeneration of the suprachiasmatic nucleus of the hypothalamus and decreased production of melatonin. A variety of treatment options have been found to be helpful to ameliorate the neuropsychiatric symptoms associated with this phenomenon: bright light therapy, melatonin, acetylcholinesterase inhibitors, N-methyl-d-aspartate receptor antagonists, antipsychotics, and behavioral modifications. To decrease the morbidity from this specific condition, improve patient's well being, lessen caregiver burden, and delay institutionalization, further attention needs to be given to development of clinically operational definition of sundown syndrome and investigations on etiology, risk factors, and effective treatment options.

  16. Memory Impairments Underlying Language Difficulties in Dementia.

    ERIC Educational Resources Information Center

    Azuma, Tamiko; Bayles, Kathryn A.

    1997-01-01

    The memory deficits associated with the dementia syndromes of Alzheimer's, Parkinson's, and Lewy body disease are outlined and related to the language impairments that have been observed in patients with these disorders. Assessment techniques are described, including commonly used individual tests and test batteries that assess memory and…

  17. Epidemiological surveillance of amyotrophic lateral sclerosis and parkinsonism-dementia in the Commonwealth of the Northern Mariana Islands.

    PubMed

    Yanagihara, R T; Garruto, R M; Gajdusek, D C

    1983-01-01

    Amyotrophic lateral sclerosis (ALS) and parkinsonism-dementia (PD), two fatal neurological diseases of unknown cause, occur in high incidence among the Chamorro people of Guam, the largest and southernmost island within the Mariana archipelago. To reassess and extend our present epidemiological knowledge of these degenerative diseases in this focal geographical region, a systematic search for both disorders was conducted on the remaining inhabited islands of Rota, Tinian, Saipan, and the four remote islands of Anatahan, Alamagan, Pagan, and Agrihan within the Marianas chain. One case of ALS (on Saipan), 2 cases of PD (on Rota and Saipan), and 6 cases of parkinsonism without dementia (2 on Rota, 3 on Saipan, 1 on Tinian) were encountered among the approximately 17,000 inhabitants. No cases of either ALS, PD, or parkinsonism were found in the four remote Northern Islands. An additional 22 cases of ALS and 8 cases of PD were identified from reports of previous case-finding surveys, hospital records, and death certificates. Among Chamorros born on Rota, the average annual age-adjusted mortality rates of ALS per 100,000 population were 37.7 for the 15-year period 1956 to 1970 and 22.5 for the past decade, 1971 to 1980. Among Chamorros born on Saipan, the average annual mortality rates were 7.2 and 3.2 per 100,000, respectively, for the two periods. The mortality rates of PD were also significantly lower on Saipan than on Rota. In general, the age-adjusted mortality rates of ALS and PD on Rota were similar to those currently observed on Guam. Since the origins and current genotypic composition of Chamorros on all the Mariana Islands are indistinguishable, the strikingly lower mortality rates of ALS and PD on Saipan suggest that environmental factors are far more important than genetic factors in the pathogenesis of these diseases.

  18. The undiscovered syndrome: Macdonald Critchley’s case of semantic dementia

    PubMed Central

    Witoonpanich, Pirada; Crutch, Sebastian J.; Warren, Jason D.; Rossor, Martin N.

    2015-01-01

    Semantic dementia is a unique clinicopathological syndrome in the frontotemporal lobar degeneration spectrum. It is characterized by progressive and relatively selective impairment of semantic memory, associated with asymmetric antero-inferior temporal lobe atrophy. Although the syndrome became widely recognized only in the 1980s, descriptions of cases with typical features of semantic dementia have been on record for over a century. Here, we draw attention to a well documented historical case of a patient with features that would have fulfilled current consensus criteria for semantic dementia, as reconstructed from the notes made by her neurologist, Macdonald Critchley, in 1938. This case raises a number of issues concerning the nosology of the semantic dementia syndrome and the potential value of archived case material. PMID:24818802

  19. Dementia and severity of parkinsonism determines the handicap of patients in late-stage Parkinson's disease: the Barcelona-Lisbon cohort.

    PubMed

    Coelho, M; Marti, M J; Sampaio, C; Ferreira, J J; Valldeoriola, F; Rosa, M M; Tolosa, E

    2015-02-01

    Handicap has not been explored as a patient-centred outcome measure in Parkinson's disease (PD). The clinical features and medication use in late stages of PD (LS-PD) were reported previously. Handicap, medical conditions, use of healthcare resources and the impact of LS-PD upon caregivers were characterized in a cross-sectional study of LS-PD stages 4 or 5 of Hoehn and Yahr (H&Y). Handicap was measured using the London Handicap Scale (LHS: 0, maximal handicap; 1, no handicap). The mean LHS score in 50 patients was 0.33 (SD ±0.15). The presence of dementia, the Unified Parkinson's Disease Rating Scale part I score and the H&Y stage in 'off' independently predicted the LHS score (adjusted R(2) = 0.62; P = 0.000). Comorbidities and past medical conditions were frequent. Thirty-five patients lived at their house. Forty-five received unpaid care. Mean visits to the family doctor in the preceding 6 months were 2.2 (SD ±3.0) and to a neurologist 1.7 (SD ±1.0). Use of other health resources was low. Unpaid caregivers spent much time with patients and reported a high burden. Handicap could be measured in LS-PD and the LHS was easily completed by patients and caregivers. The high handicap in our cohort was mostly driven by the presence of dementia, behavioural complaints and the severity of non-dopaminergic motor features. Patients visited doctors infrequently and made low use of health resources, whilst unpaid caregivers reported a high burden. © 2014 EAN.

  20. White Matter Tract Damage in the Behavioral Variant of Frontotemporal and Corticobasal Dementia Syndromes

    PubMed Central

    Tovar-Moll, Fernanda; de Oliveira-Souza, Ricardo; Bramati, Ivanei Edson; Zahn, Roland; Cavanagh, Alyson; Tierney, Michael; Moll, Jorge; Grafman, Jordan

    2014-01-01

    The phenotypes of the behavioral variant of frontotemporal dementia and the corticobasal syndrome present considerable clinical and anatomical overlap. The respective patterns of white matter damage in these syndromes have not been directly contrasted. Beyond cortical involvement, damage to white matter pathways may critically contribute to both common and specific symptoms in both conditions. Here we assessed patients with the behavioral variant of frontotemporal dementia and corticobasal syndrome with whole-brain diffusion tensor imaging to identify the white matter networks underlying these pathologies. Twenty patients with the behavioral variant of frontotemporal dementia, 19 with corticobasal syndrome, and 15 healthy controls were enrolled in the study. Differences in tract integrity between (i) patients and controls, and (ii) patients with the corticobasal syndrome and the behavioral variant of frontotemporal dementia were assessed with whole brain tract-based spatial statistics and analyses of regions of interest. Behavioral variant of frontotemporal dementia and the corticobasal syndrome shared a pattern of bilaterally decreased white matter integrity in the anterior commissure, genu and body of the corpus callosum, corona radiata and in the long intrahemispheric association pathways. Patients with the behavioral variant of frontotemporal dementia showed greater damage to the uncinate fasciculus, genu of corpus callosum and forceps minor. In contrast, corticobasal syndrome patients had greater damage to the midbody of the corpus callosum and perirolandic corona radiata. Whereas several compact white matter pathways were damaged in both the behavioral variant of frontotemporal dementia and corticobasal syndrome, the distribution and degree of white matter damage differed between them. These findings concur with the distinctive clinical manifestations of these conditions and may improve the in vivo neuroanatomical and diagnostic characterization of these

  1. White matter tract damage in the behavioral variant of frontotemporal and corticobasal dementia syndromes.

    PubMed

    Tovar-Moll, Fernanda; de Oliveira-Souza, Ricardo; Bramati, Ivanei Edson; Zahn, Roland; Cavanagh, Alyson; Tierney, Michael; Moll, Jorge; Grafman, Jordan

    2014-01-01

    The phenotypes of the behavioral variant of frontotemporal dementia and the corticobasal syndrome present considerable clinical and anatomical overlap. The respective patterns of white matter damage in these syndromes have not been directly contrasted. Beyond cortical involvement, damage to white matter pathways may critically contribute to both common and specific symptoms in both conditions. Here we assessed patients with the behavioral variant of frontotemporal dementia and corticobasal syndrome with whole-brain diffusion tensor imaging to identify the white matter networks underlying these pathologies. Twenty patients with the behavioral variant of frontotemporal dementia, 19 with corticobasal syndrome, and 15 healthy controls were enrolled in the study. Differences in tract integrity between (i) patients and controls, and (ii) patients with the corticobasal syndrome and the behavioral variant of frontotemporal dementia were assessed with whole brain tract-based spatial statistics and analyses of regions of interest. Behavioral variant of frontotemporal dementia and the corticobasal syndrome shared a pattern of bilaterally decreased white matter integrity in the anterior commissure, genu and body of the corpus callosum, corona radiata and in the long intrahemispheric association pathways. Patients with the behavioral variant of frontotemporal dementia showed greater damage to the uncinate fasciculus, genu of corpus callosum and forceps minor. In contrast, corticobasal syndrome patients had greater damage to the midbody of the corpus callosum and perirolandic corona radiata. Whereas several compact white matter pathways were damaged in both the behavioral variant of frontotemporal dementia and corticobasal syndrome, the distribution and degree of white matter damage differed between them. These findings concur with the distinctive clinical manifestations of these conditions and may improve the in vivo neuroanatomical and diagnostic characterization of these

  2. Conversion of atrial fibrillation with ajmaline in a pregnant woman with Wolff-Parkinson-White syndrome.

    PubMed

    Mozo de Rosales, F; Moreno, J; Bodegas, A; Melchor, J C; Fernández LLebrez, L; Aranguren, G

    1994-07-01

    Pregnancy is related to an increased frequency of arrhythmias in asymptomatic patients with Wolff-Parkinson-White syndrome, which might lead to sudden death. A 40-year-old woman, with Wolff-Parkinson-White syndrome which was not diagnosed until pregnancy, presented in the 34th week with an atrial fibrillation, with high risk criteria for ventricular fibrillation. Intravenous ajmaline was given to convert the tachyarrhythmia to sinus rhythm. We obtained an excellent maternal control with no maternal or fetal adverse effects.

  3. [Unusual supraventricular tachycardias in Wolff-Parkinson-White syndrome].

    PubMed

    Belhassen, B; Laniado, S

    1983-01-01

    Two unusual types of narrow-QRS tachycardias were initiated during electrophysiological investigation of a patient with the Wolff-Parkinson-White syndrome. In the first type, the QRS complexes were preceded by atrial activation, the chronological sequence of depolarisation of which was similar to the sinus rhythm, suggesting the mechanism of sino-atrial reentry. The narrow QRS complex during tachycardia was related to the fact that the refractory period of the accessory pathway was longer than the tachycardia cycle. The second type of tachycardia was associated with I/I retrograde atrial activation within the QRS complex, suggesting the mechanism of intranodal reentry. The main point of interest of this case is that the accessory pathway, which only conducted in the anterograde direction, did not participate in the mechanism of either of these tachycardias.

  4. A Comparison of Challenging Behaviour in an Adult Group with Down's Syndrome and Dementia Compared with an Adult Down's Syndrome Group without Dementia

    ERIC Educational Resources Information Center

    Huxley, Adam; Van-Schaik, Paul; Witts, Paul

    2005-01-01

    This study investigated the frequency and severity of challenging behaviour in adults with Down's syndrome with and without signs of dementia. Care staff were interviewed using the Aberrant Behaviour Checklist-Community version (M.G. Aman & N.N. Singh, Slosson, East Aurora, NY, 1994), to investigate the frequency and severity of challenging…

  5. A Comparison of Challenging Behaviour in an Adult Group with Down's Syndrome and Dementia Compared with an Adult Down's Syndrome Group without Dementia

    ERIC Educational Resources Information Center

    Huxley, Adam; Van-Schaik, Paul; Witts, Paul

    2005-01-01

    This study investigated the frequency and severity of challenging behaviour in adults with Down's syndrome with and without signs of dementia. Care staff were interviewed using the Aberrant Behaviour Checklist-Community version (M.G. Aman & N.N. Singh, Slosson, East Aurora, NY, 1994), to investigate the frequency and severity of challenging…

  6. Grey matter volume correlates of cerebrospinal markers of Alzheimer-pathology in Parkinson's disease and related dementia.

    PubMed

    Compta, Yaroslau; Ibarretxe-Bilbao, Naroa; Pereira, Joana B; Junqué, Carme; Bargalló, Núria; Tolosa, Eduardo; Valldeoriola, Francesc; Muñoz, Esteban; Camara, Ana; Buongiorno, Mariateresa; Martí, Maria Jose

    2012-09-01

    Regional brain grey matter volume (GMV) reductions and abnormal cerebrospinal fluid (CSF) levels of τ and Aβ, extensively studied as biomarkers of Alzheimer's disease (AD), have also been reported in Parkinson's disease (PD) and related dementia (PDD). However, the relationship between these CSF and MRI biomarkers in PD and PDD remains unexplored. We studied these associations in 33 PD patients (18 with no dementia [PDND]; 15 fulfilling PDD criteria) and 12 neurologically unimpaired controls, with neuropsychological assessment, CSF ELISA studies, and voxel-based morphometry (VBM) analysis of high-field brain MRI. Neuropsychological assessment showed a gradation in cognitive performance from controls to PDND (significantly worse on visuospatial performance) and then to PDD (more impaired on memory, naming, fluency and visuospatial functions). No CSF-VBM correlations were found in controls or PDND patients. In contrast, in the analysis of both the PDD subgroup and the entire PD (PDND + PDD) sample, we found significant negative CSF-GMV correlations for τ and phospho-τ and significant positive CSF-GMV correlations for Aβ in mostly frontal and temporal structures. The correlations in the entire PD sample fitted with a linear model and were thus unlikely to have been driven solely by the PDD subgroup. Additionally, an association between both the CSF markers and the CSF-associated GMV reductions with several neuropsychological functions was found. We interpret that CSF markers of AD pathology are associated with VBM-measures of brain atrophy in PD-related dementia and within the PD cognitive continuum, and deserve further attention as putative biomarkers of cognitive impairment and dementia in PD. Copyright © 2012 Elsevier Ltd. All rights reserved.

  7. Cognition in Individuals at Risk for Parkinson's: Parkinson Associated Risk Syndrome (PARS) Study Findings

    PubMed Central

    Chahine, Lama M.; Weintraub, Daniel; Hawkins, Keith A.; Siderowf, Andrew; Eberly, Shirley; Oakes, David; Seibyl, John; Stern, Matthew B.; Marek, Kenneth; Jennings, Danna

    2016-01-01

    Objectives The Parkinson Associated Risk Syndrome Study identified a cohort of healthy adults with hyposmia and dopamine transporter binding reduction to characterize individuals at risk for Parkinson's disease (PD). We describe the cognitive profile of this cohort. Methods Individuals older than 50 y without PD were recruited. Two hundred twenty-five completed cognitive testing and were included in the final analysis. A neuropsychological test battery was administered and normative scores created for global cognition, memory, executive function/working memory, processing speed/attention, visuospatial abilities, and language domains. Other non-motor symptoms (constipation, depression, anxiety, and rapid eye movement sleep behavior disorder) were assessed through questionnaires. Results Individuals with both hyposmia and reduced dopamine transporter binding (n = 38) had lower mean scores for global cognition, executive function/working memory, and memory compared with all other participants (n = 187). In separate multivariate logistic regression models, lower global cognition (odds ratio, 1.97, P = 0.004), and specifically executive function/working memory (odds ratio, 1.84, P = 0.004) scores were associated with membership in the hyposmia with dopamine transporter reduction group. Combining hyposmia with relative impairment on specific cognitive domains increased the odds of dopamine transporter binding reduction compared with hyposmia alone, with the greatest increase in odds for hyposmia plus executive function/working memory relative impairment (68% increase in odds from 4.14 to 6.96). Conclusion Changes in global cognitive abilities, and specifically executive function/working memory, are present in individuals at risk for PD. Combining non-motor features, including cognition, improves prediction of dopamine transporter binding reduction. PMID:26293177

  8. Variability of the Aging Process in Dementia-Free Adults with Down Syndrome

    ERIC Educational Resources Information Center

    Tsao, Raphaele; Kindelberger, Cecile; Freminville, Benedicte; Touraine, Renaud; Bussey, Gerald

    2015-01-01

    The aim of this cross-sectional study was to analyze the typical aging process in adults with Down syndrome, focusing on its variability. The sample comprised 120 adults with Down syndrome who were free of dementia. Ages ranged from 20 to 69 years. Each participant was assessed on cognitive functioning and social adaptation, and was checked for…

  9. Down Syndrome and Dementia: Is Depression a Confounder for Accurate Diagnosis and Treatment?

    ERIC Educational Resources Information Center

    Wark, Stuart; Hussain, Rafat; Parmenter, Trevor

    2014-01-01

    The past century has seen a dramatic improvement in the life expectancy of people with Down syndrome. However, research has shown that individuals with Down syndrome now have an increased likelihood of early onset dementia. They are more likely than their mainstream peers to experience other significant co-morbidities including mental health…

  10. Health Co-Morbidities in Ageing Persons with Down Syndrome and Alzheimer's Dementia

    ERIC Educational Resources Information Center

    McCarron, M.; Gill, M.; McCallion, P.; Begley, C.

    2005-01-01

    Consideration of the relationship between physical and mental health co-morbidities in ageing persons with Down syndrome (DS) and Alzheimer's dementia (AD) is of clinical importance both from a care and resource perspective. To investigate and measure health co-morbidities in ageing persons with Down syndrome with and without AD. Recorded physical…

  11. Down Syndrome and Dementia: Is Depression a Confounder for Accurate Diagnosis and Treatment?

    ERIC Educational Resources Information Center

    Wark, Stuart; Hussain, Rafat; Parmenter, Trevor

    2014-01-01

    The past century has seen a dramatic improvement in the life expectancy of people with Down syndrome. However, research has shown that individuals with Down syndrome now have an increased likelihood of early onset dementia. They are more likely than their mainstream peers to experience other significant co-morbidities including mental health…

  12. Health Co-Morbidities in Ageing Persons with Down Syndrome and Alzheimer's Dementia

    ERIC Educational Resources Information Center

    McCarron, M.; Gill, M.; McCallion, P.; Begley, C.

    2005-01-01

    Consideration of the relationship between physical and mental health co-morbidities in ageing persons with Down syndrome (DS) and Alzheimer's dementia (AD) is of clinical importance both from a care and resource perspective. To investigate and measure health co-morbidities in ageing persons with Down syndrome with and without AD. Recorded physical…

  13. Variability of the Aging Process in Dementia-Free Adults with Down Syndrome

    ERIC Educational Resources Information Center

    Tsao, Raphaele; Kindelberger, Cecile; Freminville, Benedicte; Touraine, Renaud; Bussey, Gerald

    2015-01-01

    The aim of this cross-sectional study was to analyze the typical aging process in adults with Down syndrome, focusing on its variability. The sample comprised 120 adults with Down syndrome who were free of dementia. Ages ranged from 20 to 69 years. Each participant was assessed on cognitive functioning and social adaptation, and was checked for…

  14. Apathy and Related Executive Syndromes in Dementia Associated with Parkinson’s Disease and in Alzheimer’s Disease

    PubMed Central

    Grossi, Dario; Santangelo, Gabriella; Barbarulo, Anna Maria; Vitale, Carmine; Castaldo, Giovanna; Proto, Maria Grazia; Siano, Pietro; Barone, Paolo; Trojano, Luigi

    2013-01-01

    Apathy is defined as a lack of motivation and has been reported to be common in Alzheimer’s disease (AD) and Parkinson's disease (PD). To explore the neuropsychological correlates of apathy in patients with PD related dementia (PDD) and AD and to identify the specific cognitive profile of apathy in the two forms of neurodegenerative disease, 61 non-depressed patients (29 PDD and 32 AD) were selected. Out of these, 29 patients (47.5%) were detected as apathetic (14 PDD-A+ and 15 AD-A+), and 32 patients as non-apathetic (15 PDD-A- and 17 AD-A-). All patients underwent cognitive tasks tapping memory, visuospatial and executive functions, behavioral rating scales and Clinical Judgment for Apathy Syndrome (CJ-AS), an inventory developed to measure severity of apathy. The four subgroups differed significantly on memory and frontal tasks. The PDD-A+ performed significantly worse than PDD-A- on frontal tasks. The AD-A+ had poorer performance than AD-A- on frontal tasks. Last, PDD-A+ achieved significantly higher scores than AD-A+ on memory tasks. The four groups differed significantly on CJ-AS and behavioral rating scales. The results showed that apathetic patients with both forms of dementia showed a common neuropsychological and behavioral picture, characterized by defects on frontal tasks, thus strongly supporting the existence of an ‘apathetic syndrome’, characterized by specific cognitive and psychological symptoms. PMID:23242363

  15. Presenile dementia syndromes: an update on taxonomy and diagnosis

    PubMed Central

    Greicius, M; Geschwind, M; Miller, B

    2002-01-01

    The four major degenerative dementias that often begin in presenescence: are reviewed. These are Alzheimer's disease, frontotemporal dementia, dementia with Lewy bodies, and Creutzfeldt–Jakob disease. Their epidemiological, genetic, and clinical features are reviewed, and controversies in taxonomy arising from recent discoveries described. Particular attention is given to the pathological role of protein aggregation, which appears to be a factor in each disease. PMID:12023408

  16. Restless legs syndrome in Korean patients with drug-naïve Parkinson's disease: a nation-wide study.

    PubMed

    Shin, Hee-Young; Youn, Jinyoung; Yoon, Won Tae; Kim, Ji Sun; Cho, Jin Whan

    2013-03-01

    Restless legs syndrome is a common neurologic disorder, and there is increasing evidence for a dopaminergic link between Parkinson's disease and restless legs syndrome. However, most previous studies did not take into account the effects of dopaminergic medication. We conducted a nation-wide, cross-sectional study to determine the prevalence and clinical characteristics of restless legs syndrome in Korean drug-naïve Parkinson's disease patients. One hundred and fifty-one drug-naïve patients with Parkinson's disease were enrolled from 18 centers in South Korea over the course of one year. Clinical profiles of parkinsonism, restless legs syndrome, psychiatric symptoms, and laboratory data were collected. The findings of subjects with and without restless legs syndrome were compared. The prevalence of restless legs syndrome in drug-naïve patients with Parkinson's disease was 16.5%. Subjects with restless legs syndrome had a higher mean Hoehn and Yahr stage and more severe limb parkinsonism, especially tremor. There was, however, no difference in iron metabolism between patients with and without restless legs syndrome. Analysis demonstrated that Beck's depression inventory score was associated with the severity of restless legs syndrome. Our study demonstrated an increased prevalence of restless leg syndrome in drug-naïve patients with Parkinson's disease than in the general population. Based on the association between parkinsonism and restless legs syndrome, and the unique characteristics of restless legs syndrome in patients with Parkinson's disease, we suggest that the pathophysiology of restless legs syndrome in Parkinson's disease differs from that in patients without Parkinson's disease. Copyright © 2012 Elsevier Ltd. All rights reserved.

  17. Dementia with Lewy bodies: current concepts.

    PubMed

    Buracchio, Teresa; Arvanitakis, Zoe; Gorbien, Martin

    2005-01-01

    As life expectancy continues to increase over time, dementia is becoming an increasingly more common problem and a major cause of disability in older persons. It is now more important than ever to identify and manage common causes of dementia given variations in disease course, treatments and the possibility for modification of risk factors. Dementia with Lewy bodies (DLB) is a dementia syndrome characterized by progressive cognitive decline, with fluctuating cognition, recurrent detailed and well-formed hallucinations, and parkinsonism. This article aims to provide an overview of current concepts of DLB, including a description of the key clinical features and neuropathology, neurochemistry, and genetics of DLB, then a discussion of the relationship of DLB with Alzheimer's disease and Parkinson's disease, and, finally, a summary of current management strategies available for this disorder.

  18. Mechanism and treatment of dropped head syndrome associated with parkinsonism.

    PubMed

    Oyama, Genko; Hayashi, Akito; Mizuno, Yoshikuni; Hattori, Nobutaka

    2009-03-01

    Dropped head syndrome (DHS) associated with parkinsonism is not frequent, but it markedly reduces the activities of daily living and is refractory. To elucidate the mechanism and treatment of DHS associated with parkinsonism, we assessed 28 parkinsonian patients with DHS (2 men and 26 women) by examining their clinical features and cervical-muscle-needle and surface electromyographic (EMG) recordings. We also evaluated the effects of lidocaine, muscle afferent block (MAB; 1% lidocaine mixed with ethanol), and botulinum toxin injected into the bilateral sternocleidomastoid muscles (SCMs), which were considered to be the affected muscles. In some patients, DHS occurred after the initiation or loading of dopamine agonists (less common after pergolide than cabergoline and pramipexole). Improvement was noted after a reduction in the dopamine agonist dose in some patients, and loading of l-dopa in others. Needle EMG revealed no evidence for weakness of the dorsal neck muscles. Surface EMG showed a gradual increase in SCMs activity upon passive head lifting. Lidocaine injection into SCMs markedly improved DHS, but the effect was temporary. The effect of botulinum toxin and MAB was not satisfactory. Whereas DHS could have a heterogeneous etiology, dopamine receptor sensitivity may play a role in its pathogenesis. For the treatment of DHS in parkinsonian patients, an increase in the dosage of l-dopa and a decrease in that of the dopamine agonist should be considered. Lidocaine injection (lidocaine test) could be useful for determining the most affected muscle before using botulinum toxin or MAB. Further studies are needed to examine the outcome of such treatments that include GPi-DBS.

  19. Capturing the costs of end-of-life care: comparisons of multiple sclerosis, Parkinson's disease, and dementia.

    PubMed

    McCrone, Paul

    2009-07-01

    The need for end-of-life care is going to increase substantially in many countries in the coming decades. In addition to considering the effectiveness of such care, it is also vital, given resource scarcity, to assess its costs and cost-effectiveness. Costs include the direct care inputs, other related services, and indirect costs, such as lost employment. These need to be measured in an appropriate way, and one relevant questionnaire is the Client Service Receipt Inventory. Studies of multiple sclerosis, Parkinson's disease, and dementia that have used this questionnaire are described in this article. What is clear is that the costs of providing end-of-life care can be high and that informal care from family members and friends accounts for a substantial amount of the overall costs.

  20. The history of the wolff-Parkinson-white syndrome.

    PubMed

    Scheinman, Melvin M

    2012-07-01

    While Drs Wolff, Parkinson, and White fully described the syndrome in 1930, prior case reports had described the essentials. Over the ensuing century this syndrome has captivated the interest of anatomists, clinical cardiologists, and cardiac surgeons. Stanley Kent described lateral muscular connections over the atrioventricular (AV) groove which he felt were the normal AV connections. The normal AV connections were, however, clearly described by His and Tawara. True right-sided AV connections were initially described by Wood et al., while Öhnell first described left free wall pathways. David Scherf is thought to be the first to describe our current understanding of the pathogenesis of the WPW syndrome in terms of a re-entrant circuit involving both the AV node-His axis as well as the accessory pathway. This hypothesis was not universally accepted, and many theories were applied to explain the clinical findings. The basics of our understanding were established by the brilliant work of Pick, Langendorf, and Katz who by using careful deductive analysis of ECGs were able to define the basic pathophysiological processes. Subsequently, Wellens and Durrer applied invasive electrical stimulation to the heart in order to confirm the pathophysiological processes. Sealy and his colleagues at Duke University Medical Center were the first to successfully surgically divide an accessory pathway and ushered in the modern era of therapy for these patients. Morady and Scheinman were the first to successfully ablate an accessory pathway (posteroseptal) using high-energy direct-current shocks. Subsequently Jackman, Kuck, Morady, and a number of groups proved the remarkable safety and efficiency of catheter ablation for pathways in all locations using radiofrequency energy. More recently, Gollob et al. first described the gene responsible for a familial form of WPW. The current ability to cure patients with WPW is due to the splendid contributions of individuals from diverse

  1. COMPARATIVE EFFECTIVENESS OF MCI and DEMENTIA TREATMENTS IN A COMMUNITY-BASED DEMENTIA PRACTICE

    ClinicalTrials.gov

    2016-08-04

    Mild Cognitive Impairment; Dementia; Hypoxia; Hyperhomocysteinemia; Vitamin B 12 Deficiency; Iron Deficiency; Anemia; TBI; Neurodegenerative Disorders; Alzheimer's Disease; Vascular Dementia; Brain Injuries; Tauopathies; Parkinson's Disease; Lewy Body Dementia; Frontotemporal Dementia; TDP-43 Proteinopathies

  2. How We Developed a Multidisciplinary Screening Project for People with Down's Syndrome Given the Increased Prevalence of Early Onset Dementia

    ERIC Educational Resources Information Center

    Jervis, Nicola; Prinsloo, Linda

    2008-01-01

    There has been much research that has identified an increased prevalence of Dementia in adults with Down's syndrome when compared with the general population. Neuropathological changes associated with Alzheimer's dementia in the brain have been found in most people with Down's syndrome who die over the age of 35 years. Given the limitations of…

  3. Pushing and pulling with the upper extremities while standing: the effects of mild Alzheimer dementia and Parkinson's disease.

    PubMed

    Elble, R J; Leffler, K

    2000-03-01

    Eleven patients with mild dementia of Alzheimer type, 12 patients with mild to moderate Parkinson disease, and 27 control subjects of comparable age, education, and gender pushed or pulled on a rigid horizontal bar while maintaining stable erect stance. A target window (target force +/-10% maximum force) and a bar force cursor were displayed on a video screen, and subjects were asked to place the bar force cursor within the target window as quickly and as accurately as possible holding the target window for at least 1 sec. The target forces were 50% and 75% maximum force for each person, and three 4.0-sec push trials and three 4.0-sec pull trials were performed for each target force. Moments of force (torque), body motion, and extremity electromyography were measured with a computerized motion analysis system. The patients with Alzheimer's disease had only slightly lower Mini Mental State Examination (MMSE) scores (mean +/- standard deviation [SD] = 25.0 +/- 2.3) than the patients with Parkinson's disease (28.8 +/- 1.5) and control subjects (28.7 +/- 1.3). The patients with Alzheimer's disease had upper limb reaction times (0.827 +/- 0.399 sec) that were greater than those of the patients with Parkinson's disease (0.672 +/- 0.315 sec) and control subjects (0.606 +/- 0.263 sec). Furthermore, the patients with Alzheimer's disease achieved the designated target in only 46.2% of trials, which was comparable to the performance of the patients with Parkinson's disease (55.6%) but inferior to the control subjects (80.6%). Movement times did not differ significantly. The patients and control subjects initiated movement with comparable anticipatory postural activity in the lower limbs. The poor success rates of the patients with Alzheimer's disease and the patients with Parkinson's disease were attributable to inadequate visually guided adjustments in force after the initial movement began. This difficulty in making quick motor adjustments may be relevant to the tendency of

  4. [Wolff-Parkinson-White syndrome in a case of acute rejection of cardiac transplantation].

    PubMed

    Ollitrault, J; Daubert, J C; Ramée, M P; Ritter, P; Mabo, P; Leguerrier, A; Rioux, C; Logeais, Y

    1990-09-01

    A Wolff-Parkinson-White syndrome was observed during acute rejection in a patient who had undergone orthotopic cardiac transplantation. The sometimes intermittent nature of this syndrome could explain its postoperative appearance in this patient; the relationship with the episode of rejection is discussed.

  5. Clock drawing test: is it useful for dementia screening in patients having Parkinson disease with and without depression?

    PubMed

    Riedel, Oliver; Klotsche, Jens; Förstl, Hans; Wittchen, Hans-Ulrich

    2013-09-01

    Despite the wide use of clock drawing tests (CDTs) for screening cognitive impairment, their use in patients having Parkinson disease (PD) with dementia has not been systematically investigated until date. In this cross-sectional study, neurological and neuropsychiatric statuses of 1449 outpatients having PD with and without dementia were comprehensively assessed. The CDT revealed cognitive impairment in 42.7% of the 1383 patients whose drawings were available. Overall, CDT sensitivity and specificity were 70.7% and 68.9%, respectively. The positive and negative predictive values were 48.0% and 85.3%, respectively. In patients with depression, CDT specificity dropped significantly to 55.8% (71.3% in nondepressed patients, P < .001). Classification performance was not impacted by motor symptoms. The estimated classification performances and predictive values correspond to those reported previously for non-PD populations. Our results indicate that CDT is a suitable screening instrument in patients with PD, but test results from patients with depression warrant careful consideration.

  6. Development of an ultra-high sensitive immunoassay with plasma biomarker for differentiating Parkinson disease dementia from Parkinson disease using antibody functionalized magnetic nanoparticles.

    PubMed

    Yang, Shieh-Yueh; Chiu, Ming-Jang; Lin, Chin-Hsien; Horng, Herng-Er; Yang, Che-Chuan; Chieh, Jen-Jie; Chen, Hsin-Hsien; Liu, Bing-Hsien

    2016-06-08

    It is difficult to discriminate healthy subjects and patients with Parkinson disease (PD) or Parkinson disease dementia (PDD) by assaying plasma α-synuclein because the concentrations of circulating α-synuclein in the blood are almost the same as the low-detection limit using current immunoassays, such as enzyme-linked immunosorbent assay. In this work, an ultra-sensitive immunoassay utilizing immunomagnetic reduction (IMR) is developed. The reagent for IMR consists of magnetic nanoparticles functionalized with antibodies against α-synuclein and dispersed in pH-7.2 phosphate-buffered saline. A high-Tc superconducting-quantum-interference-device (SQUID) alternative-current magnetosusceptometer is used to measure the IMR signal of the reagent due to the association between magnetic nanoparticles and α-synuclein molecules. According to the experimental α-synuclein concentration dependent IMR signal, the low-detection limit is 0.3 fg/ml and the dynamic range is 310 pg/ml. The preliminary results show the plasma α-synuclein for PD patients distributes from 6 to 30 fg/ml. For PDD patients, the concentration of plasma α-synuclein varies from 0.1 to 100 pg/ml. Whereas the concentration of plasma α-synuclein for healthy subjects is significantly lower than that of PD patients. The ultra-sensitive IMR by utilizing antibody-functionalized magnetic nanoparticles and high-Tc SQUID magnetometer is promising as a method to assay plasma α-synuclein, which is a potential biomarker for discriminating patients with PD or PDD.

  7. Restless legs syndrome: an early clinical feature of Parkinson disease in men.

    PubMed

    Wong, Janice C; Li, Yanping; Schwarzschild, Michael A; Ascherio, Alberto; Gao, Xiang

    2014-02-01

    The association between restless legs syndrome (RLS) and Parkinson disease has been extensively studied, but the temporal relationship between the two remains unclear. We thus conduct the first prospective study to examine the risk of developing Parkinson disease in RLS. Prospective study from 2002-2010. United States. There were 22,999 US male health professionals age 40-75 y enrolled in the Health Professionals Follow-up Study without Parkinson disease, arthritis, or diabetes mellitus at baseline. RLS was assessed in 2002 using a set of standardized questions recommended by the International RLS Study Group. Incident Parkinson disease was identified by biennial questionnaires and then confirmed by review of participants' medical records by a movement disorder specialist. We documented 200 incident Parkinson disease cases during 8 y of follow-up. Compared to men without RLS, men with RLS symptoms who had symptoms greater than 15 times/mo had higher risk of Parkinson disease development (adjusted relative risk = 1.47; 95% confidence interval: 0.59, 3.65; P = 0.41). This was statistically significant only for cases diagnosed within 4 y of follow-up (adjusted relative risk = 2.77; 95% confidence interval: 1.08, 7.11; P = 0.03). Severe restless legs syndrome may be an early feature of Parkinson disease.

  8. [A 74-year-old woman with parkinsonism and dementia who died four years after the onset].

    PubMed

    Okuma, Y; Hattori, N; Nobukawa, B; Mori, H; Suda, K; Takubo, H; Mizuno, Y

    1998-07-01

    We report a 74-year-old woman with parkinsonism and dementia, who died 4 years after the onset of the disease. She was well until 70 years of the age (1993) when she noted slowness in the movement in her left hand. She also developed gait disturbance and the similar symptoms spread to the right upper and lower extremities. Two years after the onset, she had difficulty in walk, and was admitted to our hospital on March 9, 1995. Her daughter had the onset of hand tremor at 50 years of the age and gait disturbance at 52. Her gait improved after levodopa treatment, but her MRI revealed a liner T2-high signal lesion along the outer surface of each putamen. On admission, the patient was alert but slighted demented. Higher cerebral functions were normal. She had a masked face and small voice. Her gait was of small step without arm swing. Retropulsion was present. Rigidity was noted in the neck but not in the limbs. She was bradykinetic but tremor was absent. She was treated with levodopa/carbidopa, dops, and bromocriptine with considerable improvement and was discharged on March 30, 1995. On January 19, 1996, she developed fever and hallucination; she became more akinetic and admitted again. She showed marked dementia and stage IV parkinsonism. She was treated by supportive measures with improvement in the general condition, but she was found to have a gastric cancer for which a subtotal gastrectomy was performed on March 11, 1996. Post-operative course was uneventful, but her parkinsonism progressed to stage V. She was transferred to another hospital on May 13, 1996. In July 21, 1996, she developed dyspnea and fever and was admitted to our hospital again. She was somnolent. Rigidity was moderate to marked and she was unable to stand or walk. By supportive cares, her general condition improved and was discharged to home on November 4, 1996. She developed fever on June 13, 1997 and admitted to our service again. Her BP was 150/90 mmHg. She was alert but markedly demented

  9. Restless Legs Syndrome and Leg Motor Restlessness in Parkinson's Disease

    PubMed Central

    Suzuki, Keisuke; Miyamoto, Masayuki; Miyamoto, Tomoyuki; Hirata, Koichi

    2015-01-01

    Sleep disturbances are important nonmotor symptoms in Parkinson's disease (PD) that are associated with a negative impact on quality of life. Restless legs syndrome (RLS), which is characterized by an urge to move the legs accompanied by abnormal leg sensations, can coexist with PD, although the pathophysiology of these disorders appears to be different. RLS and PD both respond favorably to dopaminergic treatment, and several investigators have reported a significant relationship between RLS and PD. Sensory symptoms, pain, motor restlessness, akathisia, and the wearing-off phenomenon observed in PD should be differentiated from RLS. RLS in PD may be confounded by chronic dopaminergic treatment; thus, more studies are needed to investigate RLS in drug-naïve patients with PD. Recently, leg motor restlessness (LMR), which is characterized by an urge to move the legs that does not fulfill the diagnostic criteria for RLS, has been reported to be observed more frequently in de novo patients with PD than in age-matched healthy controls, suggesting that LMR may be a part of sensorimotor symptoms intrinsic to PD. In this paper, we provide an overview of RLS, LMR, and PD and of the relationships among these disorders. PMID:26504610

  10. [Outcome of 195 patients with Wolff-Parkinson-White syndrome].

    PubMed

    Brembilla-Perrot, B; Aliot, E; Louis, P; Terrier de la Chaise, A; Khalife, K; Marçon, F; Cherrier, F; Gilgenkrantz, J M; Pernot, C

    1987-03-01

    Between 1974 and 1984, 207 patients with Wolff-Parkinson-White syndrome (WPW) were admitted to our hospital department; 195 of them were followed up for periods ranging from 1 to 12 years (6 years in children, 3 years and 9 months in adults on average); 160 had undergone electrophysiological exploration. Fifty-seven patients were less than 16 years old: 7 died, including 6 with associated congenital heart disease; an asymptomatic 12-year old girl died suddenly while taking part in a sporting event. The signs of WPW disappeared in 5 out of 10 children under 1 year of age. One hundred and thirty-eight patients were older than 15: 15 of them died, but only 3 deaths were related to WPW: one was consecutive to surgery for WPW and one to fulguration; the third patient died of WPW tachyarrhythmia; the refractory period of his Kent's bundle was short, but his compliance with treatment was irregular. We found no correlation between changes in functional symptoms and Kent's bundle refractory period values; paradoxically, the frequency of attacks and resistance to treatment was higher in cases with long refractory period. On the whole, this series confirms that WPW usually is a benign disease. However, the risk of sudden death, of which it offers an example, indicates that all patients with WPW should be evaluated with at least an exercise test and, depending on its results or on the socio-professional context, an electrophysiological exploration.

  11. [Oral propranolol in Wolff-Parkinson-White syndrome. Electrophysiological data].

    PubMed

    Dolla, E; Levy, S; Cointe, R; Moyal, C; Bru, P; Rossi, P; Gérard, R

    1991-07-01

    The effects of oral propranolol were studied in 24 patients with the WPW syndrome. The average daily dose of propranolol was 130 +/- 24 mg administered in 3 doses over a period of 48 to 72 hours. Endocavitary electrophysiological study was performed 2 to 4 hours after the last dose. The effective anterograde refractory periods (EARP) of the accessory and normal pathways were measured before and after propranolol (and, in both studies, before and after isoproterenol). The EARP of the accessory pathway was not affected by the propranolol. However, in the 9 patients in whom its value was less than 270 ms, it increased significantly (p = 0.01). The EARP of the accessory pathway measured after administration of isoproterenol increased significantly in all patients with oral propranolol (p = 0.001). Sustained reciprocating tachycardia could be induced in 19 patients and non-sustained reciprocating tachycardia in 5 other patients during base line electrophysiological study. Oral propranolol prevented the induction of the tachycardias in 18 patients (75%), even after isoproterenol. The shortest R-R interval between two pre-excited complexes in atrial fibrillation increased after propranolol (283 +/- 45 to 343 +/- 95 ms). These results show that oral propranolol increases the EARP of the accessory pathway and the shortest R-R interval between two pre-excited complexes in atrial fibrillation in patients with short anterograde refractory periods of their accessory pathways, and is effective in preventing reciprocating tachycardia. Oral propranolol may be useful and can be used safely in patients with the Wolff-Parkinson-White syndrome.

  12. [Ventricular fibrillation in Wolff-Parkinson-White syndrome. Predictive factors].

    PubMed

    Attoyan, C; Haissaguerre, M; Dartigues, J F; Le Métayer, P; Warin, J F; Clémenty, J

    1994-07-01

    The incidence of sudden death in the Wolff-Parkinson-White (WPW) syndrome is not well documented and probably underestimated. This retrospective study concerned 28 consecutive patients presenting with ventricular fibrillation either spontaneously (20) or during electrophysiological investigation (8) but whose characteristics allowed them to be assimilated into a single group. Their clinical and electrophysiological characteristics were compared with those of 60 consecutive patients with the WPW syndrome who had documented atrial fibrillation (and even reciprocating tachycardia) but never ventricular fibrillation. There were no significant differences between the two groups with respect to the following clinical parameters: sex, duration of symptoms, the type of tachycardia previously recorded, history of syncope and presence of underlying cardiac disease. With respect to the electrophysiological data, there were no differences in the point of anterograde block, the effective anterograde refractory period of the accessory pathway, the effective and functional refractory periods of the right atrium and atrial vulnerability. On the other hand, a significant difference was observed in the age of patients with ventricular fibrillation (29 +/- 13 years vs 36 +/- 12 years; p < 0.02), the prevalence of multiple accessory pathways (25% vs 7%; p < 0.04) with a dominant localisation in the postero-septal region (75% vs 47%, p < 0.026), preexcitation during exercise stress testing and under antiarrhythmic therapy (95% vs 68%, p < 0.037). The most discriminating parameter was the shorter RR interval during atrial fibrillation (172 +/- 23 ms vs 230 +/- 50 ms, p < 0.008). Multivariate analysis only showed one independent predictive factor: the minimum preexcited RR interval.(ABSTRACT TRUNCATED AT 250 WORDS)

  13. Structure of tau exon 10 splicing regulatory element RNA and destabilization by mutations of frontotemporal dementia and parkinsonism linked to chromosome 17

    PubMed Central

    Varani, Luca; Hasegawa, Masato; Spillantini, Maria Grazia; Smith, Michael J.; Murrell, Jill R.; Ghetti, Bernardino; Klug, Aaron; Goedert, Michel; Varani, Gabriele

    1999-01-01

    Coding region and intronic mutations in the tau gene cause frontotemporal dementia and parkinsonism linked to chromosome 17. Intronic mutations and some missense mutations increase splicing in of exon 10, leading to an increased ratio of four-repeat to three-repeat tau isoforms. Secondary structure predictions have led to the proposal that intronic mutations and one missense mutation destabilize a putative RNA stem-loop structure located close to the splice-donor site of the intron after exon 10. We have determined the three-dimensional structure of this tau exon 10 splicing regulatory element RNA by NMR spectroscopy. We show that it forms a stable, folded stem-loop structure whose thermodynamic stability is reduced by frontotemporal dementia and parkinsonism linked to chromosome 17 mutations and increased by compensatory mutations. By exon trapping, the reduction in thermodynamic stability is correlated with increased splicing in of exon 10. PMID:10393977

  14. Cross-sectional and longitudinal associations of motor fluctuations and non-motor predominance with cerebrospinal τ and Aβ as well as dementia-risk in Parkinson's disease.

    PubMed

    Modreanu, Raluca; Cerquera, Sonia Catalina; Martí, María José; Ríos, José; Sánchez-Gómez, Almudena; Cámara, Ana; Fernández, Manel; Compta, Yaroslau

    2017-02-15

    Experimental, neuropathological and cerebrospinal fluid (CSF) studies support τ and amyloid-β (Aβ) relevance in Parkinson's disease (PD) related dementia. Lesser motor fluctuations (MFs) and non-motor features have also been related to PD-dementia. Yet, little is known about the association of MFs and non-motor symptoms with CSF τ and Aβ in PD. We hypothesized that lesser MFs and non-motor predominance are related to these CSF markers and dementia-risk in PD. We studied 58 PD patients (dementia at baseline, n=21; dementia at 18-months, n=35) in whom CSF Aβ and τ had been determined with ELISA techniques. MFs and a number of non-motor symptoms (apathy, anxiety, irritability, depression, visual hallucinations, spatial disorientation, memory complaints) over disease course were dichotomized as absent-mild vs. moderate-severe by retrospective clinical chart review blind to CSF findings. Non-motor predominance was defined as ≥3 non-motor symptoms (after the cohort-median of non-motor symptoms per patient) with ≥2 being moderate-severe and ≥1 having been present from onset, with all these being more disabling overall than motor features. Cross-sectionally, CSF biomarkers were non-parametrically compared according to dichotomized MFs and non-motor predominance. Longitudinally, dementia was the outcome (dependent variable), CSF markers, MFs and non-motor predominance were the predictors (independent variables), and potential modifiers as age, sex, and memory complaints were the covariates in binary regression models. Absent-mild MFs were associated with higher CSF τ markers and shorter time-to-dementia, while non-motor predominance and decreasing CSF Aβ independently increased longitudinal dementia-risk. In summary, absent-mild MFs, non-motor predominance and CSF τ and Aβ might define endophenotypes related to the timing or risk of dementia in PD.

  15. Spousal Caregivers of Persons with Alzheimer's and Parkinson's Disease Dementia: A Preliminary Comparison.

    ERIC Educational Resources Information Center

    Dura, Jason R.; And Others

    1990-01-01

    Compared self- and other-rated depression in spousal caregivers for 23 Alzheimer's patients, 23 Parkinsons' Disease patients, and 23 control subjects. Two caregiver groups were similar in length of time they had been giving care and in caregiver distress and both caregiver groups were more depressed than control subjects. (Author/NB)

  16. Slowing of oscillatory brain activity is a stable characteristic of Parkinson's disease without dementia.

    PubMed

    Stoffers, D; Bosboom, J L W; Deijen, J B; Wolters, E C; Berendse, H W; Stam, C J

    2007-07-01

    Extensive changes in resting-state oscillatory brain activity have recently been demonstrated using magnetoencephalography (MEG) in moderately advanced, non-demented Parkinson's disease patients relative to age-matched controls. The aim of the present study was to determine the onset and evolution of these changes over the disease course and their relationship with clinical parameters. In addition, we evaluated the effects of dopaminomimetics on resting-state oscillatory brain activity in levodopa-treated patients. MEG background oscillatory activity was studied in a group of 70 Parkinson's disease patients with varying disease duration and severity (including 18 de novo patients) as well as in 21 controls that were age-matched to the de novo patients. Whole head 151-channel MEG recordings were obtained in an eyes-closed resting-state condition. Levodopa-treated patients (N = 37) were examined both in a practically defined 'OFF' as well as in the 'ON' state. Relative spectral power was calculated for delta, theta, low alpha, high alpha, beta and gamma frequency bands and averaged for 10 cortical regions of interest (ROIs). Additionally, extensive clinical and neuropsychological testing was performed in all subjects. De novo Parkinson's disease patients showed widespread slowing of background MEG activity relative to controls. Changes included a widespread increase in theta and low alpha power, as well as a loss of beta power over all but the frontal ROIs and a loss of gamma power over all but the right occipital ROI. Neuropsychological assessment revealed abnormal perseveration in de novo patients, which was associated with increased low alpha power in centroparietal ROIs. In the whole group of Parkinson's disease patients, longer disease duration was associated with reduced low alpha power in the right temporal and right occipital ROI, but not with any other spectral power measure. No association was found between spectral power and disease stage, disease severity

  17. Memantine for patients with Parkinson's disease dementia or dementia with Lewy bodies: a randomised, double-blind, placebo-controlled trial.

    PubMed

    Emre, Murat; Tsolaki, Magda; Bonuccelli, Ubaldo; Destée, Alain; Tolosa, Eduardo; Kutzelnigg, Alexandra; Ceballos-Baumann, Andrés; Zdravkovic, Slobodan; Bladström, Anna; Jones, Roy

    2010-10-01

    Previous studies have suggested that patients with Lewy-body-related dementias might benefit from treatment with the N-methyl D-aspartate receptor antagonist memantine, but further data are needed. Therefore, the efficacy and safety of memantine were investigated in patients with mild to moderate Parkinson's disease dementia (PDD) or dementia with Lewy bodies (DLB). Patients (≥50 years of age) with mild to moderate PDD or DLB were recruited from 30 specialist centres in Austria, France, Germany, the UK, Greece, Italy, Spain, and Turkey. They were randomly assigned to placebo or memantine (20 mg per day) according to a computer-generated list. Patients and all physicians who had contact with them were masked to treatment assignment. No primary endpoint was defined. Safety analyses were done for all patients who took at least one dose of memantine or placebo, and efficacy analyses were done for all patients who had at least one valid postbaseline assessment. This trial is registered with ClinicalTrials.gov, number NCT00855686. Of the 199 patients randomly assigned to treatment, 34 with DLB and 62 with PDD were given memantine, and 41 with DLB and 58 with PDD were given placebo. 159 (80%) patients completed the study: 80 in the memantine group and 79 in the placebo group. 93 patients treated with memantine and 97 patients treated with placebo were included in the efficacy analysis. At week 24, patients with DLB who received memantine showed greater improvement according to Alzheimer's disease cooperative study (ADCS)-clinical global impression of change scores than did those who received placebo (mean change from baseline 3·3 vs 3·9, respectively, difference -0·6 [95% CI -1·2 to -0·1]; p=0·023). No significant differences were noted between the two treatments in patients with PDD (3·6 with memantine vs 3·8 with placebo, -0·1 [-0·6 to 0·3]; p=0·576) or in the total population (3·5 with memantine vs 3·8 with placebo, -0·3 [-0·7 to 0·1]; p=0·120

  18. Atrial depolarization in Wolf-Parkinson-White and Lown-Ganong-Levine syndrome: vectorcardiographic features.

    PubMed

    Zoneraich, O; Zoneraich, S

    1979-07-01

    The atrial depolarization pattern was studied in 22 patients with Wolff-Parkinson-White and Lown-Ganong-Levine syndrome. The influence of the accessory pathways on the shape, magnitude and conduction pattern of the PSE loop was analyzed. An accurate evaluation of the beginning of the delta wave and of the P loop distortions was obtained by using high magnification (1 mV = 30 cm) recordings. The Frank lead system was used. The influence of atrial size (documented by echocardiography) on the PSE loop is emphasized. Special attention has been focused on the terminal vectors as compared to a control group. In Wolff-Parkinson-White syndrome the size of the PSE loop was smaller than in Lown-Ganong-Levine syndrome or in the normal group. When atrial conduction disturbances and/or atrial enlargement was present the PSE loop was larger and distorted. The terminal vectors were abnormally oriented in 75 percent of the patients with Wolff-Parkinson-White syndrome, but only in one with Lown-Ganong-Levine syndrome. The beginning of the delta wave in patients with Wolff-Parkinson-White syndrome was located to the left of the E point in all but two. When the "concertina" effect was present, the direction of the terminal vectors remained unchanged. In four patients with the Lown-Ganong-Levine syndrome, the PSE loop closed, and in three patients, a small opening was present. We suggest that the changes in contour, duration and amplitude of the PSE loop are due to an abnormal pattern of atrial depolorization in Wolff-Parkinson-White syndrome.

  19. [Sleep disorders and dementia].

    PubMed

    Hess, C W

    1997-08-27

    A clinically relevant sleep-wake disturbance is found in up to half the patients with dementia, and the sundowning agitation is a common cause of institutionalisation of demented geriatric patients. The circadian rhythm of demented patients is levelled off with increased daytime sleep and disrupted night sleep. Particularly in vascular dementia, Korsakow syndrome, Parkinson's disease, and depression the alteration of sleep architecture may be pronounced, whereas in Alzheimer's disease prominent hypersomnolence or insomnia is typically only found in later stages of the diseases. Greatly increased daytime sleepiness or striking insomnia at the very beginning of suspected dementia should thus prompt the search for other, possibly treatable causes of dementia. Neuropathological and neurophysiological studies support the hypothesis of a deteriorated hypothalamic suprachiasmatic nucleus (harbouring the biological clock) as a cause for the deranged circadian sleep-wake system in dementia. Management of sundowning behaviour includes restriction of daytime sleep, exposure to bright lights, and social interaction schedules during the day. The benzodiazepines and analogues usually not being sufficiently effectual, low doses of mild neuroleptics are often needed. Whether recent reports on efficacy of melatonin in elderly insomniacs also apply to demented patients is yet uncertain. The careful search and treatment of possible extracerebral physiologic factors causing reversible hypersomnia or insomnia is an important requisite. Polysomnographic studies are needed to recognise treatable sleep disturbance which could deteriorate or mimic dementia and sundowning. Particularly, a sleep-apnea-hypopnea syndrome must be searched for at the beginning of a suspected dementia, when successful treatment is still possible. Sleep studies should also identify periodic leg movements of sleep with restless legs and/or increased daytime sleepiness, and hyperkinetic parasomnias such as REM sleep

  20. Incidence and Prevalence of Dementia in Elderly Adults with Mental Retardation without Down Syndrome

    ERIC Educational Resources Information Center

    Zigman, Warren B.; Schupf, Nicole; Devenny, Darlynne A.; Miezejeski, Charles; Ryan, Robert; Urv, Tiina K.; Schubert, Romaine; Silverman, Wayne

    2004-01-01

    Rates of dementia in adults with mental retardation without Down syndrome were equivalent to or lower than would be expected compared to general population rates, whereas prevalence rates of other chronic health concerns varied as a function of condition. Given that individual differences in vulnerability to Alzheimer's disease have been…

  1. An Experimental Approach to Detecting Dementia in Down Syndrome: A Paradigm for Alzheimer's Disease

    ERIC Educational Resources Information Center

    Nelson, Linda D.; Scheibel, Kevin E.; Ringman, John M.; Sayre, James W.

    2007-01-01

    Measures developed from animal models of aging may detect dementia of the Alzheimer's type in a population at-risk for Alzheimer's disease (AD). Although, by middle age, individuals with Down syndrome (DS) show an extraordinarily high prevalence of AD-type pathology, their severe idiopathic cognitive deficits tend to confound the "clinical"…

  2. An Experimental Approach to Detecting Dementia in Down Syndrome: A Paradigm for Alzheimer's Disease

    ERIC Educational Resources Information Center

    Nelson, Linda D.; Scheibel, Kevin E.; Ringman, John M.; Sayre, James W.

    2007-01-01

    Measures developed from animal models of aging may detect dementia of the Alzheimer's type in a population at-risk for Alzheimer's disease (AD). Although, by middle age, individuals with Down syndrome (DS) show an extraordinarily high prevalence of AD-type pathology, their severe idiopathic cognitive deficits tend to confound the "clinical"…

  3. "It's All Changed:" Carers' Experiences of Caring for Adults Who Have Down's Syndrome and Dementia

    ERIC Educational Resources Information Center

    McLaughlin, Katrina; Jones, Aled

    2011-01-01

    A qualitative interview study was undertaken to determine the information and support needs of carers of adults who have Down's syndrome and dementia. The data were analysed thematically. Carers' information and support needs were seen to change at pre-diagnosis, diagnosis and post-diagnosis. Helping carers to manage the changing nature of the…

  4. Incidence and Prevalence of Dementia in Elderly Adults with Mental Retardation without Down Syndrome

    ERIC Educational Resources Information Center

    Zigman, Warren B.; Schupf, Nicole; Devenny, Darlynne A.; Miezejeski, Charles; Ryan, Robert; Urv, Tiina K.; Schubert, Romaine; Silverman, Wayne

    2004-01-01

    Rates of dementia in adults with mental retardation without Down syndrome were equivalent to or lower than would be expected compared to general population rates, whereas prevalence rates of other chronic health concerns varied as a function of condition. Given that individual differences in vulnerability to Alzheimer's disease have been…

  5. Psychologists' Clinical Practices in Assessing Dementia in Individuals with Down Syndrome

    ERIC Educational Resources Information Center

    Auty, Ellen; Scior, Katrina

    2008-01-01

    There are now ample guidelines for the assessment and diagnosis of possible dementia in individuals with intellectual disabilities (ID) and Down syndrome. However, little is known about their implementation in clinical practice. This study set out to examine the clinical practice of one key professional group, namely clinical psychologists. A…

  6. "It's All Changed:" Carers' Experiences of Caring for Adults Who Have Down's Syndrome and Dementia

    ERIC Educational Resources Information Center

    McLaughlin, Katrina; Jones, Aled

    2011-01-01

    A qualitative interview study was undertaken to determine the information and support needs of carers of adults who have Down's syndrome and dementia. The data were analysed thematically. Carers' information and support needs were seen to change at pre-diagnosis, diagnosis and post-diagnosis. Helping carers to manage the changing nature of the…

  7. Behavioural Excesses and Deficits Associated with Dementia in Adults Who Have Down Syndrome

    ERIC Educational Resources Information Center

    Oliver, Chris; Kalsy, Sunny; McQuillan, Sharna; Hall, Scott

    2011-01-01

    Background: Informant-based assessment of behavioural change and difference in dementia in Down syndrome can aid diagnosis and inform service delivery. To date few studies have examined the impact of different types of behavioural change. Methods: The Assessment for Adults with Developmental Disabilities (AADS), developed for this study, assesses…

  8. Behavioural Excesses and Deficits Associated with Dementia in Adults Who Have Down Syndrome

    ERIC Educational Resources Information Center

    Oliver, Chris; Kalsy, Sunny; McQuillan, Sharna; Hall, Scott

    2011-01-01

    Background: Informant-based assessment of behavioural change and difference in dementia in Down syndrome can aid diagnosis and inform service delivery. To date few studies have examined the impact of different types of behavioural change. Methods: The Assessment for Adults with Developmental Disabilities (AADS), developed for this study, assesses…

  9. [Electrophysiological characteristics of asymptomatic Wolff-Parkinson-White syndromes].

    PubMed

    Brembilla-Perrot, B; Ghawi, R; Dechaux, J P

    1991-11-01

    The management of the Wolff-Parkinson-White syndrome (WPW) is controversial especially when the patient is asymptomatic. The aim of this study was to evaluate the electrophysiological characteristics of such patients. Thirty two asymptomatic subjects with overt WPW on the surface ECG aged 14 to 68 years (average 36 +/- 15 years) underwent endocavitary or oesophageal electrophysiological study with the following protocol: programmed atrial stimulation using 1 or 2 extrastimuli over 3 cycles to evaluate the induction of paroxysmal junctional tachycardia and atrial fibrillation; atrial pacing at increasing frequencies to assess the shortest cycle conducted by the bundle of Kent. This protocol was repeated during intravenous infusion of 20 to 30 mg of Isoproterenol. Four electrophysiological characteristics were identified: the incidence of induction of junctional tachycardia was very low (2 cases, 6%); the incidence of induction of atrial fibrillation or tachycardia was similar to that of symptomatic WPW (9 cases 30%); the incidence of rapid conduction via the bundle of Kent (cycle conducted by the Kent less than 250 ms under basal conditions less than 200 ms with Isoproterenol) was 19% (6 cases); the incidence of potentially serious forms of WPW with rapid conduction in the bundle of Kent and atrial vulnerability (induction of atrial fibrillation at a frequency less than the Wenckebach point by programmed atrial stimulation) was similar to that in symptomatic WPW, 3 cases (10%). In conclusion, the asymptomatic character of the WPW is very probably due to the absence of junctional tachycardias. Nevertheless, these patients are at risk of atrial fibrillation with an incidence of potentially serious forms of 10%.(ABSTRACT TRUNCATED AT 250 WORDS)

  10. Atrial flutter with 1:1 conduction in undiagnosed Wolff-Parkinson-White syndrome.

    PubMed

    Nelson, Jessie G; Zhu, Dennis W

    2014-05-01

    Atrial flutter with 1:1 atrioventricular conduction via an accessory pathway is an uncommon presentation of Wolff-Parkinson-White syndrome not previously reported in the emergency medicine literature. Wolff-Parkinson-White syndrome, a form of ventricular preexcitation sometimes initially seen and diagnosed in the emergency department (ED), can present with varied tachydysrhythmias for which certain treatments are contraindicated. For instance, atrial fibrillation with preexcited conduction needs specific consideration of medication choice to avoid potential degeneration into ventricular fibrillation. We describe an adult female presenting with a very rapid, regular wide complex tachycardia successfully cardioverted in the ED followed by a normal electrocardiogram (ECG). Electrophysiology study confirmed atrial flutter with 1:1 conduction and revealed an accessory pathway consistent with Wolff-Parkinson-White syndrome, despite lack of ECG findings of preexcitation during sinus rhythm. Why should an emergency physician be aware of this? Ventricular tachycardia must be the first consideration in patients with regular wide complex tachycardia. However, clinicians should consider atrial flutter with 1:1 conduction related to an accessory pathway when treating patients with the triad of very rapid rate (>250 beats/min), wide QRS complex, and regular rhythm, especially when considering pharmacologic treatment. Emergency physicians also should be aware of electrocardiographically concealed accessory pathways, and that lack of delta waves does not rule out preexcitation syndromes such as Wolff-Parkinson-White syndrome. Copyright © 2014 Elsevier Inc. All rights reserved.

  11. [Surgical atrioventricular disconnection in Wolff-Parkinson-White syndrome].

    PubMed

    Pavie, A; Mesnildrey, P; Gandjbakhch, I; Cabrol, C; Fontaine, G; Franck, R; Grosgogeat, Y; Slama, R

    1984-06-01

    Surgical atrioventricular disconnection is a possible means of treating patients with severe paroxysmal arrhythmias resistant to medical treatment due to the Wolff-Parkinson-White syndrome. Between 1971 and April 1982 we operated 50 patients (38 men and 12 women) with the WPW syndrome. Thirty seven patients were operated for arrhythmias (paroxysmal tachycardia) resistant to medical therapy or with a high risk of sudden death. Thirteen patients had associated cardiac disease with less severe arrhythmias (aortic valve disease: 6 cases; mitral and aortic valve disease: 3 cases; mitral valve disease: 3 cases, and atrial septal defect: 1 case). The causes of paroxysmal tachycardia were atrial fibrillation (13 cases), atrial flutter (2 cases), orthodromic reciprocating tachycardia (30 cases), with associated atrial fibrillation in 9 cases, and with associated atrial flutter in 4 cases. Antidromic reciprocating tachycardia was present in 2 cases. In 3 cases, the preexcitation was a chance finding. Electrophysiological studies performed before and after antiarrhythmic drug administration showed type A WPW (LV preexcitation) in 23 cases, and type B WPW (RV preexcitation) in 20 cases. The ECG was normal in the horizontal plane in 7 cases. The atrioventricular accessory pathway was permeable in both directions in 39 cases; in 9 cases the pathway was permeable only in the retrograde direction and in 2 cases it was permeable only in the anterograde direction. In 7 patients an atrio-hisian short circuit was demonstrated. The site of the accessory conduction pathway was located by epicardial mapping, the first surgical stage, in the left lateral region of the atrioventricular junction (28 cases), in the right lateral region (6 cases), in the posterior septal region (15 cases) (right sided in 4 cases, left sided in 11 cases), and in the anterior septal region (1 case). The accessory pathway (so-called Bundle of Kent) was interrupted by atrioventricular disconnection. Six patients

  12. The Movement Disorders Society criteria for the diagnosis of Parkinson's disease dementia: their usefulness and limitations in elderly patients.

    PubMed

    Kiesmann, Michèle; Chanson, Jean-Baptiste; Godet, Julien; Vogel, Thomas; Schweiger, Laetitia; Chayer, Saïd; Kaltenbach, Georges

    2013-10-01

    The aim of this study was to assess the performance of the Movement Disorders Society (MDS) criteria for the diagnosis of Parkinson's disease dementia (PDD) in the elderly, and also to evaluate the relevance of applying other tests in this patient population. The MDS criteria include a first short part in checklist form, and a second part which is used as a basis for reference and consists of an in-depth neuropsychological examination. Forty consecutive PD patients presenting with cognitive complaints were enrolled. An assessment was made of the performances of the MDS checklist compared with the MDS exhaustive cognitive examination which was used as a basis for reference, and with other cognitive tests including the Mattis Dementia Rating Scale (MDRS), the French version of the Grober and Buschke test, the verbal fluency test, the Rey-Osterreith complex figure and the paced auditory serial addition test. Out of a total of 40 PD subjects (mean age: 80.5 ± 4.9 years), 20 were diagnosed with PDD according to the checklist and 31 on the basis of the exhaustive examination, i.e. with 11 more patients diagnosed via the latter. The sensitivity of the checklist for the diagnosis of PDD was 0.64, with a specificity of 1.00. The use of the MDRS for PDD diagnosis with a cut-off at ≤ 120 showed a sensitivity of 0.80 and a specificity of 1.00, while at ≤ 132 it displayed a sensitivity of 1.00 and a specificity of 0.444. The specificity of the checklist for the diagnosis of PDD in the elderly was confirmed, but it was lacking in sensitivity. It was also found that the MDRS could be helpful in the diagnosis and screening of PDD.

  13. Acceleration of ventricular rate by amiodarone in atrial fibrillation associated with the Wolff-Parkinson-White syndrome

    PubMed Central

    Sheinman, Bryan D; Evans, Tom

    1982-01-01

    Amiodarone has proved to be a valuable drug in atrial fibrillation associated with the Wolff-Parkinson-White syndrome. When it was administered to a patient with this syndrome in atrial fibrillation, who had previously suffered an inferior myocardial infarction, the ventricular rate accelerated from 170 to 230 beats/minute. This unusual case emphasises the need for full electrophysiological assessment of patients with the Wolff-Parkinson-White syndrome for whom amiodarone treatment is being considered. Imagesp1000-a PMID:6812745

  14. Polymorphisms in BACE2 may affect the age of onset Alzheimer's dementia in Down syndrome.

    PubMed

    Mok, Kin Y; Jones, Emma L; Hanney, Marisa; Harold, Denise; Sims, Rebecca; Williams, Julie; Ballard, Clive; Hardy, John

    2014-06-01

    It is known that Alzheimer's disease (AD) presents at an early age in people with Down syndrome (DS). The trisomy 21 in DS provides an opportunity to study the effect of duplicated genes in AD. APP and BACE2 are 2 genes located in chromosome 21 and related to AD. We looked into our cohort of 67 DS cases with dementia for the effect of BACE2 variants in age of onset of dementia. Of the 83 single-nucleotide polymorphisms (SNPs), 6 were associated with age of onset and another 8 SNPs were borderline associated. Our finding also replicated a previous study showing association of rs2252576 with AD.

  15. Acute parietal lobe infarction presenting as Gerstmann's syndrome and cognitive decline mimicking senile dementia.

    PubMed

    Chen, Tien-Yu; Chen, Chun-Yen; Yen, Che-Hung; Kuo, Shin-Chang; Yeh, Yi-Wei; Chang, Serena; Huang, San-Yuan

    2013-01-01

    Gerstmann's syndrome encompasses the tetrad of finger agnosia, agraphia, acalculia, and right-left confusion. An elderly man with a history of several cardiovascular diseases was initially brought to the psychiatric outpatient department by his family because of worsening of recent memory, executive function, and mixed anxious-depressive mood. Gerstmann's syndrome without obvious motor function impairment and dementia-like features could be observed at first. Emergent brain computed tomography scan revealed new left-middle cerebral artery infarction over the left posterior parietal lobe. This case reminds us that acute cerebral infarction involving the parietal lobe may present as Gerstmann's syndrome accompanied by cognitive decline mimicking dementia. As a result, emergent organic workups should be arranged, especially for elderly patients at high risk for cerebral vascular accident.

  16. Acute parietal lobe infarction presenting as Gerstmann’s syndrome and cognitive decline mimicking senile dementia

    PubMed Central

    Chen, Tien-Yu; Chen, Chun-Yen; Yen, Che-Hung; Kuo, Shin-Chang; Yeh, Yi-Wei; Chang, Serena; Huang, San-Yuan

    2013-01-01

    Gerstmann’s syndrome encompasses the tetrad of finger agnosia, agraphia, acalculia, and right-left confusion. An elderly man with a history of several cardiovascular diseases was initially brought to the psychiatric outpatient department by his family because of worsening of recent memory, executive function, and mixed anxious-depressive mood. Gerstmann’s syndrome without obvious motor function impairment and dementia-like features could be observed at first. Emergent brain computed tomography scan revealed new left-middle cerebral artery infarction over the left posterior parietal lobe. This case reminds us that acute cerebral infarction involving the parietal lobe may present as Gerstmann’s syndrome accompanied by cognitive decline mimicking dementia. As a result, emergent organic workups should be arranged, especially for elderly patients at high risk for cerebral vascular accident. PMID:23847420

  17. Delusional misidentification syndrome and other unusual delusions in advanced Parkinson's disease.

    PubMed

    Moro, Adriana; Munhoz, Renato Puppi; Moscovich, Mariana; Arruda, Walter O; Teive, Hélio A G

    2013-08-01

    Unusual delusional syndromes are rare protean diseases with speculative etiopathogenic mechanisms. Seven consecutive patients with advanced PD were evaluated over a 15-year period at the Movement Disorders Unit in the Neurology Service, Hospital de Clínicas, Federal University of Paraná, and the Paraná State Parkinson's Patients Association. We describe advanced Parkinson's disease patients presenting with unusual delusional syndromes, including cases of Ekbom, Othello, Capgras' and Diogenes syndromes, reduplicative paramnesia and mirrored-self misidentification. There are a few isolated reports of unusual neuropsychiatric disorders in patients with PD. We believe that these syndromes associated with advanced PD in elderly patients presenting with cognitive impairment and polypharmacy are probably often underestimated. Neurologists should be aware for these rare and treatable conditions. Copyright © 2013 Elsevier Ltd. All rights reserved.

  18. Parkinson-dementia complex and development of a new stable isotope dilution assay for BMAA detection in tissue

    SciTech Connect

    Snyder, Laura R.; Cruz-Aguado, Reyniel; Sadilek, Martin; Galasko, Douglas; Shaw, Christopher A.; Montine, Thomas J.

    2009-10-15

    {beta}-Methylamino-L-alanine (BMAA) has been proposed as a global contributor to neurodegenerative diseases, including Parkinson-dementia complex (PDC) of Guam and Alzheimer's disease (AD). The literature on the effects of BMAA is conflicting with some but not all in vitro data supporting a neurotoxic action, and experimental animal data failing to replicate the pattern of neurodegeneration of these human diseases, even at very high exposures. Recently, BMAA has been reported in human brain from individuals afflicted with PDC or AD. Some of the BMAA in human tissue reportedly is freely extractable (free) while some is protein-associated and liberated by techniques that hydrolyze the peptide bond. The latter is especially intriguing since BMAA is a non-proteinogenic amino acid that has no known tRNA. We attempted to replicate these findings with techniques similar to those used by others; despite more than adequate sensitivity, we were unable to detect free BMAA. Recently, using a novel stable isotope dilution assay, we again were unable to detect free or protein-associated BMAA in human cerebrum. Here we review the development of our new assay for tissue detection of BMAA and show that we are able to detect free BMAA in liver but not cerebrum, nor do we detect any protein-associated BMAA in mice fed this amino acid. These studies demonstrate the importance of a sensitive and specific assay for tissue BMAA and seriously challenge the proposal that BMAA is accumulating in human brain.

  19. Parkinson-dementia complex and development of a new stable isotope dilution assay for BMAA detection in tissue.

    PubMed

    Snyder, Laura R; Cruz-Aguado, Reyniel; Sadilek, Martin; Galasko, Douglas; Shaw, Christopher A; Montine, Thomas J

    2009-10-15

    Beta-methylamino-L-alanine (BMAA) has been proposed as a global contributor to neurodegenerative diseases, including Parkinson-dementia complex (PDC) of Guam and Alzheimer's disease (AD). The literature on the effects of BMAA is conflicting with some but not all in vitro data supporting a neurotoxic action, and experimental animal data failing to replicate the pattern of neurodegeneration of these human diseases, even at very high exposures. Recently, BMAA has been reported in human brain from individuals afflicted with PDC or AD. Some of the BMAA in human tissue reportedly is freely extractable (free) while some is protein-associated and liberated by techniques that hydrolyze the peptide bond. The latter is especially intriguing since BMAA is a non-proteinogenic amino acid that has no known tRNA. We attempted to replicate these findings with techniques similar to those used by others; despite more than adequate sensitivity, we were unable to detect free BMAA. Recently, using a novel stable isotope dilution assay, we again were unable to detect free or protein-associated BMAA in human cerebrum. Here we review the development of our new assay for tissue detection of BMAA and show that we are able to detect free BMAA in liver but not cerebrum, nor do we detect any protein-associated BMAA in mice fed this amino acid. These studies demonstrate the importance of a sensitive and specific assay for tissue BMAA and seriously challenge the proposal that BMAA is accumulating in human brain.

  20. Rapid assessment of cognitive function in down syndrome across intellectual level and dementia status.

    PubMed

    Walsh, D M; Doran, E; Silverman, W; Tournay, A; Movsesyan, N; Lott, I T

    2015-11-01

    Adults with Down syndrome (DS) are at risk of developing dementia and cognitive assessment is a fundamental part of the diagnostic process. Previously, we developed a Rapid Assessment for Developmental Disabilities (RADD), a brief, broadly focused direct test of cognition. In the current report, we assess whether the RADD is sensitive to dementia in DS and the degree to which it compares with other cognitive measures of dementia in this population. In a sample of 114 individuals with DS, with dementia diagnosed in 62%, the RADD was compared with the Dementia Questionnaire for Mentally Retarded Persons (DMR), the Bristol Activities of Daily Living Scale, Severe Impairment Battery (SIB), and the Brief Praxis Test (BPT). The RADD showed predicted effects across intellectual disability (ID) levels and dementia status (p < 0.001). Six-month test-retest reliability for the subset of individuals without dementia was high (r(41) = 0.95, p < 0.001). Criterion-referenced validity was demonstrated by correlations between RADD scores and ID levels based upon prior intelligence testing and clinical diagnoses (rs (114) = 0.67, p = 0.001) and with other measures of cognitive skills, such as the BPT, SIB, and DMR-Sum of Cognitive scores (range 0.84 through 0.92). Using receiver operating characteristic curves for groups varying in pre-morbid severity of ID, the RADD exhibited high sensitivity (0.87) and specificity (0.81) in discriminating among individuals with and without dementia, although sensitivity was somewhat lower (0.73) for the subsample of dementia cases diagnosed no more than 2 years prior to their RADD assessment. Taken together, findings indicated that the RADD, a relatively brief, easy-to-administer test for cognitive function assessment across ID levels and dementia status, would be a useful component of cognitive assessments for adults with DS, including assessments explicitly focused on dementia. © 2015 MENCAP and International Association of the Scientific

  1. Alternating Wolff-Parkinson-White syndrome associated with attack of angina

    SciTech Connect

    Mangiafico, R.A.; Petralito, A.; Grimaldi, D.R. )

    1990-07-01

    In a patient with Wolff-Parkinson-White syndrome and an inferior-posterior bypass tract, transient restoration of normal conduction occurred during an attack of angina. The ECG pattern of inferior posterior ischemia was present when the conduction was normal. Thallium scintigraphy showed a reversible posterolateral perfusion defect. The possible mechanisms for production of intermittent preexcitation are discussed.

  2. Normalization of reverse redistribution of thallium-201 with procainamide pretreatment in Wolff-Parkinson-White syndrome

    SciTech Connect

    Nii, T.; Nakashima, Y.; Nomoto, J.; Hiroki, T.; Ohshima, F.; Arakawa, K. )

    1991-03-01

    Stress thallium-201 myocardial perfusion imaging was performed in a patient with Wolff-Parkinson-White syndrome. Reverse redistribution phenomenon was observed in the absence of coronary artery disease. This seems to be the first report of normalization of this phenomenon in association with reversion of accessory pathway to normal atrioventricular conduction after pretreatment with procainamide.

  3. [Multi-infarct dementia clinically simulating dementia of Alzheimer type. A comparison with angular gyrus syndrome].

    PubMed

    Nitta, E; Ohkawa, Y; Kuzuhara, S; Yamanouchi, H; Toyokura, Y

    1989-01-01

    A 74-year-old right-handed man with multiple cerebral infarction who presented with dementia simulating dementia of Alzheimer type (DAT) is reported. He had been well until April 20, 1987 when he developed transient right hand palsy lasting overnight. Eleven days later, he became confused, disorientated, and amnestic. He was admitted to this hospital on June 8. Physical examination revealed hypertension (170/90mmHg). On neurological examination, his consciousness was clear but he was demented. He showed disorientation, amnesia, and urinary incontinence. His most prominent symptom was disturbance of speech, including fluent aphasia and alexia with agraphia. Additionally, he showed ideomotor apraxia, construction apraxia, right-left agnosia, finger agnosia, and acalculia. On July 9, he had a transient attack of right hemiplegia with confusion. The brain CT scan performed on admission was unremarkable except for cavum septi pellucidum and a small low density area in the right basal ganglia. However, single photon emission computed tomography (SPECT) by 123I-labeled N-isopropyl-p-iodoamphetamine disclosed hypoperfusion of the cerebral blood flow in the border zones of the temporoparietal and frontal lobes on the left. A follow-up brain CT scan taken one month later demonstrated low density in the new areas corresponding to hypoperfusion shown by SPECT. Although the clinical features of the present case resembled those of DAT, dementia in this case was regarded as the result of multiple cerebral infarction since it occurred acutely with mild motor deficits, and brain CT scans and SPECT showed lesions indicating focal cerebral ischemia.(ABSTRACT TRUNCATED AT 250 WORDS)

  4. [Subtypes of mild cognitive impairment in Parkinson's disease and factors predicting its becoming dementia].

    PubMed

    Toribio-Diaz, M Elena; Carod-Artal, Francisco J

    2015-07-01

    Introduccion. El deterioro cognitivo puede aparecer en las etapas mas iniciales de la enfermedad de Parkinson (EP). Determinar la prevalencia del deterioro cognitivo leve (DCL) como etapa de transicion o sus diferentes perfiles resulta complicado por la ausencia de criterios diagnosticos consensuados. Objetivo. Revisar el concepto de DCL en la EP, sus criterios diagnosticos y los factores predictores de conversion a demencia. Pacientes y metodos. Revision sistematica de los articulos publicados en Medline (PubMed) utilizando la combinacion de las palabras clave 'deterioro cognitivo leve' y 'enfermedad de Parkinson'. Resultados. Los criterios diagnosticos del DCL en la EP publicados por la Sociedad de Trastornos del Movimiento, a pesar de no estar validados, constituyen una importante herramienta para el diagnostico de estos pacientes. Su aplicacion se ve influida por las siguientes limitaciones: la heterogeneidad de los deficits cognitivos descritos en la EP, su evolucion variable, que dificulta el hallazgo de factores predictores de conversion a demencia, la seleccion de las pruebas neuropsicologicas mas apropiadas y la determinacion de los puntos de corte mas idoneos, y las caracteristicas del paciente, etapa de la enfermedad y tipo de tratamiento antiparkinsoniano. Conclusiones. Marcadores neuropsicologicos, de neuroimagen, biomarcadores o la limitacion en algunas actividades instrumentales son muy prometedores para la deteccion de pacientes con DCL en la EP y riesgo elevado de conversion a demencia.

  5. Evidence of semantic processing impairments in behavioural variant frontotemporal dementia and Parkinson's disease.

    PubMed

    Cousins, Katheryn A Q; Grossman, Murray

    2017-09-13

    Category-specific impairments caused by brain damage can provide important insights into how semantic concepts are organized in the brain. Recent research has demonstrated that disease to sensory and motor cortices can impair perceptual feature knowledge important to the representation of semantic concepts. This evidence supports the grounded cognition theory of semantics, the view that lexical knowledge is partially grounded in perceptual experience and that sensory and motor regions support semantic representations. Less well understood, however, is how heteromodal semantic hubs work to integrate and process semantic information. Although the majority of semantic research to date has focused on how sensory cortical areas are important for the representation of semantic features, new research explores how semantic memory is affected by neurodegeneration in regions important for semantic processing. Here, we review studies that demonstrate impairments to abstract noun knowledge in behavioural variant frontotemporal degeneration (bvFTD) and to action verb knowledge in Parkinson's disease, and discuss how these deficits relate to disease of the semantic selection network. Findings demonstrate that semantic selection processes are supported by the left inferior frontal gyrus (LIFG) and basal ganglia, and that disease to these regions in bvFTD and Parkinson's disease can lead to categorical impairments for abstract nouns and action verbs, respectively.

  6. Atypical parkinsonism and cerebrotendinous xanthomatosis: report of a family with corticobasal syndrome and a literature review.

    PubMed

    Rubio-Agusti, Ignacio; Kojovic, Maja; Edwards, Mark J; Murphy, Elaine; Chandrashekar, Hoskote S; Lachmann, Robin H; Bhatia, Kailash P

    2012-12-01

    Cerebrotendinous xanthomatosis is an autosomal recessive inborn error of cholesterol metabolism. It presents with systemic and neurological symptoms, rarely including parkinsonism. Presented here are a clinical description of a new family with cerebrotendinous xanthomatosis and parkinsonism and a review of 13 additional cases reported in the literature. The index case developed corticobasal syndrome, previously not reported in cerebrotendinous xanthomatosis. His brother had parkinsonism with cerebellar features and cognitive impairment. In a literature review, median age of onset of parkinsonism was found to be 40 years. Nearly all patients had other neurological symptoms: cognitive (93%), pyramidal (93%), or cerebellar (53%). All patients had walking difficulties, with falls in 27%. Systemic features were common: cataracts (93%) or tendon xanthomata (87%). Frequent MRI abnormalities included cerebellar atrophy (100%), cerebral atrophy (80%), and dentate nuclei signal changes (80%). Functional dopaminergic imaging often demonstrated presynaptic denervation. Improvement with levodopa was frequent (91%) but mild. Progressive neurological decline occurred in 92% of patients despite treatment with chenodeoxycholic acid. Cerebrotendinous xanthomatosis should be considered in the differential diagnosis of atypical parkinsonism, including corticobasal syndrome, particularly with early age of onset and in the context of a complex neurological phenotype. Tendon xanthomata, early-onset cataracts, and radiological findings of cerebellar atrophy with lesions of the dentate nuclei are useful clinical clues. Symptomatic treatment with levodopa may help, but progressive neurological decline is frequent despite treatment with chenodeoxycholic acid.

  7. Cholinergic imaging in corticobasal syndrome, progressive supranuclear palsy and frontotemporal dementia.

    PubMed

    Hirano, Shigeki; Shinotoh, Hitoshi; Shimada, Hitoshi; Aotsuka, Akiyo; Tanaka, Noriko; Ota, Tsuneyoshi; Sato, Koichi; Ito, Hiroshi; Kuwabara, Satoshi; Fukushi, Kiyoshi; Irie, Toshiaki; Suhara, Tetsuya

    2010-07-01

    Corticobasal syndrome, progressive supranuclear palsy and frontotemporal dementia are all part of a disease spectrum that includes common cognitive impairment and movement disorders. The aim of this study was to characterize brain cholinergic deficits in these disorders. We measured brain acetylcholinesterase activity by [11C] N-methylpiperidin-4-yl acetate and positron emission tomography in seven patients with corticobasal syndrome (67.6+/-5.9 years), 12 with progressive supranuclear palsy (68.5+/-4.1 years), eight with frontotemporal dementia (59.8+/-6.9 years) and 16 healthy controls (61.2+/-8.5 years). Two-tissue compartment three-parameter model and non-linear least squares analysis with arterial input function were performed. k3 value, an index of acetylcholinesterase activity, was calculated voxel-by-voxel in the brain of each subject. The k3 images in each disease group were compared with the control group by using Statistical Parametric Mapping 2. Volume of interest analysis was performed on spatially normalized k3 images. The corticobasal syndrome group showed decreased acetylcholinesterase activity (k3 values) in the paracentral region, frontal, parietal and occipital cortices (P<0.05, cluster corrected). The group with progressive supranuclear palsy had reduced acetylcholinesterase activity in the paracentral region and thalamus (P<0.05, cluster corrected). The frontotemporal dementia group showed no significant differences in acetylcholinesterase activity. Volume of interest analysis showed mean cortical acetylcholinesterase activity to be reduced by 17.5% in corticobasal syndrome (P<0.001), 9.4% in progressive supranuclear palsy (P<0.05) and 4.4% in frontotemporal dementia (non-significant), when compared with the control group. Thalamic acetylcholinesterase activity was reduced by 6.4% in corticobasal syndrome (non-significant), 24.0% in progressive supranuclear palsy (P<0.03) and increased by 3.3% in frontotemporal dementia (non-significant). Both

  8. Behavioural and psychological symptoms of dementia in Down syndrome: Early indicators of clinical Alzheimer's disease?

    PubMed

    Dekker, Alain D; Strydom, André; Coppus, Antonia M W; Nizetic, Dean; Vermeiren, Yannick; Naudé, Petrus J W; Van Dam, Debby; Potier, Marie-Claude; Fortea, Juan; De Deyn, Peter P

    2015-12-01

    Behavioural and Psychological Symptoms of Dementia (BPSD) are a core symptom of dementia and are associated with suffering, earlier institutionalization and accelerated cognitive decline for patients and increased caregiver burden. Despite the extremely high risk for Down syndrome (DS) individuals to develop dementia due to Alzheimer's disease (AD), BPSD have not been comprehensively assessed in the DS population. Due to the great variety of DS cohorts, diagnostic methodologies, sub-optimal scales, covariates and outcome measures, it is questionable whether BPSD have always been accurately assessed. However, accurate recognition of BPSD may increase awareness and understanding of these behavioural aberrations, thus enabling adaptive caregiving and, importantly, allowing for therapeutic interventions. Particular BPSD can be observed (long) before the clinical dementia diagnosis and could therefore serve as early indicators of those at risk, and provide a new, non-invasive way to monitor, or at least give an indication of, the complex progression to dementia in DS. Therefore, this review summarizes and evaluates the rather limited knowledge on BPSD in DS and highlights its importance and potential for daily clinical practice.

  9. Telomere longitudinal shortening as a biomarker for dementia status of adults with Down syndrome.

    PubMed

    Jenkins, Edmund C; Ye, Lingling; Krinsky-McHale, Sharon J; Zigman, Warren B; Schupf, Nicole; Silverman, Wayne P

    2016-03-01

    Previous studies have suggested that Alzheimer's disease (AD) causes an accelerated shortening of telomeres, the ends of chromosomes consisting of highly conserved TTAGGG repeats that, because of unidirectional 5'-3' DNA synthesis, lose end point material with each cell division. Our own previous work suggested that telomere length of T-lymphocytes might be a remarkably accurate biomarker for "mild cognitive impairment" in adults with Down syndrome (MCI-DS), a population at dramatically high risk for AD. To verify that the progression of cognitive and functional losses due to AD produced this observed telomere shortening, we have now examined sequential changes in telomere length in five individuals with Down syndrome (3F, 2M) as they transitioned from preclinical AD to MCI-DS (N = 4) or dementia (N = 1). As in our previous studies, we used PNA (peptide nucleic acid) probes for telomeres and the chromosome 2 centromere (as an "internal standard" expected to be unaffected by aging or dementia status), with samples from the same individuals now collected prior to and following development of MCI-DS or dementia. Consistent shortening of telomere length was observed over time. Further comparisons with our previous cross-sectional findings indicated that telomere lengths prior to clinical decline were similar to those of other adults with Down syndrome (DS) who have not experienced clinical decline while telomere lengths following transition to MCI-DS or dementia in the current study were comparable to those of other adults with DS who have developed MCI-DS or dementia. Taken together, findings indicate that telomere length has significant promise as a biomarker of clinical progression of AD for adults with DS, and further longitudinal studies of a larger sample of individuals with DS are clearly warranted to validate these findings and determine if and how factors affecting AD risk also influence these measures of telomere length. © 2015 Wiley Periodicals, Inc.

  10. [A case of elderly onset Parkinson's disease complicated by dropped head syndrome].

    PubMed

    Horiuchi, M; Uehara, K; Komo, T; Sugihara, H; Takahashi, Y

    2001-09-01

    An 86-year old man presented with a 7-year history of gait disturbance. He was admitted to our hospital on April 2000 because he was experiencing difficulty eating due to progression of dropped head syndrome. Upon standing and sitting, remarkable dropped head and kyphosis were observed. When lying, the patient was able to stretch his neck, and he could stand and walk with the aid of a walker. Rigidity and resting tremor were present predominantly in the lower limbs. Parkinson's disease was diagnosed therefore L-dopa and Cabergoline were administered. Parkinsonism and dropped head syndrome improved in response to treatment. Cases involving dropped head syndrome due to Parkinson's disease are reportedly improved by L-dopa, but exasperated by dopamine agonists. The mechanism of dropped head is thought to be an imbalance in the tonus of the anterior and posterior neck muscles. Dropped head in the present case may have been a complication of Parkinson's disease since it improved in response to L-dopa.

  11. [Radiofrequency catheter ablation in children with Wolff-Parkinson-White syndrome and sudden cardiac death who had been resuscitated].

    PubMed

    Benito Bartolomé, F; Sánchez Fernández-Bernal, C

    2001-04-01

    Sudden death may be the first manifestation of the Wolff-Parkinson-White syndrome, especially in children and adolescents. The aim of this study was to evaluate the usefulness of radiofrequency catheter ablation in children with Wolff-Parkinson-White syndrome with aborted sudden death. We report four patients with Wolff-Parkinson-White syndrome who survived cardiac arrest. The patients were aged from 2.5 months to 16 years. The two first patients were lactating infants; in the first sudden death occurred during digoxin treatment for supraventricular tachycardia secondary to Wolff-Parkinson-White syndrome and in the second the syndrome was diagnosed after an episode of sudden death. In these patients a free wall accessory pathway (left posterior and left lateral, respectively) was successfully ablated using a transseptal approach. The third patient was diagnosed with asymptomatic Wolff-Parkinson-White syndrome; sudden death occurred during exercise. In the fourth patient, sudden death occurred after intravenous therapy with adenosine triphosphate and amiodarone for rapid atrial fibrillation. In both patients, one accessory pathway, located in right posteroseptal and right anterior free wall, respectively, was ablated. After a mean follow-up of 43.5 26.4 months, no recurrence of sudden death had occurred and electrocardiogram showed sinus rhythm without delta wave. The third patient presented severe sequelae of hypoxemic encephalopathy, which persisted during the follow-up. Radiofrequency catheter ablation is the treatment of choice in Wolff-Parkinson-White syndrome with episodes of aborted sudden death.

  12. Creutzfeldt-jakob, Parkinson, lewy body dementia and Alzheimer diseases: from diagnosis to therapy.

    PubMed

    Dupiereux, Ingrid; Zorzi, Willy; Quadrio, Isabelle; Perret-Liaudet, Armand; Kovacs, Gabor G; Heinen, Ernst; Elmoualij, Benaïssa

    2009-03-01

    Depositions of proteins in form of amyloid and non-amyloid plaques are common pathogenic signs of more than 20 degenerative diseases affecting the central nervous system or a variety of peripheral tissues. Among the neuropathological conditions, Alzheimer's, Parkinson's and the prion diseases, such as Creutzfeldt-Jakob disease (CJD), present ambiguities as regarding their differential diagnosis. At present, their diagnosis must be confirmed by post-mortem examination of the brain. Currently the ante-mortem diagnosis is still based on the integration of multiple data (clinical, paraclinical and biological analyses) because no unique marker exists for such diseases. The detection of specific biomarkers would be useful to develop a differential diagnostic, distinguishing not only different neurodegenerative diseases but also the disease from the non-pathological effects of aging. Several neurodegenerative biomarkers are present at very low levels during the early stages of the disease development and their ultra-low detection is needed for early diagnosis, which should permit more effective therapeutic interventions, before the disease concerned can progress to a stage where considerable damage to the brain has already occurred. In the case of prion diseases, there are concerns regarding not only patient care, but the wider community too, with regard to the risk of transmission of prions, especially during blood transfusion, for which, four cases of variant CJD infection associated with transfusion of non-leukocyte-depleted blood components have been confirmed. Therefore the development of techniques with high sensitivity and specificity represent the major challenge in the field of the protein misfolding diseases. In this paper we review the current analytical and/or biochemical diagnostic technologies used mainly in prion, but also in Alzheimer and Parkinson diseases and emphasizing work on the protein detection as a surrogates and specific biomarker in the body

  13. [Does patient age influence the indications for investigating asympatomatic Wolff-Parkinson-White syndrome?].

    PubMed

    Brembilla-Perrot, B; Holban, I; Houriez, P; Beurrier, D; Claudon, O; Vançon, A C

    2000-12-01

    Sudden death may be the presenting symptom of a Wolff-Parkinson-White syndrome. Electrophysiological investigation is the best method of identifying high risk cases. The aim of this study was to determine whether this investigation should be proposed to all patients, irrespective of age. Transoesophageal stimulation was performed in 85 asymptomatic patients with the Wolff-Parkinson-White syndrome. Of the 85 subjects, 13 were under 20 years of age, 30 under 30 years, 15 under 40 years, 16 under 50 years and 11 between 50 and 69 years of age. A protocol of incremental stimulation until 2nd degree AVB was attained and programmed atrial stimulation with one or two extrastimuli delivered on 2 paced cycles (600 and 400 ms) was used under basal conditions and with Isoprenaline. A malignant form of the condition was defined as the demonstration of two abnormalities: rapid conduction in the bundle of Kent (over 240/min) under basal conditions or over 300/min after Isoprenaline, and if it induced sustained atrial fibrillation (> 1 min). The results were: [table: see text] In conclusion, the number of malignant forms of the Wolff-Parkinson-White syndrome is exactly the same, irrespective of age. Elderly patients remain at risk of malignant WPW syndrome because of the increased incidence of atrial fibrillation. Therefore, the authors recommend systematic evaluation of this syndrome if the patient has an active life-style especially with regard to sporting activities.

  14. Evidence that PICALM affects age at onset of Alzheimer's dementia in Down syndrome.

    PubMed

    Jones, Emma L; Mok, Kin; Hanney, Marisa; Harold, Denise; Sims, Rebecca; Williams, Julie; Ballard, Clive

    2013-10-01

    It is known that individuals with Down syndrome develop Alzheimer's disease with an early age at onset, although associated genetic risk factors have not been widely studied. We tested whether genes that increase the risk of late-onset Alzheimer's disease influence the age at onset in Down syndrome using genome-wide association data for age at onset of dementia in a small sample of individuals (N = 67) with Down syndrome. We tested for association with loci previously associated with Alzheimer's disease risk and, despite the small size of the study, we detected associations with age at onset of Alzheimer's disease in Down syndrome with PICALM (β = 3.31, p = 0.011) and the APOE loci (β = 3.58, p = 0.014). As dementia in people with Down syndrome is relatively understudied, we make all of these data publicly available to encourage further analyses of the problem of Alzheimer's disease in Down syndrome.

  15. Differentiating Aging Among Adults With Down Syndrome and Comorbid Dementia or Psychopathology.

    PubMed

    Esbensen, Anna J; Johnson, Emily Boshkoff; Amaral, Joseph L; Tan, Christine M; Macks, Ryan

    2016-01-01

    Differences were examined between three groups of adults with Down syndrome in their behavioral presentation, social life/activities, health, and support needs. We compared those with comorbid dementia, with comorbid psychopathology, and with no comorbid conditions. Adults with comorbid dementia were more likely to be older, have lower functional abilities, have worse health and more health conditions, and need more support in self-care. Adults with comorbid psychopathology were more likely to exhibit more behavior problems and to be living at home with their families. Adults with no comorbidities were most likely to be involved in community employment. Differences in behavioral presentation can help facilitate clinical diagnoses in aging in Down syndrome, and implications for differential diagnosis and service supports are discussed.

  16. A fetal Wolff-Parkinson-White syndrome diagnosed prenatally by magnetocardiography.

    PubMed

    Hosono, T; Chiba, Y; Shinto, M; Kandori, A; Tsukada, K

    2001-01-01

    We report a case of fetal Wolff-Parkinson-White (WPW) syndrome diagnosed prenatally by magnetocardiography (MCG). At 32 weeks' gestation, the fetus was diagnosed to have a paroxysmal supraventricular tachycardia by ultrasonography and direct fetal electrocardiogram (ECG). Transplacental fetal therapy by maternal oral administration of propranolol resolved the fetal tachyarrhythmia. Although the wave forms of the fetal MCG at 32 weeks' gestation were normal, the fetal MCG at 35 weeks' gestation showed a short PR interval and a long QRS complex duration with a delta wave, indicating WPW syndrome. The findings of the fetal MCG were confirmed by the postnatal ECG. MCG made the prenatal diagnosis of WPW syndrome possible.

  17. Agraphia in patients with frontotemporal dementia and parkinsonism linked to chromosome 17 with P301L MAPT mutation: dysexecutive, aphasic, apraxic or spatial phenomenon?

    PubMed Central

    Sitek, Emilia J.; Narożańska, Ewa; Barczak, Anna; Jasińska-Myga, Barbara; Harciarek, Michał; Chodakowska-Żebrowska, Małgorzata; Kubiak, Małgorzata; Wieczorek, Dariusz; Konieczna, Seweryna; Rademakers, Rosa; Baker, Matt; Berdyński, Mariusz; Brockhuis, Bogna; Barcikowska, Maria; Żekanowski, Cezary; Heilman, Kenneth M.; Wszołek, Zbigniew K.; Sławek, Jarosław

    2013-01-01

    Objectives Patients with frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17) may be agraphic. The study aimed at characterizing agraphia in individuals with a P301L MAPT mutation. Methods Two pairs of siblings with FTDP-17 were longitudinally examined for agraphia in relation to language and cognitive deficits. Results All patients presented with dysexecutive agraphia. In addition, in the first pair of siblings one sibling demonstrated spatial agraphia with less pronounced allographic agraphia and the other sibling had aphasic agraphia. Aphasic agraphia was also present in one sibling from the second pair. Conclusion Agraphia associated with FTDP-17 is very heterogeneous. PMID:23121543

  18. Cognition enhancers for the treatment of dementia.

    PubMed

    Malek, Naveed; Greene, John

    2015-02-01

    Dementia is a broad term used to describe several chronic progressive neurological disorders that adversely affect higher mental functions including memory, language, behaviour, abstract thinking, comprehension, calculation, learning capacity and judgement. Alzheimer's disease is the most common form of dementia but other neurological conditions such as Parkinson's disease, cerebrovascular disease and chronic infections such as syphilis can also lead to the clinical syndrome of dementia. Initial investigations should always focus on finding any treatable cause for dementia such as HIV, structural lesions such as subdural haematomas or specific nutritional deficiency states such as that due to vitamin B12 and treated appropriately. Where no treatable or reversible aetiology is found, a referral to a specialist should be considered who may initiate further investigations including magnetic resonance imaging or perfusion single-photon emission computerised tomography scans of the brain, and sometimes cerebrospinal fluid examination or an electroencephalogram.

  19. The impact of MRI white matter hyperintensities on dementia in Parkinson's disease in relation to the homocysteine level and other vascular risk factors.

    PubMed

    Sławek, Jarosław; Roszmann, Anna; Robowski, Piotr; Dubaniewicz, Mirosława; Sitek, Emilia J; Honczarenko, Krystyna; Gorzkowska, Agnieszka; Budrewicz, Sławomir; Mak, Monika; Gołąb-Janowska, Monika; Koziorowska-Gawron, Ewa; Droździk, Marek; Kurzawski, Mateusz; Bandurski, Tomasz; Białecka, Monika

    2013-01-01

    The role of white matter hyperintensities (WMH) and homocysteine (Hcy) and other vascular risk factors in the pathogenesis of Parkinson's disease (PD) dementia (PDD) remains unclear. The aim of the study was to assess the impact of WMH, Hcy and other biochemical and vascular risk factors on PDD. A total of 192 patients with PD and 184 age- and sex-matched healthy controls were included. A semistructured interview was used to assess demographic and clinical variables with respect to vascular risk factors (arterial hypertension, diabetes mellitus, atrial fibrillation, ischemic heart disease, obliterative atherosclerosis, hypercholesterolemia, smoking, alcohol intake). Unified Parkinson's Disease Rating Scale score, Hoehn-Yahr staging and the Schwab-England activities of daily living scale were used to assess motor abilities and activities of daily living. A complex neuropsychological examination with a battery of tests was used to classify patients into a group with dementia (PDD) and a group without dementia (PD). Neuroradiological examination of MRI scans included visual rating scales for WMH (according to the Wahlund and Erkinjunntti rating scales) and the Scheltens scale for hippocampal atrophy. Blood samples for Hcy, folate, vitamin B12, fibrinogen, lipids, glucose, creatinine, transaminases and thyroid stimulating hormone (TSH) were examined. Among all patients, 57 (29.7%) fulfilled the diagnostic criteria for dementia. Significantly higher Hcy plasma levels were noted in PD and PDD groups compared to controls (p < 0.05) and in PDD when compared to PD (p < 0.05). According to multivariate regression analysis, WMH (Erkinjuntti scale), high Hcy, low vitamin B12 and folate plasma levels were independent risk factors for PDD. Vascular risk factors did not play any role in the pathogenesis of PDD and WMH. WMH along with Hcy, folate and vitamin B12 may impact cognition in PD. Therapy with vitamin B12, folate and catechol-O-methyltransferase inhibitors may play a

  20. A placebo-controlled study of memantine (Ebixa) in dementia of Wernicke-Korsakoff syndrome.

    PubMed

    Rustembegović, Avdo; Kundurović, Zlata; Sapcanin, Aida; Sofic, Emin

    2003-01-01

    We evaluated the responses of 16 patients to preliminarily explore the spectrum of effectiveness and tolerability of the memantine, and NMDA antagonist, in the treatment of dementia in Wernicke-Korsakoff syndrome. In this study, for the first time in dementia of Wernicke-Korsakoff syndrome, the response to memantine was assessed. 16 patients with median age of 64 years and median body weight of 77 kg were treated with memantine 10 mg twice daily for up to 28 weeks. Clinical global impressions (CGI), and Mini Mental Status Examination (MMSE) were performed during the treatment period (after 2, 4, and 28 weeks). Efficacy measures also included the ADCS-Activities of Daily Living scale (ADCS-ADL). At 28 weeks, the ADCS-ADL showed significantly less deterioration in memantine treated patients compared with placebo (-2.3 compared with -4.3: p = 0.005). The results of MMSE demonstrate a significant and clinically relevant benefit for memantine relative to placebo as shown by positive outcomes in cognitive and functional assessments. Memantine (10 mg) was safe and well tolerated. The preliminarily findings of this study with 16 patients suggested that memantine is effective in the treatment of dementia in Wernicke-Korsakoff syndrome.

  1. Ceftriaxone prevents and reverses behavioral and neuronal deficits in an MPTP-induced animal model of Parkinson's disease dementia.

    PubMed

    Hsu, Chao-Yu; Hung, Ching-Sui; Chang, Hung-Ming; Liao, Wen-Chieh; Ho, Shih-Chun; Ho, Ying-Jui

    2015-04-01

    Glutamatergic hyperactivity plays an important role in the pathophysiology of Parkinson's disease (PD). Ceftriaxone increases expression of glutamate transporter 1 (GLT-1) and affords neuroprotection. This study was aimed at clarifying whether ceftriaxone prevented, or reversed, behavioral and neuronal deficits in an 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP)-induced PD rat model. Male Wistar rats were injected daily with either ceftriaxone starting 5 days before or 3 days after MPTP lesioning (day 0) or saline and underwent a bar-test on days 1-7, a T-maze test on days 9-11, and an object recognition test on days 12-14, then the brains were taken for histological evaluation on day 15. Dopaminergic degeneration in the substantia nigra pars compacta and striatum was observed on days 3 and 15. Motor dysfunction in the bar test was observed on day 1, but disappeared by day 7. In addition, lesioning resulted in deficits in working memory in the T-maze test and in object recognition in the object recognition task, but these were not observed in rats treated pre- or post-lesioning with ceftriaxone. Lesioning also caused neurodegeneration in the hippocampal CA1 area and induced glutamatergic hyperactivity in the subthalamic nucleus, and both changes were suppressed by ceftriaxone. Increased GLT-1 expression and its co-localization with astrocytes were observed in the striatum and hippocampus in the ceftriaxone-treated animals. To our knowledge, this is the first study showing a relationship between ceftriaxone-induced GLT-1 expression, neuroprotection, and improved cognition in a PD rat model. Ceftriaxone may have clinical potential for the prevention and treatment of dementia associated with PD. Copyright © 2014 Elsevier Ltd. All rights reserved.

  2. Clioquinol rescues Parkinsonism and dementia phenotypes of the tau knockout mouse.

    PubMed

    Lei, Peng; Ayton, Scott; Appukuttan, Ambili Thoppuvalappil; Volitakis, Irene; Adlard, Paul A; Finkelstein, David I; Bush, Ashley I

    2015-09-01

    Iron accumulation and tau protein deposition are pathological features of Alzheimer's (AD) and Parkinson's diseases (PD). Soluble tau protein is lower in affected regions of these diseases, and we previously reported that tau knockout mice display motor and cognitive behavioral abnormities, brain atrophy, neuronal death in substantia nigra, and iron accumulation in the brain that all emerged between 6 and 12 months of age. This argues for a loss of tau function in AD and PD. We also showed that treatment with the moderate iron chelator, clioquinol (CQ) restored iron levels and prevented neuronal atrophy and attendant behavioral decline in 12-month old tau KO mice when commenced prior to the onset of deterioration (6 months). However, therapies for AD and PD will need to treat the disease once it is already manifest. So, in the current study, we tested whether CQ could also rescue the phenotype of mice with a developed phenotype. We found that 5-month treatment of symptomatic (13 months old) tau KO mice with CQ increased nigral tyrosine hydroxylase phosphorylation (which induces activity) and reversed the motor deficits. Treatment also reversed cognitive deficits and raised BDNF levels in the hippocampus, which was accompanied by attenuated brain atrophy, and reduced iron content in the brain. These data raise the possibility that lowering brain iron levels in symptomatic patients could reverse neuronal atrophy and improve brain function, possibly by elevating neurotrophins.

  3. Regional cortical grey matter loss in Parkinson's disease without dementia is independent from visual hallucinations.

    PubMed

    Meppelink, Anne Marthe; de Jong, Bauke M; Teune, Laura K; van Laar, Teus

    2011-01-01

    In our previous functional magnetic resonance imaging study, Parkinson's disease (PD) patients with visual hallucinations (VH) showed reduced activations in ventral/lateral visual association cortices preceding image recognition, compared with both PD patients without VH and healthy controls. The primary aim of the current study was to investigate whether functional deficits are associated with grey matter volume changes. In addition, possible grey matter differences between all PD patients and healthy controls were assessed. By using 3-Tesla magnetic resonance imaging (MRI) and voxel-based morphometry (VBM), we found no differences between PD patients with (n = 11) and without VH (n = 13). However, grey matter decreases of the bilateral prefrontal and parietal cortex, left anterior superior temporal, and left middle occipital gyrus were found in the total group of PD patients, compared with controls (n = 14). This indicates that previously demonstrated functional deficits in PD patients with VH are not associated with grey matter loss. The strong left parietal reduction in both nondemented patient groups was hemisphere specific and independent of the side of PD symptoms.

  4. Prevalence of amyloid PET positivity in dementia syndromes: a meta-analysis.

    PubMed

    Ossenkoppele, Rik; Jansen, Willemijn J; Rabinovici, Gil D; Knol, Dirk L; van der Flier, Wiesje M; van Berckel, Bart N M; Scheltens, Philip; Visser, Pieter Jelle; Verfaillie, Sander C J; Zwan, Marissa D; Adriaanse, Sofie M; Lammertsma, Adriaan A; Barkhof, Frederik; Jagust, William J; Miller, Bruce L; Rosen, Howard J; Landau, Susan M; Villemagne, Victor L; Rowe, Christopher C; Lee, Dong Y; Na, Duk L; Seo, Sang W; Sarazin, Marie; Roe, Catherine M; Sabri, Osama; Barthel, Henryk; Koglin, Norman; Hodges, John; Leyton, Cristian E; Vandenberghe, Rik; van Laere, Koen; Drzezga, Alexander; Forster, Stefan; Grimmer, Timo; Sánchez-Juan, Pascual; Carril, Jose M; Mok, Vincent; Camus, Vincent; Klunk, William E; Cohen, Ann D; Meyer, Philipp T; Hellwig, Sabine; Newberg, Andrew; Frederiksen, Kristian S; Fleisher, Adam S; Mintun, Mark A; Wolk, David A; Nordberg, Agneta; Rinne, Juha O; Chételat, Gaël; Lleo, Alberto; Blesa, Rafael; Fortea, Juan; Madsen, Karine; Rodrigue, Karen M; Brooks, David J

    2015-05-19

    Amyloid-β positron emission tomography (PET) imaging allows in vivo detection of fibrillar plaques, a core neuropathological feature of Alzheimer disease (AD). Its diagnostic utility is still unclear because amyloid plaques also occur in patients with non-AD dementia. To use individual participant data meta-analysis to estimate the prevalence of amyloid positivity on PET in a wide variety of dementia syndromes. The MEDLINE and Web of Science databases were searched from January 2004 to April 2015 for amyloid PET studies. Case reports and studies on neurological or psychiatric diseases other than dementia were excluded. Corresponding authors of eligible cohorts were invited to provide individual participant data. Data were provided for 1359 participants with clinically diagnosed AD and 538 participants with non-AD dementia. The reference groups were 1849 healthy control participants (based on amyloid PET) and an independent sample of 1369 AD participants (based on autopsy). Estimated prevalence of positive amyloid PET scans according to diagnosis, age, and apolipoprotein E (APOE) ε4 status, using the generalized estimating equations method. The likelihood of amyloid positivity was associated with age and APOE ε4 status. In AD dementia, the prevalence of amyloid positivity decreased from age 50 to 90 years in APOE ε4 noncarriers (86% [95% CI, 73%-94%] at 50 years to 68% [95% CI, 57%-77%] at 90 years; n = 377) and to a lesser degree in APOE ε4 carriers (97% [95% CI, 92%-99%] at 50 years to 90% [95% CI, 83%-94%] at 90 years; n = 593; P < .01). Similar associations of age and APOE ε4 with amyloid positivity were observed in participants with AD dementia at autopsy. In most non-AD dementias, amyloid positivity increased with both age (from 60 to 80 years) and APOE ε4 carriership (dementia with Lewy bodies: carriers [n = 16], 63% [95% CI, 48%-80%] at 60 years to 83% [95% CI, 67%-92%] at 80 years; noncarriers [n = 18], 29% [95% CI, 15%-50%] at 60

  5. [Significance of tachycardia induced by atrial stimulation in Wolff-Parkinson-White syndrome].

    PubMed

    Brembilla-Perrot, B

    1992-04-01

    Increased atrial vulnerability is one of the criteria of malignant Wolff-Parkinson-White syndrome. The aim of this study was to try to define the methods of induction of atrial tachycardias (tachycardia, flutter, fibrillation) by endocavitary and oesophageal stimulation characterising an increased vulnerability. The incidence of induced sustained tachycardia by fixed atrial stimulation at incremental rates until the Wenckebach point is attained and programmed atrial stimulation using 1 and 2 extrastimuli under basal conditions and then with isoproterenol was compared in subjects without cardiac disease, Wolff-Parkinson-White or spontaneous tachycardia (Group I) and patients with Wolff-Parkinson-White and spontaneous tachycardias (Group II). Atrial stimulation only induced tachycardia in 2.5% of normal subjects under basal conditions or with isoproterenol, by the endocavitary or oesophageal approaches. Programmed stimulation induced tachycardia in 15% of normal subjects under basal conditions or with isoproterenol by the endocavitary approach alone. In Group II, tachycardia was reproduced under basal conditions or with isoproterenol by atrial stimulation or programmed stimulation in all patients. In conclusion, the induction of a tachyarrhythmia by incremental atrial stimulation up to the Wenckebach point is always pathological even with isoproterenol. Programmed atrial stimulation is less specific except by the oesophageal approach. The use of bursts of very rapid stimuli in the Wolff-Parkinson-White syndrome is of no value as tachycardia can be induced by classical methods in all subjects at risk.

  6. Impairment of brainstem implicit learning paradigms differentiates multiple system atrophy (MSA) from idiopathic Parkinson syndrome.

    PubMed

    von Lewinski, Friederike; Schwan, Michaela; Paulus, Walter; Trenkwalder, Claudia; Sommer, Martin

    2013-09-13

    Learning as measured by eyeblink classical conditioning is preserved in patients with idiopathic Parkinson's disease, but severely affected in patients with progressive supranuclear palsy. We here sought to clarify whether procedural learning is impaired in multiple system atrophy (MSA), and whether it may be helpful for the differentiation of parkinsonian syndromes. We investigated learning using (1) eyeblink classical conditioning with a delay (interstimulus interval 0 ms) and a trace (600 ms) paradigm and (2) a serial reaction time task. Participants were recruited from academic research centres. 11 patients with MSA and 11 healthy controls. Implicit learning in eyeblink classical conditioning (acquisition of conditioned responses) as well as the serial reaction time task measures of implicit learning (reaction time change) are impaired in patients with MSA as compared with controls, whereas explicit learning as measured by the sequence recall of the serial reaction time task is relatively preserved. We hypothesise that the learning deficits of patients with MSA are due to lesions of cerebellar and connected brainstem areas. A retrospective synopsis of these novel data on patients with MSA and groups of patients with idiopathic Parkinson's disease and progressive supranuclear palsy studied earlier suggest that eyeblink classical conditioning may contribute to the early differentiation of atypical Parkinson syndromes from idiopathic Parkinson's disease. This hypothesis should be tested in a prospective trial.

  7. [Electrophysiological effects of chloroacetyl ajmaline in Wolff-Parkinson-White syndrome].

    PubMed

    Touboul, P; Gressard, A; Atallah, G; Chatelain, M T; Delahaye, J P

    1978-07-01

    The electrophysiological effects of chloro-acetyl-ajmaline in the Wolff-Parkinson-White syndrome have been studied in 7 patients after an intravenous dose of 1.5 mg/kg of the drug. Preexcitation was abolished in 3 cases, while 3 other subjects showed a slight increase in effective refractory period of the abnormal route of excitation (a mean of 13 ms). The possibility of bringing about a reciprocal rhythm was removed in one case out of two. During tachycardia, chloro-acetyl-ajmaline produced significant lengthening of the ventriculo-atrial conduction time (p less than 0.05). These results show the usefulness of chloro-acetyl-ajmaline in the control of the arrhythmias associated with the Wolff-Parkinson-White syndrome.

  8. [Atrioventricular and ventriculoatrial conduction in patients operated on for the Wolff-Parkinson-White syndrome].

    PubMed

    Colín, L; Dorado, M; Iturralde, P; Romero, L; Kershenovich, S; de Micheli, A; Barragán, R; Ramírez, S; González-Hermosillo, J A

    1992-01-01

    Over the last decade the surgical treatment of the Wolff-Parkinson-White syndrome has been well accepted. It is important to make an early diagnosis for surgical success. For this purpose we utilized programmed electrical stimulation to assess the functional characteristics of atrioventricular and ventriculoatrial conduction in our post-operative patients. In 55% of the cases we found accelerated nodal conduction. Programmed electrical stimulation correctly identified 90% of successfully treated patients. We did not found any false positive curve, therefore, this method has a high specificity. We concluded that in post-operative patients with the Wolff-Parkinson-White syndrome: 1- There is a high incidence of accelerated nodal conduction and 2- programmed electrical stimulation can correctly identify most of the patients who were successfully treated.

  9. Differential diagnostic value of eye movement recording in PSP-parkinsonism, Richardson's syndrome, and idiopathic Parkinson's disease.

    PubMed

    Pinkhardt, Elmar H; Jürgens, Reinhart; Becker, Wolfgang; Valdarno, Federica; Ludolph, Albert C; Kassubek, Jan

    2008-12-01

    Vertical gaze palsy is a highly relevant clinical sign in parkinsonian syndromes. As the eponymous sign of progressive supranuclear palsy (PSP), it is one of the core features in the diagnosis of this disease. Recent studies have suggested a further differentiation of PSP in Richardson's syndrome (RS) and PSP-parkinsonism (PSPP). The aim of this study was to search for oculomotor abnormalities in the PSP-P subset of a sample of PSP patients and to compare these findings with those of (i) RS patients, (ii) patients with idiopathic Parkinson's disease (IPD), and (iii) a control group. Twelve cases of RS, 5 cases of PSP-P, and 27 cases of IPD were examined by use of video-oculography (VOG) and compared to 23 healthy normal controls. Both groups of PSP patients (RS, PSP-P) had significantly slower saccades than either IPD patients or controls, whereas no differences in saccadic eye peak velocity were found between the two PSP groups or in the comparison of IPD with controls. RS and PSP-P were also similar to each other with regard to smooth pursuit eye movements (SPEM), with both groups having significantly lower gain than controls (except for downward pursuit); however, SPEM gain exhibited no consistent difference between PSP and IPD. A correlation between eye movement data and clinical data (Hoehn & Yahr scale or disease duration) could not be observed. As PSP-P patients were still in an early stage of the disease when a differentiation from IPD is difficult on clinical grounds, the clear-cut separation between PSP-P and IPD obtained by measuring saccade velocity suggests that VOG could contribute to the early differentiation between these patient groups.

  10. Rivastigmine in apathetic but dementia and depression-free patients with Parkinson's disease: a double-blind, placebo-controlled, randomised clinical trial.

    PubMed

    Devos, David; Moreau, Caroline; Maltête, David; Lefaucheur, Romain; Kreisler, Alexandre; Eusebio, Alexandre; Defer, Gilles; Ouk, Thavarak; Azulay, Jean-Philippe; Krystkowiak, Pierre; Witjas, Tatiana; Delliaux, Marie; Destée, Alain; Duhamel, Alain; Bordet, Régis; Defebvre, Luc; Dujardin, Kathy

    2014-06-01

    Even with optimal dopaminergic treatments, many patients with Parkinson's disease (PD) are frequently incapacitated by apathy prior to the development of dementia. We sought to establish whether rivastigmine's ability to inhibit acetyl- and butyrylcholinesterases could relieve the symptoms of apathy in dementia-free, non-depressed patients with advanced PD. We performed a multicentre, parallel, double-blind, placebo-controlled, randomised clinical trial (Protocol ID: 2008-002578-36; clinicaltrials.gov reference: NCT00767091) in patients with PD with moderate to severe apathy (despite optimised dopaminergic treatment) and without dementia. Patients from five French university hospitals were randomly assigned 1:1 to rivastigmine (transdermal patch of 9.5 mg/day) or placebo for 6 months. The primary efficacy criterion was the change over time in the Lille Apathy Rating Scale (LARS) score. 101 consecutive patients were screened, 31 were eligible and 16 and 14 participants were randomised into the rivastigmine and placebo groups, respectively. Compared with placebo, rivastigmine improved the LARS score (from -11.5 (-15/-7) at baseline to -20 (-25/-12) after treatment; F(1, 25)=5.2; p=0.031; adjusted size effect: -0.9). Rivastigmine also improved the caregiver burden and instrumental activities of daily living but failed to improve quality of life. No severe adverse events occurred in the rivastigmine group. Rivastigmine may represent a new therapeutic option for moderate to severe apathy in advanced PD patients with optimised dopaminergic treatment and without depression dementia. These findings require confirmation in a larger clinical trial. Our results also confirmed that the presence of apathy can herald a pre-dementia state in PD. Clinicaltrials.gov reference: NCT00767091.

  11. Asymptomatic Wolff-Parkinson-White Syndrome: Who Should Be Treated?

    PubMed

    Obeyesekere, Manoj N; Leong-Sit, Peter; Krahn, Andrew D; Gula, Lorne J; Yee, Raymond; Skanes, Allan C; Klein, George J

    2012-09-01

    This article discusses the merits of electrophysiology study (EPS) and/or ablation for asymptomatic preexcitation Wolff-Parkinson-White (WPW) ECG pattern. Sudden deaths in asymptomatic patients are too few to merit broad screening and aggressive intervention. It also discusses the risks of ablation and the low predictive accuracy of EPS. When WPW is an incidental finding, the decision to proceed with investigation and ablation can be made considering patients' situations and preferences. An invasive strategy is targeted at patients concerned about the low risk of life-threatening arrhythmia as a first presentation after a discussion of the risks and benefits.

  12. Agitation and resistiveness to care are two separate behavioral syndromes of dementia.

    PubMed

    Volicer, Ladislav; Bass, Elizabeth A; Luther, Stephen L

    2007-10-01

    To distinguish two behavioral syndromes of dementia: Agitation and resistiveness to care. Analysis of Minimum Data Set (MDS) data. MDS data from Veterans Administration nursing homes collected from October 13, 2000, through October 14, 2004. Participants were 23,837 residents with a positive diagnosis for Alzheimer's disease or dementia other than Alzheimer's. Presence of agitation in each patient was based on the recorded value for 6 MDS variables: repetitive questions, repetitive verbalizations, expressions of what appear to be unrealistic fears, repetitive health complaints, repetitive anxious complaints or concerns, and repetitive physical movements. Patients who exhibited the MDS variable "resists care; resisted taking medications/injections, ADL assistance or eating" anytime within the last 7 days of the assessment and whose behavior was not easily altered were considered "resistive to care." Severity of dementia was measured by the Cognitive Performance Scale using 3 MDS items: short-term memory, cognitive skills for daily decision making, and making self understood. Agitation alone was present in 17%, resistiveness to care alone in 9%, and both syndromes in 8% of residents. Agitation was present in a significant number of residents who were borderline intact, was most common in subjects with moderate cognitive impairment, and decreased thereafter. In contrast, resistiveness to care was relatively rare in borderline intact and mildly impaired residents and increased gradually, with the highest prevalence in those with very severe cognitive impairment. The prevalence of resistiveness to care increased as the ability to understand deteriorated. Most residents who were rated as having abusive symptoms were also resistive to care. Agitation and resistiveness to care are 2 separate behavioral syndromes that may also occur together. It is important to distinguish between agitation and resistiveness to care because these syndromes require different management

  13. Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson's syndrome and PSP-parkinsonism.

    PubMed

    Williams, David R; de Silva, Rohan; Paviour, Dominic C; Pittman, Alan; Watt, Hilary C; Kilford, Linda; Holton, Janice L; Revesz, Tamas; Lees, Andrew J

    2005-06-01

    The clinical diagnosis of progressive supranuclear palsy (PSP) relies on the identification of characteristic signs and symptoms. A proportion of pathologically diagnosed cases do not develop these classic features, prove difficult to diagnose during life and are considered as atypical PSP. The aim of this study was to examine the apparent clinical dichotomy between typical and atypical PSP, and to compare the biochemical and genetic characteristics of these groups. In 103 consecutive cases of pathologically confirmed PSP, we have identified two clinical phenotypes by factor analysis which we have named Richardson's syndrome (RS) and PSP-parkinsonism (PSP-P). Cases of RS syndrome made up 54% of all cases, and were characterized by the early onset of postural instability and falls, supranuclear vertical gaze palsy and cognitive dysfunction. A second group of 33 (32%) were characterized by asymmetric onset, tremor, a moderate initial therapeutic response to levodopa and were frequently confused with Parkinson's disease (PSP-P). Fourteen cases (14%) could not be separated according to these criteria. In RS, two-thirds of cases were men, whereas the sex distribution in PSP-P was even. Disease duration in RS was significantly shorter (5.9 versus 9.1 years, P < 0.001) and age at death earlier (72.1 versus 75.5 years, P = 0.01) than in PSP-P. The isoform composition of insoluble tangle-tau isolated from the basal pons also differed significantly. In RS, the mean four-repeat:three-repeat tau ratio was 2.84 and in PSP-P it was 1.63 (P < 0.003). The effect of the H1,H1 PSP susceptibility genotype appeared stronger in RS than in PSP-P (odds ratio 13.2 versus 4.5). The difference in genotype frequencies between the clinical subgroups was not significant. There were no differences in apolipoprotein E genotypes. The classic clinical description of PSP, which includes supranuclear gaze palsy, early falls and dementia, does not adequately describe one-third of cases in this series

  14. Genetics Underlying Atypical Parkinsonism and Related Neurodegenerative Disorders.

    PubMed

    Scholz, Sonja W; Bras, Jose

    2015-10-16

    Atypical parkinsonism syndromes, such as dementia with Lewy bodies, multiple system atrophy, progressive supranuclear palsy and corticobasal degeneration, are neurodegenerative diseases with complex clinical and pathological features. Heterogeneity in clinical presentations, possible secondary determinants as well as mimic syndromes pose a major challenge to accurately diagnose patients suffering from these devastating conditions. Over the last two decades, significant advancements in genomic technologies have provided us with increasing insights into the molecular pathogenesis of atypical parkinsonism and their intriguing relationships to related neurodegenerative diseases, fueling new hopes to incorporate molecular knowledge into our diagnostic, prognostic and therapeutic approaches towards managing these conditions. In this review article, we summarize the current understanding of genetic mechanisms implicated in atypical parkinsonism syndromes. We further highlight mimic syndromes relevant to differential considerations and possible future directions.

  15. Wolff-Parkinson-White syndrome: lessons learnt and lessons remaining.

    PubMed

    Benson, D Woodrow; Cohen, Mitchell I

    2017-01-01

    The Wolff-Parkinson-White pattern refers to the electrocardiographic appearance in sinus rhythm, wherein an accessory atrioventricular pathway abbreviates the P-R interval and causes a slurring of the QRS upslope - the "delta wave". It may be asymptomatic or it may be associated with orthodromic reciprocating tachycardia; however, rarely, even in children, it is associated with sudden death due to ventricular fibrillation resulting from a rapid response by the accessory pathway to atrial fibrillation, which itself seems to result from orthodromic reciprocating tachycardia. Historically, patients at risk for sudden death were characterised by the presence of symptoms and a shortest pre- excited R-R interval during induced atrial fibrillation <250 ms. Owing to the relatively high prevalence of asymptomatic Wolff-Parkinson-White pattern and availability of catheter ablation, there has been a need to identify risk among asymptomatic patients. Recent guidelines recommend invasive evaluation for such patients where pre-excitation clearly does not disappear during exercise testing. This strategy has a high negative predictive value only. The accuracy of this approach is under continued investigation, especially in light of other considerations: Patients having intermittent pre-excitation, once thought to be at minimal risk may not be, and the role of isoproterenol in risk assessment.

  16. Rivastigmine as a Symptomatic Treatment for Apathy in Parkinson's Dementia Complex: New Aspects for This Riddle

    PubMed Central

    2017-01-01

    Over 90% of PDD patients show at least one neuropsychiatric symptom (NPS); in the 60–70% two or more NPS are present. Their incidence is important in terms of prognosis and severity of pathology. However, among all NPS, apathy is often the most disturbing, associated with greater caregiver's burden. Similar to other NPS, apathy may be due to a dysfunction of the nigrostriatal pathway, even though, not all the PD patients become apathetic, indicating that apathy should not entirely be considered a dopamine-dependent syndrome, and in fact it might also be related to acetylcholine defects. Apathy has been treated in many ways, without sure benefits; among these, Rivastigmine may present benefic properties. We present a series of 48 patients, suffering from PDD, treated with Rivastigmine, and followed-up for one year; they have been devotedly studied for apathy, even though all the other NPS disorders have been registered. Rivastigmine did not have a prolonged benefic effect on apathy, in our work, on the contrary of what had been observed in the literature, probably due to the longer follow-up of our patients. PMID:28409049

  17. Clinical characteristics of leg restlessness in Parkinson's disease compared with idiopathic Restless Legs Syndrome.

    PubMed

    Zhu, Xiao-Ying; Liu, Ye; Zhang, Xiao-Jin; Yang, Wen-Hao; Feng, Ya; Ondo, William G; Tan, Eng-King; Wu, Yun-Cheng

    2015-10-15

    There is limited data on motor restlessness in Parkinson's disease (PD). Here we evaluate for clinical differences between cohorts of idiopathic Restless Legs Syndrome (iRLS), PD patients with leg restlessness, and PD with RLS. We examined 276 consecutive PD patients for leg restlessness symptoms, we compared clinical features of PD patients with RLS, PD patients with leg restlessness but not meeting RLS criteria, PD patient without RLS and iRLS. A total of 262 PD patients who satisfied the inclusion criteria were analyzed. After excluding 23 possible secondary RLS or mimics, 28 were diagnosed with RLS and 18 with leg motor restlessness (LMR). Compared with iRLS patients, PD patients with RLS or LMR had older age of RLS/LMR onset, shorter duration of leg restlessness, less positive family history, different seasonal trends and more unilaterality of leg restlessness symptom (P<0.01) which were often in accordance with dominant Parkinsonism side and related with Parkinsonism severity. PD patients with RLS/LMR had lower daily dosage (P<0.01) and shorter duration (P<0.05) of dopaminergic medication when RLS/LMR symptom onset than PD without leg restlessness. PD with LMR had less severe Parkinsonism (P<0.05) and leg restlessness (P<0.01) symptoms than PD with RLS. Clinical characteristics of PD patients with RLS and LMR were different from iRLS, differentiating these various subtypes can facilitate optimal treatment. Copyright © 2015 Elsevier B.V. All rights reserved.

  18. Genetics Home Reference: Wolff-Parkinson-White syndrome

    MedlinePlus

    ... related to changes in the regulation of certain ion channels in the heart. These channels, which transport positively ... White Syndrome: a disease of glycogen storage or ion channel dysfunction? J Cardiovasc Electrophysiol. 2006 May;17 Suppl ...

  19. Shorter telomeres may indicate dementia status in older individuals with Down Syndrome

    PubMed Central

    Jenkins, Edmund C.; Ye, Lingling; Gu, Hong; Ni, Samantha A.; Velinov, Milen; Pang, Deborah; Krinsky-McHale, Sharon J.; Zigman, Warren B.; Schupf, Nicole; Silverman, Wayne P.

    2009-01-01

    We have recently reported reduced telomere length in T lymphocytes of individuals with Down syndrome (DS) and dementia due to Alzheimer’s disease (AD). We have now replicated and extended that study by finding that people with DS and mild cognitive impairment (MCI-DS) also have shorter telomeres than people with DS without MCI-DS. Additional new findings demonstrated that light intensity measurements from chromosome 21 alone, or in concert with chromosomes 1, 2, and 16, exhibited shorter telomeres in adults with DS and with either dementia or MCI-DS compared to aging per se. Chromosome 21 measurements appeared to be especially promising for use as a biomarker because there was no overlap in the distribution of light intensity measurement scores between demented or MCI-DS and non-demented participants. Given that early clinical symptoms of AD can be very difficult to recognize in this population of adults due to their pre-existing cognitive impairments, a valid biomarker would be of great value. Early detection is especially important because it would allow treatments to begin before significant damage to the central nervous system has occurred. Our findings suggest that it may be feasible to use telomere shortening as a biomarker for accurately inferring dementia status. PMID:18635289

  20. Anxiety has specific syndromal profiles in Parkinson disease: a data-driven approach.

    PubMed

    Starkstein, Sergio E; Dragovic, Milan; Dujardin, Kathy; Marsh, Laura; Martinez-Martin, Pablo; Pontone, Gregory M; Richard, Irene H; Weintraub, Daniel; Leentjens, Albert F G

    2014-12-01

    Anxiety symptoms are common in Parkinson disease (PD). Recent evidence suggests that anxiety syndromes as encountered in clinical practice may not correspond to the DSM-IV classification of anxiety disorders. To examine the syndromal pattern of the anxiety spectrum in a large series of patients with PD, as determined with a data-driven approach using latent class analysis (LCA). 342 patients with PD were recruited from referrals to movement disorders or psychiatry clinics at six tertiary centers. Participants were assessed with a structured psychiatric interview and specific scales rating the severity of anxiety, depression, cognition and parkinsonism. The main outcome measure was classes of patients with a specific syndromal profile of anxiety symptoms based on LCA. LCA identified four classes that were interpreted as "no anxiety or depression", "episodic anxiety without depression", "persistent anxiety with depression", and "both persistent and episodic anxiety with depression". Symptoms of persistent anxiety were almost invariably associated with symptoms of depression. There were significant differences between classes in terms of history of depression and anxiety, use of psychoactive medication, and on the Mentation and Complications sections of the Unified Parkinson Disease Rating Scale. Patients with PD show different syndromic profiles of anxiety that do not align with the symptom profiles represented by DSM-IV anxiety disorders and major depression. Accordingly, DSM-IV criteria for anxiety disorders may not be clinically useful in PD. The different classes identified here provide empirically validated phenotypes for future research. Copyright © 2014 American Association for Geriatric Psychiatry. Published by Elsevier Inc. All rights reserved.

  1. Early-onset Parkinson's Disease Associated with Chromosome 22q11.2 Deletion Syndrome.

    PubMed

    Oki, Mitsuaki; Hori, Shin-ichiro; Asayama, Shinya; Wate, Reika; Kaneko, Satoshi; Kusaka, Hirofumi

    2016-01-01

    We herein report the case of a 43-year-old man with a 4-year history of resting tremor and akinesia. His resting tremor and rigidity were more prominent on the left side. He also presented retropulsion. His symptoms responded to the administration of levodopa. The patient also had a cleft lip and palate, cavum vergae, and hypoparathyroidism. A chromosome analysis disclosed a hemizygous deletion in 22q11.2, and he was diagnosed with early-onset Parkinson's disease associated with 22q11.2 deletion syndrome. However, the patient lacked autonomic nerve dysfunction, and his cardiac uptake of (123)I-metaiodobenzylguanidine was normal, indicating an underlying pathological mechanism that differed to that of sporadic Parkinson's disease.

  2. Suppression of refractory arrhythmias by aprindine in patients with the Wolff-Parkinson-White syndrome.

    PubMed Central

    Reid, P R; Greene, H L; Varghese, P J

    1977-01-01

    Four patients with supraventricular tachycardia associated with the Wolff-Parkinson-White syndrome were refractory to conventional pharmacological therapy and received aprindine hydrochloride intravenously and orally. Electrophysiological studies disclosed that intravenous aprindine caused increased refractoriness and slowed conduction in the atria, atrioventricular node, ventricles, and accessory pathway. The ability to induce supraventricular tachycardia with timed atrial and ventricular premature stimuli was totally abolished in all 4 patients after intravenous aprindine. Oral aprindine therapy, twice daily thereafter, provided symptomatic relief of the supraventricular tachycardia without significant side effects. Aprindine is useful in the management of supraventricular tachycardia associated with Wolff-Parkinson-White and may offer significant advantages over currently available therapy. Images PMID:603737

  3. Dysexecutive syndrome in Parkinson's disease: the GREFEX study.

    PubMed

    Roussel, Martine; Lhommée, Eugénie; Narme, Pauline; Czernecki, Virginie; Gall, Didier Le; Krystkowiak, Pierre; Diouf, Momar; Godefroy, Olivier

    2016-09-01

    The objectives of this study were to characterize the frequencies and profiles of behavioral and cognitive dysexecutive syndromes in PD (based on validated battery and diagnostic criteria) and to develop a shortened diagnostic battery. Eighty-eight non-demented patients with a diagnosis of PD were examined with an executive validated battery. Using a validated framework, the patients' test results were interpreted with respect to normative data from 780 controls. A dysexecutive syndrome was observed in 80.6% of the patients [95% confidence interval: 71.1-90.1]. The dysexecutive profile was characterized by prominent impairments in deduction, flexibility, inhibition and initiation in the cognitive domain, and by global hypoactivity with apathy and hyperactivity in the behavioral domain. This finding implies that patients with PD should be assessed with cognitive tests and a validated inventory for behavioral dysexecutive syndromes. A shortened battery (based on three cognitive tests and three behavioral domains) provided high diagnostic accuracy.

  4. [Parkinson syndrome, a possible adverse effect of calcium inhibitors].

    PubMed

    Malaterre, H R; Lauribe, P; Paganelli, F; Ramond, B; Lévy, S

    1992-09-01

    The effects of calcium inhibitors are not limited to the muscles and may affect other systems and cause varied side effects. Two cases of Parkinsonian syndrome occurring after starting therapy with calcium inhibitors (verapamil in one case and diltiazem in the other) are reported. Complete regression of the symptoms after withdrawing the drugs was strongly in favour of a causal relationship. The condition could be due to inhibition of the calcium channels in the central nervous system disturbing neurotransmission. This seems to be a rare side effect as there have only been three other reported cases of secondary extrapyramidal syndromes in the literature. However, a Parkinsonian syndrome is very invalidating and clinicians using this family of drugs should be aware of this possible complication.

  5. Impulse control disorders and dopamine dysregulation syndrome associated with dopamine agonist therapy in Parkinson's disease.

    PubMed

    Fenu, Sandro; Wardas, Jadwiga; Morelli, Micaela

    2009-09-01

    Over the last decade, evidence has emerged linking disorders in the impulsive-compulsive spectrum in Parkinson's disease to dopamine receptor agonist treatment. These disorders include hypersexuality, gambling and, to a minor extent, compulsive shopping and eating, as well as dopamine dysregulation syndrome, characterized by an addictive pattern toward dopamine replacement therapy and stereotyped behaviors, such as punding. These syndromes, which have only recently been recognized and are still underdiagnosed, have deleterious social consequences that warrant interventions at the clinical level and promotion of research at the preclinical level. In this review, we first provide a summary of features of Parkinson's disease and current pharmacological therapies associated with the development of dopamine dysregulation syndrome and impulsive-compulsive disorders. We also examine the dopamine receptors and brain areas important in reward and compulsive behaviors. We then critically examine the neuroadaptations in dopaminergic circuitries and the literature concerning gambling, hypersexuality, and other addictive behaviors in parkinsonian patients. Finally, we focus on suggestions pointing to a role for dopamine D(3) receptors and sensitization phenomena as the main factors which may be the origin of these disorders.

  6. Diffusion tensor MRI contributes to differentiate Richardson's syndrome from PSP-parkinsonism.

    PubMed

    Agosta, Federica; Pievani, Michela; Svetel, Marina; Ječmenica Lukić, Milica; Copetti, Massimiliano; Tomić, Aleksandra; Scarale, Antonio; Longoni, Giulia; Comi, Giancarlo; Kostić, Vladimir S; Filippi, Massimo

    2012-12-01

    This study investigated the regional distribution of white matter (WM) damage in Richardson's syndrome (PSP-RS) and progressive supranuclear palsy-Parkinsonism (PSP-P) using diffusion tensor (DT) magnetic resonance imaging (MRI). The DT MRI classificatory ability in diagnosing progressive supranuclear palsy (PSP) syndromes, when used in combination with infratentorial volumetry, was also quantified. In 37 PSP (21 PSP-RS, 16 PSP-P) and 42 controls, the program Tract-Based Spatial Statistics (TBSS; www.fmrib.ox.ac.uk/fsl/tbss) was applied. DT MRI metrics were derived from supratentorial, thalamic, and infratentorial tracts. The magnetic resonance parkinsonism index (MRPI) was calculated. All PSP harbored diffusivity abnormalities in the corpus callosum, frontoparietal, and frontotemporo-occipital tracts. Infratentorial WM and thalamic radiations were severely affected in PSP-RS and relatively spared in PSP-P. When MRPI and DT MRI measures were combined, the discriminatory power increased for each comparison. Distinct patterns of WM alterations occur in PSP-RS and PSP-P. Adding DT MRI measures to MRPI improves the diagnostic accuracy in differentiating each PSP syndrome from healthy individuals and each other.

  7. [Effects of mexiletine in the Wolff-Parkinson-White syndrome].

    PubMed

    Touboul, P; Gressard, A; Kirkorian, G; Atallah, G

    1981-11-01

    The effects of intravenous mexiletine were studied by His bundle recordings and programmed stimulation in 7 patients aged from 20 to 40 years old (average age : 32 years), 5 of whom had an overt WPW syndrome (4 type A, and 1 type B), and 2 of whom had concealed pre excitation. All had reciprocating tachycardia. Electrophysiological investigation was performed under basal conditions, and then several times in the hour following intravenous mexiletine (3,5 mg/Kg in 5 minutes) followed by an infusion of 0,07 mg/Kg/min. The following results were obtained : 1. mexiletine did not cause any significant changes in the normal AV conduction pathway; 2. the refractory periods of the atria and ventricles were unaffected by the drug; 3. pre excitation regressed in 2 of the 5 patients with overt WPW. In a third patient, the effective anterograde refractory period of the accessory pathway increased by 210 ms. The retrograde refractory period of the accessory pathways increased in three patients and remained unchanged in the others; 4. there was little change in the ability to induce reciprocating tachycardia by stimulation. However, in two patients, the attacks were shortened, terminating spontaneously within a few seconds. This study shows the electrophysiological basis of the use of mexiletine in the WPW syndrome. Although the results do show some variability, they justify the use of mexiletine in patients with paroxysmal tachycardia in the WPW syndrome.

  8. Spinal Anaesthesia is Safe in a Patient with Wolff-Parkinson-White Syndrome Undergoing Evacuation of Molar Pregnancy

    PubMed Central

    Pujari, Vinayak S

    2016-01-01

    Wolff-Parkinson-White (WPW) syndrome is an uncommon cardiac condition where there is an abnormal band of atrial tissue connecting atria and ventricles which can electrically bypass atrioventricular node. The anaesthetic management in these patients is challenging as life threatening complications can occur perioperatively like paroxysmal supraventricular tachycardia and atrial fibrillation. Also, regional anaesthetic technique like subarachnoid block is a safe and cost effective alternative to general anaesthesia as it avoids polypharmacy. We report the successful anaesthetic management of Wolff Parkinson White syndrome in a primi with hydatiform mole posted for suction and evacuation. PMID:27042562

  9. [Treatment of junctional paroxysmal tachycardia, without patent Wolff-Parkinson-White syndrome, by sectioning an accessory Kent-His bundle].

    PubMed

    Slama, R; Attuel, P; Flammang, D; Coumel, P; Guiraudon, G

    1976-11-01

    The authors report the case of a patient suffering from a Bouveret's tachycardia without syndrome of Wolff-Parkinson-White. The analysis of the tachycardic spells however showed that during a reciprocal crisis, the circuit went through a left accessory ventriculo-atrial bundle, functioning only in the reverse direction. This accessory bundle was successfully cut by the surgeon, following the procedure of wide atrioventricular desinsertion as described by the authors of Duke University for the surgical treatment of the Wolff-Parkinson-White syndrome.

  10. Types of Dementia

    MedlinePlus

    ... Normal pressure hydrocephalus Huntington's disease Wernicke-Korsakoff Syndrome Alzheimer's disease Most common type of dementia; accounts for an ... and death in the brain. Learn more about Alzheimer's disease . Vascular dementia back to top Previously known as ...

  11. C9orf72 repeat expansions are a rare genetic cause of parkinsonism

    PubMed Central

    Lesage, Suzanne; Le Ber, Isabelle; Condroyer, Christel; Broussolle, Emmanuel; Gabelle, Audrey; Thobois, Stéphane; Pasquier, Florence; Mondon, Karl; Dion, Patrick A.; Rochefort, Daniel; Rouleau, Guy A.; Dürr, Alexandra; Brice, Alexis

    2013-01-01

    The recently identified C9ORF72 gene accounts for a large proportion of amyotrophic lateral sclerosis and frontotemporal lobar degenerations. Since several forms of these disorders are associated with parkinsonism, we hypothesized that some patients with Parkinson’s disease or other forms of parkinsonism might carry pathogenic C9OFR72 expansions. Therefore, we looked for C9ORF72 repeat expansions in 1,446 parkinsonian unrelated patients consisted of 1,225 clinically diagnosed with Parkinson’s disease, 123 with progressive supranuclear palsy, 21 with corticobasal degeneration syndrome, 43 with Lewy body dementia and 25 with multiple system atrophy-parkinsonism. Of the 1,446 parkinsonian patients, five carried C9ORF72 expansions: three patients with typical Parkinson’s disease, one with corticobasal degeneration syndrome and another with progressive supranuclear palsy. This study shows that: i) although rare, C9ORF72 repeat expansions may be associated with clinically typical Parkinson’s disease, but also with other parkinsonism; ii) in several patients, parkinsonism was dopa-responsive and remained pure, without associated dementia, for more than 10 years; iii) interestingly, all C9ORF72 repeat expansion carriers had positive family histories of parkinsonism, degenerative dementias or amyotrophic lateral sclerosis. This study also provides the tools for identifying parkinsonian patients with C9ORF72 expansions, with important consequences for genetic counseling. PMID:23413259

  12. High incidence of carpal tunnel syndrome after deep brain stimulation in Parkinson's disease.

    PubMed

    Loizon, Marine; Laurencin, Chloé; Vial, Christophe; Danaila, Teodor; Thobois, Stéphane

    2016-12-01

    We observed several cases of carpal tunnel syndrome (CTS) revealed after subthalamic nucleus deep brain stimulation (STN-DBS) in Parkinson's disease (PD). 115 consecutive PD patients who underwent STN-DBS between 2010 and 2014 at the Neurological Hospital in Lyon were retrospectively included. CTS was accepted as the diagnosis only if clinical examination and ENMG both confirmed it. Nine patients (7.8 %) developed CTS in the 2 years following surgery, which is far beyond the 2.7/1000 incidence in the general population. The present study shows an overrepresentation of CTS occurrence after STN-DBS in PD.

  13. Practitioner-Raised Issues and End-of-Life Care for Adults with Down Syndrome and Dementia

    ERIC Educational Resources Information Center

    Watchman, Karen

    2005-01-01

    The author interviewed a small group of practitioners working in intellectual disability and palliative care settings about their perceptions of a number of end-of-life issues related to people with Down syndrome who were affected by dementia. The study, which took place in Scotland, identified a number of issues and perceptions expressed by the…

  14. Practitioner-Raised Issues and End-of-Life Care for Adults with Down Syndrome and Dementia

    ERIC Educational Resources Information Center

    Watchman, Karen

    2005-01-01

    The author interviewed a small group of practitioners working in intellectual disability and palliative care settings about their perceptions of a number of end-of-life issues related to people with Down syndrome who were affected by dementia. The study, which took place in Scotland, identified a number of issues and perceptions expressed by the…

  15. Comparative effects of ajmaline on intermittent bundle branch block and the Wolff-Parkinson-White syndrome.

    PubMed

    Chiale, P A; Przybylski, J; Halpern, M S; Lazzari, J O; Elizari, M V; Rosenbaum, M B

    1977-05-04

    Phase 4 or phase 3 block or both occurred in the His bundle branch system of 11 patients with intermittent bundle branch block and in the anomalous bundle of 6 of 46 patients with the Wolff-Parkinson-White syndrome (13%). Administration of a single dose of ajmaline (50 mg intravenously) in these patients caused a similar response: expansion of the range of phase 3 and phase 4 block at the expense of the intermediate normal conduction range and total interruption of conduction in the affected fascicle when the effect of the drug was maximal. The great similarity in physiologic behavior and pharmacologic response in these groups of patients suggests that the anomalous bundle was probably diseased or abnormal in the six patients with Wolff-Parkinson-White conduction. In addition, ajmaline caused the first appearance of phase 4 or phase 3 block, or both, but not total interruption of conduction in 26 of the 46 patients with Wolff-Parkinson-White conduction (56.5%). Ajmaline does not cause fascicular block in normal subjects; thus this finding suggests either that the anomalous bundle is diseased or that the safety margin for conduction in the anomalous bundle is much narrower than in the bundle branch system. The conduction-depressing action of ajmaline may be greater at relatively rapid or relatively slow rates of stimulation, and smaller or absent at intermediate rates.

  16. Down Syndrome Disintegrative Disorder: New-Onset Autistic Regression, Dementia, and Insomnia in Older Children and Adolescents With Down Syndrome.

    PubMed

    Worley, Gordon; Crissman, Blythe G; Cadogan, Emily; Milleson, Christie; Adkins, Deanna W; Kishnani, Priya S

    2015-08-01

    Over a 10-year period in a Down syndrome Clinic, 11 children and adolescents were encountered with a history of new-onset (8) or worsening (3) autistic characteristics. Ten of the 11 (91%) had cognitive decline to a dementia-like state and 9 of the 11 (82%) new-onset insomnia. The mean age at which symptoms developed was 11.4 years (standard deviation = 3.6 years; range 5-14 years), an older age than usual for autistic regression in Down syndrome. Ten of 11 cases (91%) had elevated ("positive") thyroperoxidase antibody titers compared to only 5 of 21 (23%) age-matched control subjects with Down syndrome (P < .001). At follow-up at a mean age of 20.7 years (standard deviation = 3.9 years), 8 of the 11 (73%) were at least somewhat better. Down syndrome disintegrative disorder seems an appropriate name for this newly recognized clinical association, which may be due to autoimmunity. © The Author(s) 2014.

  17. Anomalies occurring in lipid profiles and protein distribution in frontal cortex lipid rafts in dementia with Lewy bodies disclose neurochemical traits partially shared by Alzheimer's and Parkinson's diseases.

    PubMed

    Marin, Raquel; Fabelo, Noemí; Martín, Virginia; Garcia-Esparcia, Paula; Ferrer, Isidre; Quinto-Alemany, David; Díaz, Mario

    2017-01-01

    Lipid rafts are highly dynamic membrane microdomains intimately associated with cell signaling. Compelling evidence has demonstrated that alterations in lipid rafts are associated with neurodegenerative diseases such Alzheimer's disease, but at present, whether alterations in lipid raft microdomains occur in other types of dementia such dementia with Lewy bodies (DLB) remains unknown. Our analyses reveal that lipid rafts from DLB exhibit aberrant lipid profiles including low levels of n-3 long-chain polyunsaturated fatty acids (mainly docosahexaenoic acid), plasmalogens and cholesterol, and reduced unsaturation and peroxidability indexes. As a consequence, lipid raft resident proteins holding principal factors of the β-amyloidogenic pathway, including β-amyloid precursor protein, presenilin 1, β-secretase, and PrP, are redistributed between lipid rafts and nonraft domains in DLB frontal cortex. Meta-analysis discloses certain similarities in the altered composition of lipid rafts between DLB and Parkinson's disease which are in line with the spectrum of Lewy body diseases. In addition, redistribution of proteins linked to the β-amyloidogenic pathway in DLB can facilitate generation of β-amyloid, thus providing mechanistic clues to the intriguing convergence of Alzheimer's disease pathology, particularly β-amyloid deposition, in DLB. Copyright © 2016 Elsevier Inc. All rights reserved.

  18. [Electrophysiologic effects of amiodarone in Wolf-Parkinson-White syndrome].

    PubMed

    Touboul, P; Porte, J; Huerta, F; Delahaye, J P

    1976-08-01

    Eight patients with WPW syndrome were catheterised and, during the course of this investigation, the electrophysiological effects of amiodarone were assessed. By registering the potentials of the bundle of His and by using the stimulus-test technique, we were able to measure the refractory periods of the atrium and of the normal and accessory conducting pathways both before and during the first 40 minutes after an intra-atrial injection of 5 mg/kg of amiodarone chlorhydrate. The action of the conduction time was also studied. In the five cases in which we were able to measure it, the effective refractory period of the abnormal pathway increased, which led in two instances to the temporary suppression of all pre-excitation. At the same time, it was repeatedly found that the refractory periods of the A-V node were increased: the effective refractory period in 3/3 cases, and the functional refractory period in 2/2 cases. The effective refractory period of the right atrium was increased in 5 cases, and did not change in the others. The intranodal conduction time (A-H- interval) was always increased after amiodarone. Finally, in three patients runs of reciprocal tachycardia could be initiated by premature atrial stimulation. In one case, this was no longer possible after amiodarone. In the other two cases, although the attacks could still be brought on, they were slower because of the lengthening of the A-H interval. These findings explain why amiodarone is effective in controlling the tachycardia of WPW syndrome.

  19. [Wolff-Parkinson-White syndrome after 50 yars of age. Clinical and electrophysiological data].

    PubMed

    Lévy, S; Borde, C; Dupon, J; Bémurat, M; Gérard, R; Bricaud, H

    1980-07-01

    The Wolff-Parkinson-White syndrome is usually observed in young people and is much rarer in patients over 50 years old. This fact may be explained by the demise of a certain number of patients before the age of 50 and/or a change in the clinical features of the syndrome with age and/or of the electrophysiological properties of the normal and accessory conduction pathways. To test the latter hypothesis, the clinical and electrophysiological data of 15 patients over 50 years old with the Wolff-Parkinson-White syndrome (Group I) were compared with that of 10 patients under 30 years old with the same syndrome (Group II). The same protocol of electrophysiological investigation was used in both groups of patients. The results showed a significant difference (p < 0.001) between the two groups in the incidence of associated cardiac disease. This was more common in Group I (1 4 out of 15 patients) than in Group II (2 out of 10 patients). The cardiothoracic ratio was significantly higher in Group I (p < 0.01). The two groups also differed in the age at which tachycardia first occured. 9 out of 11 patients in Group I only had symptoms after thirty years. On the other hand, there was no significant difference in the types of tachycardia and the frequency of attacks. There was no significant difference in QRS, PR, AH, HV intervals, in the ventriculo-atrial conduction time and the effective refractory periods of the atrium, right ventricle or atrio-ventricular node. There was no significant difference in the anterograde and retrograde refractory periods of the accessory pathways between the two groups. Reciprocating tachycardia, initiated by electrical stimulation in 7 patients in Group I and 6 patients in Group II, was conducted anterogradely to the ventricles through the normal pathway and retrogradely to the atria through the the accessory pathway. This study suggest that age-related changes in the electrophysiological properties of the accessory are not an important

  20. [Polymorphic atrial tachycardia and Wolff-Parkinson-White syndrome in a newborn infant].

    PubMed

    Chantepie, A; Ramponi, N; Vaillant, M C; Laugier, J; Raynaud, P; Fauchier, J P

    1986-08-01

    The authors report a case of polymorphic supraventricular tachycardia in a premature neonate born at 33 weeks by caesarean section because of foeto-placental insufficiency and hydramnios due to foetal tachycardia diagnosed in utero. This arrhythmia was of interest because of the association of chaotic atrial tachycardia and the Wolff-Parkinson-White syndrome (WPW), which has rarely been described in the neonate. The mechanism of atrial tachycardia in the WPW syndrome is variable. In our case, there was retrograde atrial activation by the accessory pathway with atrial desynchronisation aided by left atrial dilatation. Digoxin, an effective anti arrhythmic agent in neonatal tachycardia, should not be used in cases of atrial tachycardia associated with ventricular preexcitation because of the risk of dangerous ventricular tachycardia.

  1. [Current treatment of Wolff-Parkinson-White syndrome and ventricular tachycardia: surgical ablation versus catheter ablation?].

    PubMed

    Misaki, T; Watanabe, G; Iwa, T; Watanabe, Y

    1992-09-01

    From November 1973, 454 patients with Wolff-Parkinson-White syndrome underwent surgical ablation of accessory pathways. Overall curative rate was 94% in our series including 65 cases of simultaneous surgical repair for combined heart diseases. In recent months, radiofrequency catheter ablation was applied in 7 cases. There has been 2 failures, which have taken more than 2 hours of radiation exposure and have required surgery. There has been 47 patients who underwent surgical ablation for non-ischemic ventricular tachycardia. Forty cases (85%) had a successful outcome of surgical ablation and another 2 cases required DC catheter ablation postoperatively to eliminate ventricular tachycardias. In conclusion, radiofrequency ablation of WPW syndrome in patients without combined heart disease or multiple accessory pathways is feasible. Surgical ablation is effective and safe technique compared with catheter ablation in patients with ventricular tachycardia.

  2. Induction of a transient dysexecutive syndrome in Parkinson's disease using a~subclinical dose of scopolamine.

    PubMed

    Bédard, M.A.; Lemay, S.; Gagnon, J.F.; Masson, H.; Paquet, F.

    1998-01-01

    Parkinson's Disease (PD) is often associated with a subcortico-frontal syndrome (SCFS) that is mainly characterized by executive dysfunctions. The complete biochemistry of these dysfunctions remain misunderstood although many studies have suggested a role of the dopaminergic lesions. However, cholinergic lesions in this disease may also account for the SCFS occurrence. The present study has assessed the effects of an acute subclinical dose of scopolamine in normal controls and in PD patients who were devoid of cognitive deficit. Results indicates that PD patients but not normal controls developed a transient SCFS for the duration of the drug action. In contrast to other populations with cholinergic depletions - such as Alzheimer's disease - cholinergic blockage in PD exacerbates specifically the dysexecutive syndrome without inducing amnesia or sedation. Such a discrepancy between these two neuropsychological profiles are discussed in terms of the specificity of the underlying cholinergic lesions.

  3. The epidemiology and clinical manifestations of dysexecutive syndrome in Parkinson's disease.

    PubMed

    Ceravolo, Roberto; Pagni, Cristina; Tognoni, Gloria; Bonuccelli, Ubaldo

    2012-01-01

    This mini-review summarizes the evidence of the cognitive and behavioral features of dysexecutive syndrome in Parkinson's disease (PD). Deficits in response inhibition, set-shifting, mental flexibility, and strategy have been frequently described from the earliest stages of PD, although there are inconsistencies in study findings due to the complexity of the executive function (EF) construct and methodological limitations. Behavioral disorders of PD, e.g., apathy, distractibility, perseverative behavior, and impulse-control disorders, may be viewed as the other side of dysexecutive syndrome. Despite the interrelationship between the cognitive and behavioral domains, some reports reveal that the two syndromes may be dissociated, suggesting that both aspects must be clinically assessed. EFs are widely associated with the prefrontal areas, although dysexecutive syndrome may be observed in patients with damage to other brain regions. EFs drive numerous abilities essential to daily life, such as prospective remembering and language comprehension, which may be impaired in PD subjects. Considering the impact of dysexecutive syndrome on independence and quality of life, early detection of executive impairment is crucial in the management of PD.

  4. Systemic mitochondrial dysfunction and the etiology of Alzheimer's disease and down syndrome dementia.

    PubMed

    Coskun, Pinar E; Wyrembak, Joanne; Derbereva, Olga; Melkonian, Goar; Doran, Eric; Lott, Ira T; Head, Elizabeth; Cotman, Carl W; Wallace, Douglas C

    2010-01-01

    Increasing evidence is implicating mitochondrial dysfunction as a central factor in the etiology of Alzheimer's disease (AD). The most significant risk factor in AD is advanced age and an important neuropathological correlate of AD is the deposition of amyloid-beta peptide (Abeta40 and Abeta42) in the brain. An AD-like dementia is also common in older individuals with Down syndrome (DS), though with a much earlier onset. We have shown that somatic mitochondrial DNA (mtDNA) control region (CR) mutations accumulate with age in post-mitotic tissues including the brain and that the level of mtDNA mutations is markedly elevated in the brains of AD patients. The elevated mtDNA CR mutations in AD brains are associated with a reduction in the mtDNA copy number and in the mtDNA L-strand transcript levels. We now show that mtDNA CR mutations increase with age in control brains; that they are markedly elevated in the brains of AD and DS and dementia (DSAD) patients; and that the increased mtDNA CR mutation rate in DSAD brains is associated with reduced mtDNA copy number and L-strand transcripts. The increased mtDNA CR mutation rate is also seen in peripheral blood DNA and in lymphoblastoid cell DNAs of AD and DSAD patients, and distinctive somatic mtDNA mutations, often at high heteroplasmy levels, are seen in AD and DSAD brain and blood cells DNA. In aging, DS, and DSAD, the mtDNA mutation level is positively correlated with beta-secretase activity and mtDNA copy number is inversely correlated with insoluble Abeta40 and Abeta42 levels. Therefore, mtDNA alterations may be responsible for both age-related dementia and the associated neuropathological changes observed in AD and DSAD.

  5. [Fitness for sports of patients with Wolff-Parkinson-White syndrome].

    PubMed

    Montgermont, P; Adams, C; Heulin, A; Vacheron, A

    1987-06-01

    The fitness of patients with Wolff-Parkinson-White syndrome to indulge in sporting activities is a practical cardiology problem. The major risk is sudden death due to atrial fibrillation deteriorating to ventricular fibrillation. This risk is small or even theoretical, but signing a fitness certificate engages the clinician's responsibility. Non invasive complementary examinations are useful. Echocardiography may detect a heart disease that would preclude any sport. Exercise tests explore the behaviour of the accessory pathway and rarely trigger off arrhythmias. Holter recordings mainly investigate disorders of the atrial rhythm. The decision concerning fitness may be based on clinical symptoms. Exercise-induced tachycardia is a classical contra-indication to competitive sports. In patients whose tachycardia is unrelated to exercise, fitness may be discussed according to the results of exercise tests and of the electrophysiological study. A refractory period which would be considered as rather prolonged at rest does not protect against fast ventricular rate during passage to atrial fibrillation. If pre-excitation disappears during the exercise test in an asymptomatic patient, then competitive sports can be authorized without limitations. If not, only surgical excision or fulguration would provide full protection against a potentially dangerous fibrillation. It is concluded that Wolff-Parkinson-White syndrome contra-indicates competitive sports in most cases. Games played outside competitions remain possible in the absence of symptoms or when arrhythmias are well controlled by medical treatment.

  6. Memory and the creation of art: the syndrome, as in de Kooning, of 'creating in the midst of dementia'.

    PubMed

    Espinel, Carlos Hugo

    2007-01-01

    The creation of abstract art demands high intellectual capacities. Willem de Kooning, nonetheless, accomplished his last paintings while crippled by impairments diagnosed as Alzheimer's disease. Until my research neither art nor science offered an explanation, or a thinking method, for identifying this phenomenon, for solving a mystery that pertains to human discovery and creation. In this 'ArtScience' study of the de Kooning phenomenon I use 'Thinking Methods' which I devised by a systematic application of information on the workings of the brain, the 'Brain Methods'. With my 'Method of Observation' I examined de Kooning's paintings, created both before and during dementia, and found them comparable in technique and expression. This demonstrates the authenticity of art created in dementia. With my 'Cognitive Analysis' I identified in de Kooning the syndrome herein called 'Creating in the Midst of Dementia'. This Syndrome, unique in mechanisms and presentation, is characterized by (1) a specific combination of brain functions and malfunctions, in this case, preservation of three memory systems - working, procedural, and episodic - and deficit of the semantic memory system, and by (2) a response to precise stimuli, one that triggers brain reactivation, and as a consequence enables creating in the midst of dementia. My work offers: (1) The identification of the 'Syndrome of Creating in the Midst of Dementia'. It (a) presents its definition, the criteria of diagnosis, mechanism, rationale, and possibility of 'cognitive repair' and (b) solves the mystery of de Kooning creating art while crippled by dementia. (2) New Thinking Methods, the 'Brain Methods', that based on information on the workings of the brain, open a new path in the pursuit of truth. The 'Method of Observation' serves to define stimuli, and the 'Cognitive Analysis' to assess cognitive faculties. (3) The integration of art and science into a new discipline of study, 'ArtScience'.

  7. Effects of gabapentin enacarbil on restless legs syndrome and leg pain in dementia with Lewy bodies.

    PubMed

    Fujishiro, Hiroshige

    2014-06-01

    Restless legs syndrome (RLS) is a common neurological disorder. Dementia with Lewy bodies (DLB) is the second most common form of neurodegenerative dementia after Alzheimer's disease. Both RLS and DLB can be effectively treated by dopaminergic medications, suggesting the role of dopamine dysfunction in the pathogenesis of both diseases. Here, I report on a Japanese woman with probable DLB and RLS who was treated with gabapentin enacarbil, a non-dopaminergic agent. Because a dopamine agonist, a first-line therapy for moderate to severe RLS, caused the occurrence of metamorphopsia, an alternative treatment of gabapentin enacarbil was used; this treatment improved the patient's RLS without worsening her psychiatric symptoms. An alternative treatment is desirable for DLB patients with RLS because they often experience intolerable side-effects with a dopamine agonist, especially visual hallucinations. Administering gabapentin enacarbil also improved the continuous leg pain that occurred in conjunction with the development of RLS. Although the neurobiological mechanism in the development of pain remains unclear, a range of non-dopaminergic structures likely mediated pain processing in DLB in the present case based on neuropharmacological results. This is the first report reporting the effects of gabapentin enacarbil for RLS and leg pain in a DLB patient with psychiatric symptoms. © 2014 The Author. Psychogeriatrics © 2014 Japanese Psychogeriatric Society.

  8. Does the Well-Being of Individuals with Down Syndrome and Dementia Improve When Using Life Story Books and Rummage Boxes? A Randomized Single Case Series Experiment

    ERIC Educational Resources Information Center

    Crook, Nicola; Adams, Malcolm; Shorten, Nicola; Langdon, Peter E.

    2016-01-01

    Background: This study investigated whether a personalized life story book and rummage box enhanced well-being and led to changes in behaviour for people with Down syndrome (DS) who have dementia. Materials and Methods: A randomized single case series design was used with five participants who had DS and a diagnosis of dementia. Participants were…

  9. Does the Well-Being of Individuals with Down Syndrome and Dementia Improve When Using Life Story Books and Rummage Boxes? A Randomized Single Case Series Experiment

    ERIC Educational Resources Information Center

    Crook, Nicola; Adams, Malcolm; Shorten, Nicola; Langdon, Peter E.

    2016-01-01

    Background: This study investigated whether a personalized life story book and rummage box enhanced well-being and led to changes in behaviour for people with Down syndrome (DS) who have dementia. Materials and Methods: A randomized single case series design was used with five participants who had DS and a diagnosis of dementia. Participants were…

  10. [Is the transesophageal approach preferable to endocavitary approach in the evaluation of Wolff-Parkinson-White syndrome?].

    PubMed

    Brembilla-Perrot, B; Beurrier, D

    1995-03-01

    Now that the radical treatment of the Wolff-Parkinson-White syndrome is established, it is essential to evaluate the prognosis of this condition accurately. Initiation of atrial fibrillation is one of the factors which influence the prognosis. The aim of this study was to compare the results of electrophysiological studies performed by the endocavitary and transoesophageal approaches in the measurement of the initiation of atrial fibrillation. Twenty-six patients with a patent Wolff-Parkinson-White syndrome were studied by the two methods with a similar protocol: incremental atrial pacing to the Wenckebach point, programmed atrial stimulation using up to two extrastimuli, repeated with an infusion of 20 to 30 ug of isoproterenol. Sixteen patients had reciprocating nodal tachycardia or were asymptomatic (group I) and the other 10 had spontaneous atrial fibrillation (group II). In group I, atrial fibrillation was induced in 9 cases (56%) by the endocavitary and in two cases (12.5%) by the transoesophageal method. In group II, spontaneous atrial fibrillation was reproduced in all cases by the endocavitary and transoesophageal protocols. None of the patients in group I developed atrial fibrillation during follow-up (average 2 years +/- 9 months). The authors observe that all spontaneous atrial fibrillation of the Wolff-Parkinson-White syndrome can be triggered by oesophageal stimulation. The prevalence of atrial fibrillation was overestimated by endocavitary studies in asymptomatic or paucisymptomatic patients. The assessment of atrial vulnerability of a Wolff-Parkinson-White syndrome may therefore be performed by transoesophageal electro-physiological studies.

  11. Venlafaxine-associated serotonin syndrome causing severe rhabdomyolysis and acute renal failure in a patient with idiopathic Parkinson disease.

    PubMed

    Rajapakse, Senaka; Abeynaike, Lakshan; Wickramarathne, Thanushi

    2010-10-01

    A 43-year-old male patient with idiopathic Parkinson disease, on dopaminergic therapy, was admitted with confusion and agitation, diaphoresis, and hyperkinesia after the commencement of the serotonin-noradrenaline reuptake inhibitor venlafaxine 2 weeks prior for depression. He was found to have severe rhabdomyolysis and developed acute renal failure. The most likely diagnosis was serotonin syndrome induced by venlafaxine, although neuroleptic malignant syndrome was also considered. The differential diagnosis, atypical features in this presentation, and possible mechanisms are discussed.

  12. [Association of Wolff-Parkinson-White syndrome with isolated non-compaction of the left ventricle: a case report].

    PubMed

    Brembilla-Perrot, B; Codreanu, A; Marie, P Y; Beurrier, D; Husson, J L; Hutin, O; Pruna, A; Yangni N'Da, O; Ernst, Y; Bosser, G

    2006-06-01

    The Wolff-Parkinson-White syndrome (WPW) may be associated with a number of cardiac pathologies, especially congenital disease, in 7.5 to 17% of cases. The authors report a rare association of the WPW syndrome with two Kent bundles, right and left septal, with non-compaction of the left ventricle in a 52 year old man. This was a chance finding during systematic echocardiography after ablation, and confirmed by cardiac MRI. The patient was asymptomatic.

  13. Cognitive and Psychiatric Disturbances in Parkinsonian Syndromes.

    PubMed

    Zweig, Richard M; Disbrow, Elizabeth A; Javalkar, Vijayakumar

    2016-02-01

    Parkinsonian syndromes share clinical signs including akinesia/bradykinesia and rigidity, which are consequences of pathology involving dopaminergic substantia nigra neurons. Yet cognitive and psychiatric disturbances are common, even early in the course of disease. Executive dysfunction is often measurable in newly diagnosed Parkinson's disease. Treatment with dopaminergic medications, particularly dopamine agonists, has been associated with hallucinations and impulse control disorder. Older age, presence of APOE-4 gene, and/or other factors result in amyloid plaque deposition that, in turn, accelerates cortical Lewy body plus tau pathology, linking Dementia with Lewy Bodies and Parkinson's disease with early dementia with Alzheimer's disease. Treatments available for cognitive deficits, depression, and psychotic symptoms are discussed.

  14. Assessing Specific Cognitive Deficits Associated with Dementia in Older Adults with Down Syndrome: Use and Validity of the Arizona Cognitive Test Battery (ACTB)

    PubMed Central

    Sinai, Amanda; Hassiotis, Angela; Rantell, Khadija; Strydom, Andre

    2016-01-01

    Background Down syndrome is associated with specific cognitive deficits. Alongside this, older adults with Down syndrome are a high risk group for dementia. The Arizona Cognitive Test Battery (ACTB), a cognitive assessment battery specifically developed for use with individuals with Down syndrome, has been proposed for use as outcome measures for clinical trials in this population. It has not been validated in older adults with Down syndrome. This study aims to assess the use and validity of the ACTB in older adults with Down syndrome. Methods Participants with Down syndrome aged 45 and over were assessed using the ACTB, standard tabletop tests and informant ratings. Results Assessment outcomes of 49 participants were analysed. Of these, 19 (39%) had a diagnosis of dementia or possible dementia. Most participants were able to attempt most of the tasks, although some tasks had high floor effects (including CANTAB Intra-Extra Dimensional shift stages completed and Modified Dots Task). Of the ACTB tasks, statistically significant differences were observed between the dementia and no dementia groups on CANTAB Simple Reaction Time median latency, NEPSY Visuomotor Precision—Car and Motorbike and CANTAB Paired Associates Learning stages completed. No significant differences were observed for CANTAB Intra-Extra Dimensional Shift, Modified Dots Task, Finger Sequencing, NEPSY Visuomotor precision—Train and Car and CANTAB Paired Associates Learning first trial memory score. Several of the tasks in the ACTB can be used in older adults with Down syndrome and have mild to moderate concurrent validity when compared to tabletop tests and informant ratings, although this varies on a test by test basis. Conclusions Overall, scores for a number of tests in the ACTB were similar when comparing dementia and no dementia groups of older adults with Down syndrome, suggesting that it would not be an appropriate outcome measure of cognitive function for clinical trials of dementia

  15. Assessing Specific Cognitive Deficits Associated with Dementia in Older Adults with Down Syndrome: Use and Validity of the Arizona Cognitive Test Battery (ACTB).

    PubMed

    Sinai, Amanda; Hassiotis, Angela; Rantell, Khadija; Strydom, Andre

    2016-01-01

    Down syndrome is associated with specific cognitive deficits. Alongside this, older adults with Down syndrome are a high risk group for dementia. The Arizona Cognitive Test Battery (ACTB), a cognitive assessment battery specifically developed for use with individuals with Down syndrome, has been proposed for use as outcome measures for clinical trials in this population. It has not been validated in older adults with Down syndrome. This study aims to assess the use and validity of the ACTB in older adults with Down syndrome. Participants with Down syndrome aged 45 and over were assessed using the ACTB, standard tabletop tests and informant ratings. Assessment outcomes of 49 participants were analysed. Of these, 19 (39%) had a diagnosis of dementia or possible dementia. Most participants were able to attempt most of the tasks, although some tasks had high floor effects (including CANTAB Intra-Extra Dimensional shift stages completed and Modified Dots Task). Of the ACTB tasks, statistically significant differences were observed between the dementia and no dementia groups on CANTAB Simple Reaction Time median latency, NEPSY Visuomotor Precision-Car and Motorbike and CANTAB Paired Associates Learning stages completed. No significant differences were observed for CANTAB Intra-Extra Dimensional Shift, Modified Dots Task, Finger Sequencing, NEPSY Visuomotor precision-Train and Car and CANTAB Paired Associates Learning first trial memory score. Several of the tasks in the ACTB can be used in older adults with Down syndrome and have mild to moderate concurrent validity when compared to tabletop tests and informant ratings, although this varies on a test by test basis. Overall, scores for a number of tests in the ACTB were similar when comparing dementia and no dementia groups of older adults with Down syndrome, suggesting that it would not be an appropriate outcome measure of cognitive function for clinical trials of dementia treatments without further modification and

  16. A core avenue for transcultural research on dementia: on the cross-linguistic generalization of language-related effects in Alzheimer's disease and Parkinson's disease.

    PubMed

    Calvo, Noelia; Ibáñez, Agustín; Muñoz, Edinson; García, Adolfo M

    2017-03-28

    Language is a key source of cross-cultural variability, which may have both subtle and major effects on neurocognition. However, this issue has been largely overlooked in two flourishing lines of research assessing the relationship between language-related neural systems and dementia. This paper assesses the limitations of the evidence on (i) the neuroprotective effects of bilingualism in Alzheimer's disease and (ii) specific language deficits as markers of Parkinson's disease. First, we outline the rationale behind each line of research. Second, we review available evidence and discuss the potential impact of cross-linguistic factors. Third, we outline ideas to foster progress in both fields and, with it, in cross-cultural neuroscience at large. On the one hand, studies on bilingualism suggest that sustained use of more than one language may protect against Alzheimer's disease symptoms. On the other hand, insights from the embodied cognition framework point to syntactic and action-verb deficits as early (and even preclinical) markers of Parkinson's disease. However, both fields share a key limitation that lies at the heart of cultural neuroscience: the issue of cross-linguistic generalizability. Relevant evidence for both research trends comes from only a handful of (mostly Indo-European) languages, which are far from capturing the full scope of structural and typological diversity of the linguistic landscape worldwide. This raises questions on the external validity of reported findings. Greater collaboration between linguistic typology and cognitive neuroscience seems crucial as a first step to assess the impact of transcultural differences on language-related effects across neurodegenerative diseases. Copyright © 2017 John Wiley & Sons, Ltd. Copyright © 2017 John Wiley & Sons, Ltd.

  17. Differential Patterns of Planning Impairments in Parkinson's Disease and Sub-Clinical Signs of Dementia? A Latent-Class Model-Based Approach

    PubMed Central

    Stahl, Christoph; Kaller, Christoph P.

    2012-01-01

    Planning impairments mark a well-documented consequence of neurodegenerative diseases such as Parkinson's disease (PD). Recently, using the Tower of London task we demonstrated that, rather than being generally impaired, PD patients selectively fail when planning requires flexible in-breadth search strategies. For a better understanding of the interindividual patterns underlying specific planning impairments, here we performed an explorative re-analysis of the original data using a latent-class model-based approach. Data-driven classification according to subjects' performance was based on a multinomial processing tree (MPT) model accommodating the impact of increased breadth versus depth of looking ahead during planning. In order to assess interindividual variability in coping with these different task demands, an extension of MPT models was used in which sample-immanent heterogeneity is accounted for by identifying different latent classes of individuals. Two latent classes were identified that differed considerably in performance for problems placing high demands on the depth of anticipatory search processes. In addition, these impairments were independent of PD diagnosis. However, latent-class mediated search depth-related deficits in planning performance were associated with poorer outcomes in dementia screenings, albeit sub-clinical. PD patients exhibited additional deficits related to the breadth of searching ahead. Taken together, results revealed dissociable impairments in specific planning processes within a single task of visuospatial problem solving. Present analyses put forward the hypothesis that cognitive sequelae of PD and sub-clinical signs of dementia may be related to differential patterns of planning impairments. PMID:22715417

  18. Regional brain amyloid-β accumulation associates with domain-specific cognitive performance in Parkinson disease without dementia.

    PubMed

    Akhtar, Rizwan S; Xie, Sharon X; Chen, Yin J; Rick, Jacqueline; Gross, Rachel G; Nasrallah, Ilya M; Van Deerlin, Vivianna M; Trojanowski, John Q; Chen-Plotkin, Alice S; Hurtig, Howard I; Siderowf, Andrew D; Dubroff, Jacob G; Weintraub, Daniel

    2017-01-01

    Parkinson disease patients develop clinically significant cognitive impairment at variable times over their disease course, which is often preceded by milder deficits in memory, visuo-spatial, and executive domains. The significance of amyloid-β accumulation to these problems is unclear. We hypothesized that amyloid-β PET imaging by 18F-florbetapir, a radiotracer that detects fibrillar amyloid-β plaque deposits, would identify subjects with global cognitive impairment or poor performance in individual cognitive domains in non-demented Parkinson disease patients. We assessed 61 non-demented Parkinson disease patients with detailed cognitive assessments and 18F-florbetapir PET brain imaging. Scans were interpreted qualitatively (positive or negative) by two independent nuclear medicine physicians blinded to clinical data, and quantitatively by a novel volume-weighted method. The presence of mild cognitive impairment was determined through an expert consensus process using Level 1 criteria from the Movement Disorder Society. Nineteen participants (31.2%) were diagnosed with mild cognitive impairment and the remainder had normal cognition. Qualitative 18F-florbetapir PET imaging was positive in 15 participants (24.6%). Increasing age and presence of an APOE ε4 allele were associated with higher composite 18F-florbetapir binding. In multivariable models, an abnormal 18F-florbetapir scan by expert rating was not associated with a diagnosis of mild cognitive impairment. However, 18F-florbetapir retention values in the posterior cingulate gyrus inversely correlated with verbal memory performance. Retention values in the frontal cortex, precuneus, and anterior cingulate gyrus retention values inversely correlated with naming performance. Regional cortical amyloid-β amyloid, as measured by 18F-florbetapir PET, may be a biomarker of specific cognitive deficits in non-demented Parkinson disease patients.

  19. Executive dysfunction using behavioral assessment of the dysexecutive syndrome in Parkinson's disease.

    PubMed

    Kamei, Satoshi; Hara, Motohiko; Serizawa, Kan; Murakami, Masato; Mizutani, Tomohiko; Ishiburo, Motoko; Kawahara, Ritsuko; Takagi, Yukiko; Ogawa, Katsuhiko; Yoshihashi, Hirokazu; Shinbo, Satoru; Suzuki, Yutaka; Yamaguchi, Mai; Morita, Akihiko; Takeshita, Jun; Hirayanagi, Kaname

    2008-03-15

    The objective of this study was to evaluate the executive dysfunction (ExD) in Parkinson's disease (PD) using the Behavioral Assessment of the Dysexecutive Syndrome (BADS), which provides a wide-range assessment of ExD. The BADS and the Unified Parkinson's Disease Rating Scale (UPDRS) were investigated in 63 nondemented PD patients who revealed scores of >or=24 points on the Mini-Mental State Examination based on the DSM-IV. Multiple logistic regression analysis was performed to evaluate the predisposing factors to ExD, which was defined as <70 points on the age-controlled standardized score. The total score on the UPDRS was a significant independent predisposing factor to ExD. Among the various parts of the UPDRS, part II was the significant factor for ExD. The profile scores of all subtests on the BADS in patients with ExD were significantly lower than those of patients without ExD. All profile scores decreased with severity of PD, but the changes among these scores differed. ExD in nondemented PD predisposed to a greater severity of PD, particularly as regards the activity of daily living impairment. Nondemented PD revealed wide-range components of ExD. All components of ExD were impaired with severity of PD, but the patterns of each component exhibited variety.

  20. [Wolf-Parkinson-White syndrome. Intensive physical activity: the value of fulguration].

    PubMed

    Warin, J F; Haissaguerre, M; Le Métayer, P; Montserrat, P

    1989-08-01

    In subjects with Wolff-Parkinson-White syndrome an intense physical activity or the practice of sports may not only trigger off cardiac arrhythmias but also worsen their consequences and become life-threatening. A full electrophysiological study, including measurement of the anterograde refractory period of the accessory pathway, induction of atrial fibrillation and study of the effects of isoprenaline, seems to be indispensable to detect those patients who are most at risk. When the risk of potentially serious arrhythmia appears to be confirmed, catheter ablation of the accessory pathway may be the ideal solution, as it may cure the disease without the sequelae inherent in surgery. The results obtained in 19 athletes or subjects with intense physical activity (19) successes without preventive anti-arrhythmic treatment and at the cost of a single case of asymptomatic atrioventricular block) suggest that the catheter ablation technique will greatly benefit such patients.

  1. [Wolff-Parkinson-White syndrome and longitudinal dissociation of the atrioventricular node. Anatomical and electrophysiological correlates].

    PubMed

    Brechenmacher, C; Fauchier, J P; James, T N

    1981-07-01

    A 58 year old man who died of metastatic carcinoma had undergone electrophysiological investigation 4 years previously for a Wolff-Parkinson-White syndrome (Rosenbaum Type A, Frank and Boineau Type IV) associated with supraventricular tachycardia (SVT) at 180/mn, atrial fibrillation and flutter and slow junctional (or low atrial) rhythm at 70-80/mn. Atrial extrasystoles or appropriate atrial stimulation not only induced and terminated the SVT but also the junctional rhythm and allowed passage from one arrhythmia to another. These studies showed the presence of a left lateral Kent bundle responsible for orthodromic SVT with retrograde conduction through the accessory pathway, and suggested that the junctional rhythm might be due to longitudinal dissociation of the AV node. Autopsy findings confirmed the presence of the left posterolateral Kent bundle in an almost horizontal position, parallel to the mitral annulus (it might therefore have escaped eventual surgical section) and the longitudinal dissociation of the AV node.

  2. Biomagnetic noninvasive localization of accessory pathways in Wolff-Parkinson-White syndrome.

    PubMed

    Weismüller, P; Abraham-Fuchs, K; Schneider, S; Richter, P; Kochs, M; Edrich, J; Hombach, V

    1991-11-01

    It was our purpose to assess the clinical relevance of noninvasive magnetocardiographic localization of accessory pathways. Nine patients with Wolff-Parkinson-White (WPW) syndrome were studied. For all of them the site of the accessory pathway was known from invasive catheter mapping. A 37-SQUID (superconducting quantum interference device) sensor multichannel system (KRENIKON) was used, allowing synchronous registration with all channels. The site of the electrophysiological activity at the beginning of the delta wave was determined. Magnetic resonance images of the heart were obtained to correlate the biomagnetically localized activity with the anatomy. Magnetocardiographic localization of the bypass tract corresponded with catheter mapping with a spatial difference of 0-5 cm, 1.8 cm on the average, compared to the results obtained by catheter mapping. Thus, magnetocardiography is a promising new method for noninvasive localization of accessory pathways in WPW patients. This may streamline further invasive procedures.

  3. Effect of exercise on ventricular response to atrial fibrillation in Wolff-Parkinson-White syndrome.

    PubMed Central

    Crick, J C; Davies, D W; Holt, P; Curry, P V; Sowton, E

    1985-01-01

    Ten patients with Wolff-Parkinson-White syndrome underwent cardiac electrophysiological study extended to include the induction of atrial fibrillation at maximum exercise in the upright position. This was performed using a new temporary bipolar lead with a helical active fixation tip for atrial pacing. The highest rate of atrioventricular conduction via the accessory pathway was greater during exercise than at rest in all 10 patients (mean increase 28%). In three cases the resulting ventricular rate exceeded 300 beats/min, but no patient had severe symptoms or ventricular arrhythmias. The exercise induced enhancement of accessory pathway conduction may significantly but unpredictably affect the risk from spontaneous atrial fibrillation especially in patients with coronary artery disease or in those taking antiarrhythmic drugs. The test procedure was sufficiently simple and well tolerated to be included in our routine electrophysiological investigation. PMID:4015920

  4. [Genesis of auricular fibrillation in the Wolff-Parkinson-White syndrome].

    PubMed

    Gressard, A; Atallah, G; Chatelain, M T; Touboul, P

    1981-11-01

    Atrial fibrillation seems to be more common in the absence of associated cardiac disease in the Wolff-Parkinson-White syndrome (WPW) than in subjects of the same age without this condition. The aim of this study was to analyse the electrophysiological mechanism of AF and to establish its relationship to the accessory pathway. The series comprises 14 out of 51 patients with WPW undergoing classical endocavitary investigation associating the recording of cardiac potentials from the His bundle, right atrium (RA), left atrium (LA) via the coronary sinus and atrial and ventricular stimulation techniques. Three mechanisms of inducing AF were analysed : - AF triggered by RA stimulation : either by a premature extra stimulus or overdrive atrial pacing. In all cases, the accessory pathway was right sides. - AF triggered by overdrive ventricular pacing : three cases were left sided accessory pathways in which atrial desynchronisation was localised in the LA. - Conversion of reciprocating tachycardia to AF (9 cases). In 2 cases, this was preceded by a progressive acceleration of the heart rate. Of 3 left sided accessory pathways, the atrial desynchronisation was located in the LA in 2 cases. The factors which facilitate AF in THE WPW syndrome are discussed : increased atrial vulnerability, the role of the rapid return of ventricular excitation to the atria through the accessory pathway. Our observations suggest that the accessory pathway plays a role in the genesis of AF in the WPW syndrome.

  5. [Should the Isuprel test be performed systematically in Wolff-Parkinson-White syndrome?].

    PubMed

    Brembilla-Perrot, B; Terrier de la Chaise, A; Marçon, F; Cherrier, F; Pernot, C

    1988-10-01

    The isoprenaline (Is) test was designed by Wellens et al. in 1982 to evaluate the effect of catecholamines on the effective refractory period (ERP) of Kent's bundle (K). The purpose of our study was to assess the value of this test in the prognosis of Wolff-Parkinson-White syndrome (WPW), to define its criteria of severity and to determine the usefulness of the test. Out of 33 patients with WPW syndrome, 10 (group I) had a clinical history of severe arrhythmia and 23 (group II) were asymptomatic or had paroxysmal nodal tachycardia. The prognosis of WPW syndrome was evaluated by measuring Kent's bundle ERP under coupled atrial stimulation (S1 S2) and the shortest cycle conducted by K during induced atrial fibrillation (AF) and atrial pacing (AP) both in the basal state (B) and under a 20-30 micrograms Is infusion. (table; see text). Analysis of the results showed constant shortening of ERP in group I and reproduction of the clinical tachycardia in 6 cases. In group II patients isoprenaline unmasked the WPW syndrome in 3 cases and reproduced the clinical tachycardia in 5 cases. The ERP of Kent's bundle evaluated by S1 S2 became smaller or equal to 220 ms in 70 p. 100 of the cases, and this shortening was not specific. The shortest cycle in AF or AP became inferior of equal to 220 ms in only 6 cases, the history being concordant with clinical findings in 4 of them. Altogether, the most reliable and simplest way of evaluating the severity of WPW syndrome is the highest frequency conducted by Kent's bundle in atrial pacing during the Is test which should be performed in all patients in view of its specificity, simplicity and safety.

  6. Evaluating the relationship between amyloid-β and α-synuclein phosphorylated at Ser129 in dementia with Lewy bodies and Parkinson's disease.

    PubMed

    Swirski, Marta; Miners, J Scott; de Silva, Rohan; Lashley, Tammaryn; Ling, Helen; Holton, Janice; Revesz, Tamas; Love, Seth

    2014-01-01

    Lewy body and Alzheimer-type pathologies often co-exist. Several studies suggest a synergistic relationship between amyloid-β (Aβ) and α-synuclein (α-syn) accumulation. We have explored the relationship between Aβ accumulation and the phosphorylation of α-syn at serine-129 (pSer129 α-syn), in post-mortem human brain tissue and in SH-SY5Y neuroblastoma cells transfected to overexpress human α-syn. We measured levels of Aβ40, Aβ42, α-syn and pSer129 α-syn by sandwich enzyme-linked immunosorbent assay, in soluble and insoluble fractions of midfrontal, cingulate and parahippocampal cortex and thalamus, from cases of Parkinson's disease (PD) with (PDD; n = 12) and without dementia (PDND; n = 23), dementia with Lewy bodies (DLB; n = 10) and age-matched controls (n = 17). We also examined the relationship of these measurements to cognitive decline, as measured by time-to-dementia and the mini-mental state examination (MMSE) score in the PD patients, and to Braak tangle stage. In most brain regions, the concentration of insoluble pSer129 α-syn correlated positively, and soluble pSer129 α-syn negatively, with the levels of soluble and insoluble Aβ. Insoluble pSer129 α-syn also correlated positively with Braak stage. In most regions, the levels of insoluble and soluble Aβ and the proportion of insoluble α-syn that was phosphorylated at Ser129 were significantly higher in the PD and DLB groups than the controls, and higher in the PDD and DLB groups than the PDND brains. In PD, the MMSE score correlated negatively with the level of insoluble pSer129 α-syn. Exposure of SH-SY5Y cells to aggregated Aβ42 significantly increased the proportion of α-syn that was phosphorylated at Ser129 (aggregated Aβ40 exposure had a smaller, non-significant effect). Together, these data show that the concentration of pSer129 α-syn in brain tissue homogenates is directly related to the level of Aβ and Braak tangle stage, and predicts cognitive status in Lewy body diseases.

  7. Some observations on the spectrum of dementia.

    PubMed

    Jha, Sanjeev; Patel, R

    2004-06-01

    A study was designed to generate epidemiological and clinical data on dementia, in a teaching hospital in India. It was conducted on 124 (94 male and 30 female) elderly patients (aged more than 60 years) presenting with clinical syndrome of dementia (DSM-3). Their age range was 64-78 (mean 65.7 4.1) years. Detailed clinical, biochemical, radiological and electrophysiological evaluation was done to establish etiology. Patients with psychiatric ailments, cranial trauma and tumors were excluded. The study period was 4.2 years. Multi-infarct dementia (MID) was observed to be commonest cause of dementia and was present in 59 (47.6%) cases. There were 10 (8%) patients each of tuberculosis (TB) and neurocysticercosis (NCC). Alcohol-related dementia was present in 13 (10.5%), while malnutrition (Vitamin B12 deficiency) was present in 9 (7.2%). Alzheimer's Disease (AD) was present (NINCDS-ADRDA criteria) in 6 patients (4.8%). There were 3 (2.4%) cases 1 each of Huntington's disease, Parkinson's and Normal Pressure Hydrocephalus and 2 each of diabetes, hypothyroidism, hyperthyroidism and Creutzfeldt' Jakob Disease. We conclude that AD, which is irreversible and common in the west, is relatively uncommon in India as compared to MID, infections and malnutrition, which are potentially treatable.

  8. The behavioural variant frontotemporal dementia (bvFTD) syndrome in psychiatry

    PubMed Central

    Lanata, Serggio C; Miller, Bruce L

    2016-01-01

    The primary goal of this article is to critically discuss the syndromic overlap that exists between early behavioural variant frontotemporal dementia (bvFTD)—the most common clinical syndrome associated with frontotemporal lobar degeneration (FTLD)—and several primary psychiatric disorders. We begin by summarising the current state of knowledge regarding FTLD, including the recent discovery of FTLD-causative genetic mutations. Clinicopathological correlations in FTLD are subsequently discussed, while emphasising that clinical syndromes of FTD are dictated by the distribution of FTLD pathology in the brain. We then review a large number of cases with suspected and confirmed bvFTD that had previously been diagnosed with a primary psychiatric disorder. The clinical and neuroscientific implications of this overlap are discussed, focusing on the importance of early diagnosis for clinical and therapeutic reasons. We propose that largely due to the paucity of biomarkers for primary psychiatric disorders, and the limited use of FTLD-related biomarkers by psychiatrists at present, it is very difficult to separate patients with early bvFTD from those with primary psychiatric disorders based on clinical grounds. Furthermore, specific limitations of the Diagnostic and Statistical Manual of Mental Disorders (DSM) 5 criteria for bvFTD may inadvertently discourage recognition of bvFTD in mental health settings. Clinically, more research is needed to develop tools that allow early differentiation of bvFTD from primary psychiatric disease, as bvFTD therapies will likely be most effective in the earliest stages of disease. From a neuroscience perspective, we argue that bvFTD provides an excellent paradigm for investigating the neural basis of psychiatric disorders. PMID:26216940

  9. Dopaminergic Therapy Modulates Cortical Perfusion in Parkinson Disease With and Without Dementia According to Arterial Spin Labeled Perfusion Magnetic Resonance Imaging.

    PubMed

    Lin, Wei-Che; Chen, Pei-Chin; Huang, Yung-Cheng; Tsai, Nai-Wen; Chen, Hsiu-Ling; Wang, Hung-Chen; Lin, Tsu-Kung; Chou, Kun-Hsien; Chen, Meng-Hsiang; Chen, Yi-Wen; Lu, Cheng-Hsien

    2016-02-01

    Arterial spin labeling (ASL) magnetic resonance imaging analyses allow for the quantification of altered cerebral blood flow, and provide a novel means of examining the impact of dopaminergic treatments. The authors examined the cerebral perfusion differences among 17 Parkinson disease (PD) patients, 17 PD with dementia (PDD) patients, and 17 healthy controls and used ASL-MRI to assess the effects of dopaminergic therapies on perfusion in the patients. The authors demonstrated progressive widespread cortical hypoperfusion in PD and PDD and robust effects for the dopaminergic therapies. Specifically, dopaminergic medications further decreased frontal lobe and cerebellum perfusion in the PD and PDD groups, respectively. These patterns of hypoperfusion could be related to cognitive dysfunctions and disease severity. Furthermore, desensitization to dopaminergic therapies in terms of cortical perfusion was found as the disease progressed, supporting the concept that long-term therapies are associated with the therapeutic window narrowing. The highly sensitive pharmaceutical response of ASL allows clinicians and researchers to easily and effectively quantify the absolute perfusion status, which might prove helpful for therapeutic planning.

  10. Dopaminergic Therapy Modulates Cortical Perfusion in Parkinson Disease With and Without Dementia According to Arterial Spin Labeled Perfusion Magnetic Resonance Imaging

    PubMed Central

    Lin, Wei-Che; Chen, Pei-Chin; Huang, Yung-Cheng; Tsai, Nai-Wen; Chen, Hsiu-Ling; Wang, Hung-Chen; Lin, Tsu-Kung; Chou, Kun-Hsien; Chen, Meng-Hsiang; Chen, Yi-Wen; Lu, Cheng-Hsien

    2016-01-01

    Abstract Arterial spin labeling (ASL) magnetic resonance imaging analyses allow for the quantification of altered cerebral blood flow, and provide a novel means of examining the impact of dopaminergic treatments. The authors examined the cerebral perfusion differences among 17 Parkinson disease (PD) patients, 17 PD with dementia (PDD) patients, and 17 healthy controls and used ASL-MRI to assess the effects of dopaminergic therapies on perfusion in the patients. The authors demonstrated progressive widespread cortical hypoperfusion in PD and PDD and robust effects for the dopaminergic therapies. Specifically, dopaminergic medications further decreased frontal lobe and cerebellum perfusion in the PD and PDD groups, respectively. These patterns of hypoperfusion could be related to cognitive dysfunctions and disease severity. Furthermore, desensitization to dopaminergic therapies in terms of cortical perfusion was found as the disease progressed, supporting the concept that long-term therapies are associated with the therapeutic window narrowing. The highly sensitive pharmaceutical response of ASL allows clinicians and researchers to easily and effectively quantify the absolute perfusion status, which might prove helpful for therapeutic planning. PMID:26844450

  11. A Comparison of Substantia Nigra T1 Hyperintensity in Parkinson's Disease Dementia, Alzheimer's Disease and Age-Matched Controls: Volumetric Analysis of Neuromelanin Imaging

    PubMed Central

    Park, Ju-Yeon; Yun, Won-Sung; Jeon, Ji Yeong; Moon, Yeon Sil; Kim, Heejin; Kwak, Ki-Chang; Lee, Jong-Min; Han, Seol-Heui

    2016-01-01

    Objective Neuromelanin loss of substantia nigra (SN) can be visualized as a T1 signal reduction on T1-weighted high-resolution imaging. We investigated whether volumetric analysis of T1 hyperintensity for SN could be used to differentiate between Parkinson's disease dementia (PDD), Alzheimer's disease (AD) and age-matched controls. Materials and Methods This retrospective study enrolled 10 patients with PDD, 18 patients with AD, and 13 age-matched healthy elderly controls. MR imaging was performed at 3 tesla. To measure the T1 hyperintense area of SN, we obtained an axial thin section high-resolution T1-weighted fast spin echo sequence. The volumes of interest for the T1 hyperintense SN were drawn onto heavily T1-weighted FSE sequences through midbrain level, using the MIPAV software. The measurement differences were tested using the Kruskal-Wallis test followed by a post hoc comparison. Results A comparison of the three groups showed significant differences in terms of volume of T1 hyperintensity (p < 0.001, Bonferroni corrected). The volume of T1 hyperintensity was significantly lower in PDD than in AD and normal controls (p < 0.005, Bonferroni corrected). However, the volume of T1 hyperintensity was not different between AD and normal controls (p = 0.136, Bonferroni corrected). Conclusion The volumetric measurement of the T1 hyperintensity of SN can be an imaging marker for evaluating neuromelanin loss in neurodegenerative diseases and a differential in PDD and AD cases. PMID:27587951

  12. Alpha-synuclein in the cerebrospinal fluid differentiates synucleinopathies (Parkinson Disease, dementia with Lewy bodies, multiple system atrophy) from Alzheimer disease.

    PubMed

    Tateno, Fuyuki; Sakakibara, Ryuji; Kawai, Takayuki; Kishi, Masahiko; Murano, Takeyoshi

    2012-01-01

    We examined the utility of quantification of α-synuclein (SNCA) in the cerebrospinal fluid (CSF) to differentiate patients with Alzheimer disease (AD), dementia with Lewy bodies (DLB), Parkinson disease (PD), and multiple system atrophy (MSA). Thirty-seven patients were divided into 4 age-matched and sex-matched clinical groups: AD (n = 9), DLB (n = 6), PD (n = 11), and MSA (n = 11). Eleven subjects served as neurological disease controls. The total of 48 subjects included 27 men and 21 women, aged 66.5 ± 11.4 years. We performed a solid-phase sandwich enzyme-linked immunosorbent assay, which enables the sensitive quantification of CSF SNCA. In comparison with controls, CSF SNCA levels in AD were significantly higher (P < 0.05). CSF SNCA levels in PD (P < 0.001), DLB (P < 0.01), and MSA (P < 0.05) were all significantly lower than those in AD. However, CSF SNCA levels did not differ significantly among the 3 synucleinopathies. The results of the present study suggest that quantification of CSF SNCA helps in the differentiation of synucleinopathies (PD, DLB, and MSA) from AD. However, CSF SNCA levels did not differ significantly among the 3 synucleinopathies.

  13. Extrapolation-Based References Improve Motion and Eddy-Current Correction of High B-Value DWI Data: Application in Parkinson's Disease Dementia.

    PubMed

    Nilsson, Markus; Szczepankiewicz, Filip; van Westen, Danielle; Hansson, Oskar

    2015-01-01

    Conventional motion and eddy-current correction, where each diffusion-weighted volume is registered to a non diffusion-weighted reference, suffers from poor accuracy for high b-value data. An alternative approach is to extrapolate reference volumes from low b-value data. We aim to compare the performance of conventional and extrapolation-based correction of diffusional kurtosis imaging (DKI) data, and to demonstrate the impact of the correction approach on group comparison studies. DKI was performed in patients with Parkinson's disease dementia (PDD), and healthy age-matched controls, using b-values of up to 2750 s/mm2. The accuracy of conventional and extrapolation-based correction methods was investigated. Parameters from DTI and DKI were compared between patients and controls in the cingulum and the anterior thalamic projection tract. Conventional correction resulted in systematic registration errors for high b-value data. The extrapolation-based methods did not exhibit such errors, yielding more accurate tractography and up to 50% lower standard deviation in DKI metrics. Statistically significant differences were found between patients and controls when using the extrapolation-based motion correction that were not detected when using the conventional method. We recommend that conventional motion and eddy-current correction should be abandoned for high b-value data in favour of more accurate methods using extrapolation-based references.

  14. The clinical and neurobehavioral course of Down syndrome and dementia with or without new-onset epilepsy.

    PubMed

    Gholipour, Taha; Mitchell, Sara; Sarkis, Rani A; Chemali, Zeina

    2017-03-01

    Adult patients with Down syndrome (DS) are at higher risk of developing Alzheimer-type dementia and epilepsy. The relationship between developing dementia and the risk of developing seizures in DS is poorly characterized to date. In addition, treatment response and medication tolerability have not been rigorously studied. We identified 220 patients with a diagnosis of DS and dementia. Those without a history of developing seizures (DD) were compared to patients with new-onset seizures (DD+S) after the age of 35. Electronic records were reviewed for demographics, seizure characteristics, cognitive status, and psychiatric comorbidities. Of the patients included for analysis, twenty-six out of 60 patients had new-onset seizures or developed seizures during the follow-up period (the DD+S group) with a median onset of 2.0years after the dementia diagnosis. Generalized tonic-clonic seizures were the most common seizure type (61.5% of DD+S). Sixteen (61.5%) patients were reported to have myoclonus. Levetiracetam was the most commonly used initial medication, with the majority (73%) of patients treated achieving partial or complete seizure control. The DD+S patients tended to have a similar burden of new-onset neuropsychiatric symptoms compared to the DD group. New-onset epilepsy seems to occur early in the course of dementia in DS patients. Patients generally respond to treatment. A great burden of neuropsychiatric symptoms is seen. Future studies need to explore the relationship between β-amyloid accumulation and epileptiform activity and attend to the care and needs of DS patients with dementia and seizures. Copyright © 2016 Elsevier Inc. All rights reserved.

  15. [A case of subacute parkinsonism presenting as bilateral basal ganglia legions by MRI in diabetic uremic syndrome].

    PubMed

    Nishimura, Yoshiko; Shibata, Koichi; Funaki, Takenori; Ito, Hiroyuki; Ito, Eiichi; Otsuka, Kuniaki

    2013-01-01

    A 60-year-old male was admitted because he had developed tremulous movement in both upper and lower limbs and gait disturbance over the course of 3 months. He had been on continuous ambulatory peritoneal dialysis almost 1 year earlier due to end-stage diabetic nephropathy. A neurological examination revealed a mild disturbance of his consciousness, asterixis in the upper limbs, bilateral extensor plantar responses and parkinsonism, which were characterized by bradykinesia, akinesia, rigidity, and bilaterally tremors at rest. Cranial magnetic resonance imaging (MRI) revealed swollen bilateral basal ganglia legions, which appeared hyperintense on T2-weighted images. The patient was treated for metabolic acidosis and continued hemodialysis three times a week; however, the parkinsonism remained 1 year later. Follow-up MRI revealed decreased swelling of the basal ganglia, and the pattern of diffusion-weighted images and the apparent diffusion coefficient (ADC) map indicated vasogenic and cytotoxic edema in bilateral globus pallidus. The case was diagnosed as encephalopathy due to diabetic uremic syndrome, initially characterized by Wang et al. (2003). Only 17 cases with parkinsonism have been reported. Diabetic uremic syndrome is characterized by acute or subacute onset consciousness disturbance and movement disorders such as parkinsonism, chorea and the other extrapyramidal signs to various degrees related to bilateral lesions of the basal ganglia.

  16. Rotigotine is safe and efficacious in Atypical Parkinsonism Syndromes induced by both α-synucleinopathy and tauopathy

    PubMed Central

    Moretti, Davide Vito; Binetti, Giuliano; Zanetti, Orazio; Frisoni, Giovanni Battista

    2014-01-01

    Transdermal rotigotine (RTG) is a non-ergot dopamine agonist (D3>D2>D1), and is indicated for use in early and advanced Parkinson’s disease (PD). RTG patch has many potential advantages due to the immediacy of onset of the therapeutic effect. Of note, intestinal absorption is not necessary and drug delivery is constant, thereby avoiding drug peaks and helping patient compliance. In turn, transdermal RTG seems a suitable candidate in the treatment of atypical Parkinsonian disorders (APS). Fifty-one subjects with a diagnosis of APS were treated with transdermal RTG. The diagnoses were: Parkinson’s disease with dementia, multiple system atrophy Parkinsonian type, multiple system atrophy cerebellar type, progressive supranuclear palsy, corticobasal degeneration, Lewy body dementia, and frontotemporal dementia with Parkinsonism. Patients were evaluated by the Unified Parkinson’s Disease Rating Scale (UPDRS; part III), Neuropsychiatric Inventory (NPI), and mini–mental state examination (MMSE) and all adverse events (AEs) were recorded. Patients treated with RTG showed an overall decrease of UPDRS III scores without increasing behavioral disturbances. Main AEs were hypotension, nausea, vomiting, drowsiness, tachycardia, and dystonia. On the whole, 15 patients were affected by AEs and seven patients suspended RTG treatment due to AEs. The results show that transdermal RTG is effective with a good tolerability profile. RTG patch could be a good therapeutic tool in patients with APS. PMID:24940065

  17. α-Synuclein and anti-α-synuclein antibodies in Parkinson's disease, atypical Parkinson syndromes, REM sleep behavior disorder, and healthy controls.

    PubMed

    Smith, Lynnae M; Schiess, Mya C; Coffey, Mary P; Klaver, Andrea C; Loeffler, David A

    2012-01-01

    α-synuclein is thought to play a key role in Parkinson's disease (PD) because it is the major protein in Lewy bodies, and because its gene mutations, duplication, and triplication are associated with early-onset PD. There are conflicting reports as to whether serum and plasma concentrations of α-synuclein and anti-α-synuclein antibodies differ between PD and control subjects. The objectives of this study were to compare the levels of α-synuclein and its antibodies between individuals with typical PD (n=14), atypical Parkinson syndromes (n=11), idiopathic rapid eye movement sleep behavior disorder (n=10), and healthy controls (n=9), to assess the strength of association between these serum proteins, and to determine group sizes needed for a high probability (80% power) of detecting statistical significance for 25% or 50% differences between typical PD and control subjects for these measurements. Analysis of log-transformed data found no statistically significant differences between groups for either α-synuclein or its antibodies. The concentrations of these proteins were weakly correlated (Spearman rho=0.16). In subjects with typical PD and atypical Parkinson syndromes, anti-α-synuclein antibody levels above 1.5 µg/ml were detected only in subjects with no more than four years of clinical disease. Power analysis indicated that 236 and 73 samples per group would be required for an 80% probability that 25% and 50% differences, respectively, in mean α-synuclein levels between typical PD and control subjects would be statistically significant; for anti-α-synuclein antibodies, 283 and 87 samples per group would be required. Our findings are consistent with those previous studies which suggested that serum concentrations of α-synuclein and its antibodies are not significantly altered in PD.

  18. Association between nocturnal/supine hypertension and restless legs syndrome in patients with Parkinson's disease.

    PubMed

    Oh, Yoon-Sang; Kim, Joong-Seok; Park, In-Seok; Song, In-Uk; Son, Young-Min; Park, Jeong-Wook; Yang, Dong-Won; Kim, Hee-Tae; Lee, Kwang-Soo

    2014-09-15

    Autonomic disturbances and sleep problems are common non-motor symptoms in patients with Parkinson's disease (PD). Orthostatic hypotension, supine hypertension (SH), and nocturnal hypertension (NH) are inter-related in patients with PD. These abnormalities might be associated with restless legs syndrome (RLS), which occurs predominantly at rest or during sleep. Few reports have suggested an association between circadian blood pressure disturbances and RLS in the general population. We evaluated the relationship between neurocardiovascular blood pressure alterations and RLS in patients with early PD. A total of 225 patients, newly diagnosed with PD, were included in the study. RLS was diagnosed by the International Restless Legs Syndrome Study Group's diagnostic criteria. Orthostatic vital signs and ambulatory 24-h blood pressure were monitored and recorded. Thirty-six (16.0%) participating patients had RLS. SH and NH were more frequent in the PD+RLS group than in the group without RLS. Supine blood pressure, orthostatic decline in blood pressure, nighttime blood pressure, and the standard deviation of systolic blood pressure were significantly higher in the PD+RLS group than in the group without RLS. RLS is related to nocturnal/supine hypertension and blood pressure fluctuations, suggesting a neuropathological association between autonomic and sleep dysfunctions in patients with PD. RLS may be a determinant of neurocirculatory abnormalities. Detecting and effectively treating RLS might slow the rate of pressure-related neurocardiovascular damage in dysautonomic patients with PD. Copyright © 2014 Elsevier B.V. All rights reserved.

  19. [Prognosis of Wolff-Parkinson-White syndrome in infants. Apropos of 31 cases].

    PubMed

    Becquart, J; Vaksmann, G; Becquart, V; Dupuis, C

    1988-05-01

    The fate of 31 children (18 boys, 13 girls) whose Wolff-Parkinson-White syndrome (WPW) had been diagnosed before they were 2 years' old (mean 3.4 months) was investigated. The circumstances in which WPW was discovered were: evaluation of a heart disease in 9 cases, attack of orthodromic tachycardia in 16 cases (including one with cardiopathy), and routine electrocardiography in 6 cases. Type A WPW was the most frequent, being found in 20 patients of whom only 3 had a heart disease; type B WPW was present in 11 patients, and 7 of these had a heart disease. Mean follow-up was 5.9 years; 3 children died of other causes than WPW. In patients followed up for more than one year WPW disappeared in 65 p. 100 of the cases (11/17) in the absence of cardiopathy, and in only 14 p. 100 of the cases (1/7) in the presence of cardiopathy. In children who had suffered attacks of tachycardia WPW disappeared in 64 p. 100 of the cases (9/14). When WPW disappeared it was before the age of 1 year in 8 out of 12 cases. Only one child whose WPW had disappeared had further attacks of tachycardia (11 p. 100), while 3 children whose WPW persisted had short and widely spaced attacks (60 p. 100). This study confirms the high rate of spontaneous disappearance of WPW and the excellent prognosis of this syndrome in the absence of heart disease.

  20. Noninvasive Localization of Accessory Pathways in Wolff-Parkinson-White Syndrome by Three-Dimensional Speckle Tracking Echocardiography.

    PubMed

    Ishizu, Tomoko; Seo, Yoshihiro; Igarashi, Miyako; Sekiguchi, Yukio; Machino-Ohtsuka, Tomoko; Ogawa, Kojiro; Kuroki, Kenji; Yamamoto, Masahiro; Nogami, Akihiko; Kawakami, Yasushi; Aonuma, Kazutaka

    2016-06-01

    We have developed a noninvasive isochrone activation imaging (AI) system with 3-dimensional (3D) speckle tracking echocardiography (STE), which allows visualization of the wavefront image of mechanical propagation of the accessory pathway (ACP) in Wolff-Parkinson-White syndrome. Patients with manifest Wolff-Parkinson-White syndrome were imaged in 3D-STE AI mode, which quantified the time from QRS onset to regional endocardial deformation. In 2 patients with left- and right-side ACP, we confirmed that intraoperative contact endocardial electric mapping and the 3D-STE AI system showed comparable images pre- and postablation. In normal heart assessment by 3D-echo AI, the earliest activation sites were found at the attachment of the papillary muscles in the left ventricle and midseptum in the right ventricle, and none showed earliest activation at the peri-atrioventricular valve annuli. An analyzer who was unaware of the clinical information assessed 39 ACP locations in 38 Wolff-Parkinson-White syndrome patients using 3D-STE. All showed abnormal perimitral or tricuspid annular activations, and the location of 34 ACP (87%) showed agreement with the successful ablation sites within a 2-o'clock range. Especially for left free wall ACP, 17/18 (94%) showed consistency with the ablation site within a 2 o'clock range. Among 15 ACP at the ventricular septum, 9 (60%) showed early local activation in both right and left sides of the septum. Isochrone AI with 3D-STE may be a promising noninvasive imaging tool to assess cardiac synchronized activation in normal hearts and detect abnormal breakthrough of mechanical activation from both atrioventricular annuli in Wolff-Parkinson-White syndrome. © 2016 American Heart Association, Inc.

  1. The thrill of reckless driving in patients with Parkinson's disease: an additional behavioural phenomenon in dopamine dysregulation syndrome?

    PubMed

    Avanzi, Maurizio; Baratti, Mario; Cabrini, Silvia; Uber, Elena; Brighetti, Gianni; Bonfà, Flavio

    2008-01-01

    We report two male patients with Parkinson's disease who developed compulsive risk-seeking driving behaviour as a result of self-administering high doses of L-dopa despite an adequate therapeutic response at lower doses. When L-dopa reduction was feasible, it resulted in cessation of unsafe driving. We believe that this impairment in driving performance, due to deliberate overuse of dopaminergic medication, should be included as a new behavioural phenomenon in dopamine dysregulation syndrome.

  2. Language, Executive Function and Social Cognition in the Diagnosis of Frontotemporal Dementia Syndromes

    PubMed Central

    Harciarek, Michał; Cosentino, Stephanie

    2015-01-01

    Frontotemporal dementia (FTD) represents a spectrum of non-Alzheimer’s degenerative conditions associated with focal atrophy of the frontal and/or temporal lobes. Frontal and temporal regions of the brain have been shown to be strongly involved in executive function, social cognition and language processing and, thus, deficits in these domains are frequently seen in patients with FTD or may even be hallmarks of a specific FTD subtype ( i.e., relatively selective and progressive language impairment in primary progressive aphasia). In this review, we have attempted to delineate how language, executive function, and social cognition may contribute to the diagnosis of FTD syndromes, namely the behavioral variant FTD as well as the language variants of FTD including the three subtypes of primary progressive aphasia (PPA): non-fluent/agrammatic, semantic, and logopenic. This review also addresses the extent to which deficits in these cognitive areas contribute to the differential diagnosis of FTD versus AD. Finally, early clinical determinants of pathology are briefly discussed and contemporary challenges to the diagnosis of FTD are presented. PMID:23611348

  3. Fully automated structural MRI of the brain in clinical dementia workup.

    PubMed

    Persson, Karin; Selbæk, Geir; Brækhus, Anne; Beyer, Mona; Barca, Maria; Engedal, Knut

    2017-06-01

    Background The dementia syndrome has been regarded a clinical diagnosis but the focus on supplemental biomarkers is increasing. An automatic magnetic resonance imaging (MRI) volumetry method, NeuroQuant® (NQ), has been developed for use in clinical settings. Purpose To evaluate the clinical usefulness of NQ in distinguishing Alzheimer's disease dementia (AD) from non-dementia and non-AD dementia. Material and Methods NQ was performed in 275 patients diagnosed according to the criteria of ICD-10 for AD, vascular dementia and Parkinson's disease dementia (PDD); the Winblad criteria for mild cognitive impairment; the Lund-Manchester criteria for frontotemporal dementia; and the revised consensus criteria for Lewy body dementia (LBD). Receiver operating curve (ROC) analyses with calculation of area under the curve (AUC) and regression analyses were carried out. Results Forebrain parenchyma (AUC 0.82), hippocampus (AUC 0.80), and inferior lateral ventricles (AUC 0.78) yielded the highest AUCs for AD/non-dementia discrimination. Only hippocampus (AUC 0.62) and cerebellum (AUC 0.67) separated AD from non-AD dementia. Cerebellum separated AD from PDD-LBD (AUC 0.83). Separate multiple regression analyses adjusted for age and gender, showed that memory (CERAD 10-word delayed recall) (beta 0.502, P < 0.001) was more strongly associated to the hippocampus volume than the diagnostic distinction of AD versus non-dementia (beta -0.392, P < 0.001). Conclusion NQ measures could separate AD from non-dementia fairly well but generally poorer from non-AD dementia. Degree of memory impairment, age, and gender, but not diagnostic distinction, were associated to the hippocampus volume in adjusted analyses. Surprisingly, cerebellum was found relevant in separating AD from PDD-LBD.

  4. Parkinson's disease.

    PubMed Central

    Playfer, J. R.

    1997-01-01

    Parkinson's disease is a common disabling disease of old age. The diagnosis of idiopathic Parkinson's disease is based on clinical signs and has poor sensitivity, with about 25% of patients confidently diagnosed as having the disease actually having other conditions such as multi-system atrophy and other parkinsonism-plus syndromes. Benign essential tremor and arteriosclerotic pseudo-parkinsonism can easily be confused with Parkinson's disease. The cause of Parkinson's disease remains unknown. Speculative research highlights the role of oxidative stress and free radical mediated damage to dopaminergic cells. Parkinson's disease is the one neurodegenerative disorder in which drugs have been demonstrated to be of value. There is now a wide variety of drugs and formulations available, including anticholinergics, amantidine, L-dopa, dopamine agonists including apomorphine, selegiline and soon to be available catechol-O-methyltransferase inhibitors. Disabling side-effects of treatment, fluctuations, dyskinesias and psychiatric problems require strategic use of the drugs available. There is an increasing potential for neurosurgical intervention. PMID:9196696

  5. Spontaneous Transition of Double Tachycardias with Atrial Fusion in a Patient with Wolff-Parkinson-White Syndrome

    PubMed Central

    Kim, Dongmin

    2016-01-01

    Among patients with Wolff-Parkinson-White syndrome, atrioventricular reciprocating tachycardia (AVRT) and atrioventricular nodal reentrant tachycardia (AVNRT) can coexist in a single patient. Direct transition of both tachycardias is rare; however, it can occur after premature atrial or ventricular activity if the cycle lengths of the two tachycardias are similar. Furthermore, persistent atrial activation by an accessory pathway (AP) located outside of the AV node during ongoing AVNRT is also rare. This article describes a case of uncommon atrial activation by an AP during AVNRT and gradual transition of the two supraventricular tachycardias without any preceding atrial or ventricular activity in a patient with preexcitation syndrome. PMID:27482269

  6. Treatment of Frontotemporal Dementia

    PubMed Central

    Boxer, Adam L.

    2016-01-01

    Opinion statement Frontotemporal dementia (FTD) encompasses a spectrum of neurodegenerative diseases with heterogeneous clinical presentations and two predominant types of underlying neuropathology. FTD typically comprises three distinct clinical syndromes: behavioral variant frontotemporal dementia (bvFTD), semantic variant primary progressive aphasia (svPPA), and nonfluent variant primary progressive aphasia (nfvPPA). FTD also frequently overlaps both clinically and neuropathologically with three other neurodegenerative syndromes: corticobasal syndrome (CBS), progressive supranuclear palsy (PSP), and amyotrophic lateral sclerosis (ALS). Each syndrome can be associated with one or more underlying neuropathological diagnoses and are referred to as frontotemporal lobar degeneration (FTLD). Although the various FTD syndromes can substantially differ in terms of clinical symptoms and underlying pathology, the symptoms can be broadly categorized into behavioral, cognitive and motor domains. Currently there are no Food and Drug Administration (FDA) approved therapies for the above syndromes except riluzole for ALS. FTD treatment strategies generally rely on off-label use of medications for symptomatic management, and most therapies lack quality evidence from randomized, placebo-controlled clinical trials. For behavioral symptoms, selective serotonin reuptake inhibitors may be effective, while case reports hint at possible efficacy with antipsychotics or antiepileptics, but use of these latter agents is limited due to concerns regarding side effects. There are no effective therapies for cognitive complaints in FTD, which frequently involve executive function, memory, and language. Motor difficulties associated with FTD may present with parkinsonian symptoms or motor neuron disease, for which riluzole is indicated as therapy. Compared to idiopathic Parkinson’s disease, FTD-related atypical parkinsonism is generally not responsive to dopamine replacement therapies, but a

  7. A Four-Year Longitudinal Study on Restless Legs Syndrome in Parkinson Disease

    PubMed Central

    Moccia, Marcello; Erro, Roberto; Picillo, Marina; Santangelo, Gabriella; Spina, Emanuele; Allocca, Roberto; Longo, Katia; Amboni, Marianna; Palladino, Raffaele; Assante, Roberta; Pappatà, Sabina; Pellecchia, Maria Teresa; Barone, Paolo; Vitale, Carmine

    2016-01-01

    Study Objectives: Restless legs syndrome (RLS) prevalence estimates range from 0% to 52% in Parkinson disease (PD), but the causal relationship between the two disorders is still debated. The present study aims to evaluate RLS prevalence in de novo PD subjects, its incidence during the first 4 years from diagnosis, and possible relationships with clinical, laboratory, and neuroradiological data. Methods: One hundred nine newly diagnosed, drug-naïve PD subjects were evaluated at the time of PD diagnosis, and after 2- and 4-years. RLS diagnosis was performed with the RLS Diagnostic Index at each visit. Motor features, additional non-motor symptoms (NMS), and concomitant dopaminergic and nondopaminergic treatments were also gathered. Moreover, at baseline, 65 subjects were randomly selected to undergo a FP-CIT SPECT to study dopamine transporter availability. Results: RLS prevalence rose from 4.6% at baseline evaluation to 6.5% after 2 years and to 16.3% after 4 years (P = 0.007). A multinomial logistic stepwise regression model selected NMS Questionnaire items more likely to be associated with RLS at diagnosis (insomnia, OR = 15.555; P = 0.040) and with occurrence of RLS during follow-up (dizziness, OR = 1.153; P = 0.022; and daytime sleepiness; OR = 9.557; P = 0.001), as compared to patients without RLS. Older age was more likely associated to increased RLS occurrence during follow-up in a random effect logistic regression model (OR = 1.187; P = 0.036). A multinomial logistic stepwise model found increased dopaminergic transporter availability of affected caudate and putamen to be more likely associated with RLS presence at diagnosis (n = 5; OR = 75.711; P = 0.077), and RLS occurrence during follow-up (n = 16; OR = 12.004; P = 0.059), respectively, as compared to patients without RLS (n = 88). Conclusions: RLS is present since PD diagnosis, and increases in prevalence during the course of PD. PD subjects with RLS have higher age at PD onset, more preserved

  8. Association Between Early-Onset Parkinson Disease and 22q11.2 Deletion Syndrome

    PubMed Central

    Butcher, Nancy J.; Kiehl, Tim-Rasmus; Hazrati, Lili-Naz; Chow, Eva W. C.; Rogaeva, Ekaterina; Lang, Anthony E.; Bassett, Anne S.

    2015-01-01

    IMPORTANCE Clinical case reports of parkinsonism co-occurring with hemizygous 22q11.2 deletions and the associated multisystem syndrome, 22q11.2 deletion syndrome (22q11.2DS), suggest that 22q11.2 deletions may lead to increased risk of early-onset Parkinson disease (PD). The frequency of PD and its neuropathological presentation remain unknown in this common genetic condition. OBJECTIVE To evaluate a possible association between 22q11.2 deletions and PD. DESIGN, SETTING, AND PARTICIPANTS An observational study of the occurrence of PD in the world’s largest cohort of well-characterized adults with a molecularly confirmed diagnosis of 22q11.2DS (n = 159 [6 with postmortem tissue]; age range, 18.1–68.6 years) was conducted in Toronto, Ontario, Canada. Rare postmortem brain tissue from individuals with 22q11.2DS and a clinical history of PD was investigated for neurodegenerative changes and compared with that from individuals with no history of a movement disorder. MAIN OUTCOMES AND MEASURES A clinical diagnosis of PD made by a neurologist and neuropathological features of PD. RESULTS Adults with 22q11.2DS had a significantly elevated occurrence of PD compared with standard population estimates (standardized morbidity ratio = 69.7; 95% CI, 19.0–178.5). All cases showed early onset and typical PD symptom pattern, treatment response, and course. All were negative for family history of PD and known pathogenic PD-related mutations. The common use of antipsychotics in patients with 22q11.2DS to manage associated psychiatric symptoms delayed diagnosis of PD by up to 10 years. Postmortem brain tissue revealed classic loss of midbrain dopaminergic neurons in all 3 postmortem 22q11.2DS-PD cases. Typical α-synuclein–positive Lewy bodies were present in the expected distribution in 2 cases but absent in another. CONCLUSIONS AND RELEVANCE These findings suggest that 22q11.2 deletions represent a novel genetic risk factor for early-onset PD with variable neuropathological

  9. Variability in the clinical expression of Parkinson's disease.

    PubMed

    Wolters, Erik Ch

    2008-03-15

    Parkinsonism is a clinical syndrome characterized by bradykinesia, hypo-/akinesia, muscular rigidity, and resting tremor, mainly caused by Parkinson's disease (PD). Symptoms of PD are due to a progressive loss of nigral neurons causing striatal dopaminergic denervation. However, nigral degeneration is only a part of the underlying synucleinopathy, and clinical symptoms go far beyond motor parkinsonism. Olfactory disturbances, fatigue, pain, autonomic dysfunction, sleep fragmentation, depression, and dementia with or without psychosis are frequently seen. The variability in the expression of these signs and symptoms, as discussed in this paper, might be explained by the specific topographical sequence of the pathology, depending on the extent and progression of the degenerative process at defined sites. Better insight in the clinicopathological correlations of this disease may help to further develop early diagnosis and adequate therapeutic strategies.

  10. Brain Insulin-Like Growth Factor and Neurotrophin Resistance in Parkinson's Disease and Dementia with Lewy Bodies: Potential Role of Manganese Neurotoxicity

    PubMed Central

    Tong, Ming; Dong, Matthew; de la Monte, Suzanne M.

    2010-01-01

    Parkinson's disease (PD) and dementia with Lewy bodies (DLB) frequently overlap with Alzheimer's disease, which is linked to brain impairments in insulin, insulin-like growth factor (IGF), and neurotrophin signaling. We explored whether similar abnormalities occur in PD or DLB, and examined the role of manganese toxicity in PD/DLB pathogenesis. Quantitative RT-PCR demonstrated reduced expression of insulin, IGF-II, and insulin, IGF-I, and IGF-II receptors (R) in PD and/or DLB frontal white matter and amygdala, and reduced IGF-IR and IGF-IIR mRNA in DLB frontal cortex. IGF-I and IGF-II resistance was present in DLB but not PD frontal cortex, and associated with reduced expression of Hu, nerve growth factor, and Trk neurotrophin receptors, and increased levels of glial fibrillary acidic protein, α-synuclein, dopamine-β-hydroxylase, 4-hydroxy-2-nonenal (HNE), and ubiquitin immunoreactivity. MnCl2 treatment reduced survival, ATP, and insulin, IGF-I and IGF-II receptor expression, and increased α-synuclein, HNE, and ubiquitin immunoreactivity in cultured neurons. The results suggest that: 1) IGF-I, IGF-II, and neurotrophin signaling are more impaired in DLB than PD, corresponding with DLB's more pronounced neurodegeneration, oxidative stress, and α-synuclein accumulation; 2) MnCl2 exposure causes PD/DLB associated abnormalities in central nervous system neurons, and therefore may contribute to their molecular pathogenesis; and 3) molecular abnormalities in PD/DLB overlap with but are distinguishable from Alzheimer's disease. PMID:19276553

  11. Sixth nerve palsy + ipsilateral Horner's Syndrome = Parkinson's Syndrome.

    PubMed

    Ebner, Roberto N; Ayerza, Dolores Ribero; Aghetoni, Fernando

    2015-01-01

    To present five patients with VIth nerve palsy and ipsilateral Horner's Syndrome (HS), as a result of cavernous sinus alteration. Consecutive case series. Five patients presented abducens palsy with horizontal diplopia (3 in primary position and 2 in lateral gaze only) and ipsilateral HS. Apraclonidine 0.5% drops evidenced sympathetic denervation in all patients 40-60 min after instillation. All 5 cases had neuroimages (MRI in 3 cases, Computerized Tomography - CT in one case and Magnetic Resonance Angiography - MRA in one case) demonstrating cavernous sinus lesions; 2 meningiomas, 1 carotid-cavernous aneurism, 1 foreign body (bullet) and 1 squamous cell carcinoma. Lesions on the cavernous sinus need to be considered in cases of abducens nerve palsy and ipsilateral Horner's Syndrome.

  12. Study Looks At Parkinson's Effect on Life Span

    MedlinePlus

    ... gov/news/fullstory_165589.html Study Looks at Parkinson's Effect on Life Span 2-year difference seen ... HealthDay News) -- People with brain diseases such as Parkinson's and dementia with Lewy bodies die about two ...

  13. Frontal lobe dementia syndrome as a first manifestation of primary angiitis of the central nervous system (PACNS).

    PubMed

    Bönstrup, Marlene; Ott, Katja; Glatzel, Markus; Magnus, Tim

    2016-02-01

    This case presents a clinical course of a frontal lobe dysexecutive syndrome with dementia caused by a primary angiitis of the central nervous system (PACNS) of exclusively very small vessels. An isolated frontal lobe dementia syndrome as a primary manifestation of PACNS highlights the diverse clinical manifestations of the disease. The patient presented with a progressive cognitive decline with loss of memory, disinhibited behavior, inappropriate affect and frontal release signs. The diagnostic workup essentially revealed a lymphocytic pleocytosis in the cerebrospinal fluid and a generalized cortical atrophy without any vascular abnormalities. To grasp a diagnosis for this enigmatic clinical picture of a frontal lobe syndrome with signs of inflammation we targeted a tissue-based diagnosis. A brain biopsy gave the decisive hint towards a microvasculitis. Although the histopathologic picture showed peculiarities, a destruction of the vascular bed of very small vessels by lymphocytic infiltration was evident. Our case illustrates an uncommon clinical picture of a PACNS and points to shortcomings of the current histopathologic criteria if only very small vessels are involved. Copyright © 2016 Elsevier B.V. All rights reserved.

  14. Nature and extent of person recognition impairments associated with Capgras syndrome in Lewy body dementia

    PubMed Central

    Fiacconi, Chris M.; Barkley, Victoria; Finger, Elizabeth C.; Carson, Nicole; Duke, Devin; Rosenbaum, R. Shayna; Gilboa, Asaf; Köhler, Stefan

    2014-01-01

    Patients with Capgras syndrome (CS) adopt the delusional belief that persons well-known to them have been replaced by an imposter. Several current theoretical models of CS attribute such misidentification problems to deficits in covert recognition processes related to the generation of appropriate affective autonomic signals. These models assume intact overt recognition processes for the imposter and, more broadly, for other individuals. As such, it has been suggested that CS could reflect the “mirror-image” of prosopagnosia. The purpose of the current study was to determine whether overt person recognition abilities are indeed always spared in CS. Furthermore, we examined whether CS might be associated with any impairments in overt affective judgments of facial expressions. We pursued these goals by studying a patient with Dementia with Lewy bodies (DLB) who showed clear signs of CS, and by comparing him to another patient with DLB who did not experience CS, as well as to a group of healthy control participants. Clinical magnetic resonance imaging scans revealed medial prefrontal cortex (mPFC) atrophy that appeared to be uniquely associated with the presence CS. We assessed overt person recognition with three fame recognition tasks, using faces, voices, and names as cues. We also included measures of confidence and probed pertinent semantic knowledge. In addition, participants rated the intensity of fearful facial expressions. We found that CS was associated with overt person recognition deficits when probed with faces and voices, but not with names. Critically, these deficits were not present in the DLB patient without CS. In addition, CS was associated with impairments in overt judgments of affect intensity. Taken together, our findings cast doubt on the traditional view that CS is the mirror-image of prosopagnosia and that it spares overt recognition abilities. These findings can still be accommodated by models of CS that emphasize deficits in autonomic

  15. Oral glycotoxins are a modifiable cause of dementia and the metabolic syndrome in mice and humans.

    PubMed

    Cai, Weijing; Uribarri, Jaime; Zhu, Li; Chen, Xue; Swamy, Shobha; Zhao, Zhengshan; Grosjean, Fabrizio; Simonaro, Calogera; Kuchel, George A; Schnaider-Beeri, Michal; Woodward, Mark; Striker, Gary E; Vlassara, Helen

    2014-04-01

    Age-associated dementia and Alzheimer's disease (AD) are currently epidemic. Neither their cause nor connection to the metabolic syndrome (MS) is clear. Suppression of deacetylase survival factor sirtuin 1 (SIRT1), a key host defense, is a central feature of AD. Age-related MS and diabetes are also causally associated with suppressed SIRT1 partly due to oxidant glycotoxins [advanced glycation end products (AGEs)]. Changes in the modern diet include excessive nutrient-bound AGEs, such as neurotoxic methyl-glyoxal derivatives (MG). To determine whether dietary AGEs promote AD, we evaluated WT mice pair-fed three diets throughout life: low-AGE (MG(-)), MG-supplemented low-AGE (MG(+)), and regular (Reg) chow. Older MG(+)-fed mice, similar to old Reg controls, developed MS, increased brain amyloid-β42, deposits of AGEs, gliosis, and cognitive deficits, accompanied by suppressed SIRT1, nicotinamide phosphoribosyltransferase, AGE receptor 1, and PPARγ. These changes were not due to aging or caloric intake, as neither these changes nor the MS were present in age-matched, pair-fed MG(-) mice. The mouse data were enhanced by significant temporal correlations between high circulating AGEs and impaired cognition, as well as insulin sensitivity in older humans, in whom dietary and serum MG levels strongly and inversely associated with SIRT1 gene expression. The data identify a specific AGE (MG) as a modifiable risk factor for AD and MS, possibly acting via suppressed SIRT1 and other host defenses, to promote chronic oxidant stress and inflammation. Because SIRT1 deficiency in humans is both preventable and reversible by AGE reduction, a therapeutic strategy that includes AGE reduction may offer a new strategy to combat the epidemics of AD and MS.

  16. Impaired olfaction and other prodromal features in the Parkinson At-Risk Syndrome Study.

    PubMed

    Siderowf, Andrew; Jennings, Danna; Eberly, Shirley; Oakes, David; Hawkins, Keith A; Ascherio, Albert; Stern, Matthew B; Marek, Kenneth

    2012-03-01

    To test the association between impaired olfaction and other prodromal features of PD in the Parkinson At-Risk Syndrome Study. The onset of olfactory dysfunction in PD typically precedes motor features, suggesting that olfactory testing could be used as a screening test. A combined strategy that uses other prodromal nonmotor features, along with olfactory testing, may be more efficient than hyposmia alone for detecting the risk of PD. Individuals with no neurological diagnosis completed a mail survey, including the 40-item University of Pennsylvania Smell Identification Test, and questions on prodromal features of PD. The frequency of reported nonmotor features was compared across individuals with and without hyposmia. A total of 4,999 subjects completed and returned the survey and smell test. Of these, 669 were at or below the 15th percentile based on age and gender, indicating hyposmia. Hyposmics were significantly more likely to endorse nonmotor features, including anxiety and depression, constipation, and rapid eye movement sleep behavior disorder symptoms, and to report changes in motor function. Twenty-six percent of subjects with combinations of four or more nonmotor features were hyposmic, compared to 12% for those reporting three or fewer nonmotor features (P < 0.0001). Hyposmia is associated with other nonmotor features of PD in undiagnosed individuals. Further assessment of hyposmic subjects using more specific markers for degeneration, such as dopamine transporter imaging, will evaluate whether combining hyposmia and other nonmotor features is useful in assessing the risk of future neurodegeneration. Copyright © 2012 Movement Disorder Society.

  17. Restless legs syndrome in Parkinson disease: Clinical characteristics, abnormal iron metabolism and altered neurotransmitters.

    PubMed

    Piao, Ying-Shan; Lian, Teng-Hong; Hu, Yang; Zuo, Li-Jun; Guo, Peng; Yu, Shu-Yang; Liu, Li; Jin, Zhao; Zhao, Hui; Li, Li-Xia; Yu, Qiu-Jin; Wang, Rui-Dan; Chen, Sheng-Di; Chan, Piu; Wang, Xiao-Min; Zhang, Wei

    2017-09-05

    Relationships among clinical characteristics, iron metabolism and neurotransmitters in Parkinson disease (PD) patients with restless legs syndrome (RLS) remains unclear. We divided 218 patients into PD with and with no RLS (PD-RLS and PD-NRLS) groups by RLS-rating scale (RLS-RS) score. Motor and non-motor symptoms were rated by related scales. Iron and related proteins, and neurotransmitters in cerebrospinal fluid (CSF) and serum were measured. PD-RLS frequency was 40.37%. PD-RLS group had longer duration, higher stage and scores of motor symptoms, depression, anxiety, sleep disorders, fatigue and apathy, and increased transferrin and decreased iron, ferritin, dopamine (DA) and 5-hydroxytryptamine (5-HT) in CSF. In CSF of PD-RLS group, RLS-RS score was positively correlated with transferrin level and negatively correlated with iron and ferritin levels; RLS-RS score was negatively correlated with DA and 5-HT levels; transferrin level was negatively correlated with DA and 5-HT levels, and ferritin level was positively correlated with DA level. In serum, PD-RLS group had decreased iron and transferrin levels, which were negatively correlated with RLS-RS score. PD-RLS was common and severer in motor and some non-motor symptoms. Iron deficiency induced by its metabolism dysfunctions in peripheral and central systems might cause PD-RLS through decreasing brain DA and 5-HT.

  18. Does idiopathic restless legs syndrome delay onset and reduce severity of Parkinson's disease: a pilot study.

    PubMed

    Dragan, Elizabeth M; Chen, Zhongxue; Ondo, William G

    2015-01-01

    Parkinson's disease (PD) populations show that about 20% of patients meet criteria for restless legs syndrome (RLS). Nevertheless, pathological differences suggest RLS may actually prevent PD. Over 15 years, we collected patients with idiopathic RLS preceding onset of PD, as defined by onset of RLS greater than 5 years before motor symptoms of PD, or a family history of RLS and onset of RLS at least 1 year before PD. We then compared these to a control group of PD without RLS for demographics, age of onset, motor progression, dyskinesia, L-dopa equivalent dose, and PD phenotype at onset. The RLS/PD group (N = 36) had 13 females with 18 having a positive family history of RLS and six with a family history of PD. The PD control group (N = 36) had 10 females with 1 having a family history of RLS and nine with family history of PD. Age at motor onset of PD in the RLS/PD was older (64.15 ± 6.44 years vs. 56.81 ± 10.68) than for controls with PD (p < 0.001). After correcting for other risk factors and duration of follow-up, patients with idiopathic PD (21/36) developed dyskinesia more than RLS/PD (4/32) at final visit (p = 0.01). Our data suggest that idiopathic RLS may delay the onset of PD, reduce dyskinesia occurrence, and possibly reduce progression of PD.

  19. Existence of automaticity in anomalous bundle of Wolff-Parkinson-White syndrome.

    PubMed Central

    Przybylski, J; Chiale, P A; Halpern, M S; Lázzari, J O; Elizari, M V; Rosenbaum, M B

    1978-01-01

    Escape beats probably arising from the anomalous bundle were documented in 2 patients with the Wolff-Parkinson-White (WPW) syndrome. A third patient, in whom complete AV block developed both in the anomalous bundle and the normal pathway, showed the occurrence of escape beats (an escape-bigeminy pattern), as well as a regular idioventricular rhythm arising from the anomalous bundle. Phase 4 block in the anomalous bundle occurred in 7 other patients, in 4 of them spontaneously and in 3 only after the administration of ajmaline or amiodarone. Only 4 of 14 fully investigated patients (out of a total number of 23) showed absence of both escape beats and phase 4 block. The escape beats were considered as direct evidence, and the phase 4 block as indirect evidence, for the existence of automaticity in the anomalous bundle. Such evidence supports the view that the anomalous bundle, like the His bundle-branch system, may be composed of specialised tissue endowed with the property of automaticity. PMID:656241

  20. [The double ventricular response phenomenon in 2 cases of Wolff-Parkinson-White syndrome].

    PubMed

    Guize, L; Meilhac, B; Cabanis, C; Di Mattéo, J; Maurice, P

    1978-02-01

    The authors report two cases of "true" consecutive double ventricular response caused by a single premature atrial stimulation; both were young men with Wolff-Parkinson-White syndrome. In both cases, the presence of a bundle of Kent was confirmed. The phenomenon of double ventricular response arising successively from the bundle of Kent and node-His pathway is rare, being mentioned in only two cases in the literature. It is only found when there is the combination of a good bundle of Kent, fair forward conduction, and a relative ventricle-His retrograde block. Amongst the other mechanisms for double ventricular repsonse, re-entry from branch to branch presents the most difficult differential diagnosis. From our observations, the forward characteristics of the spread through the bundle of His which always procedes the bundle of His which always precedes the second ventricular complex have been confirmed, especially in view of the freat variation in the position of this potential which can easily be explained by variations in intra-nodal conduction. In one of these cases, the atriogram, taken after the second ventriculogram, was provided by retrograde activity in the bundle of Kent.

  1. Atrial pacing at multiple sites in the Wolff-Parkinson-White syndrome.

    PubMed Central

    Denes, P; Wyndham, C R; Amat-y-Leon, F; Wu, D; Dhingra, R C; Miller, R H; Rosen, K M

    1977-01-01

    Atrial pacing at multiple sites was used in an attempt to predict the site of pre-excitation in 5 patients with Wolff-Parkinson-White syndrome with 5 different anomalous pathway locations (right anterior, right posterior, septal, left posterior, and left lateral). At least 3 atrial pacing sites were tested in each patient. Pacing sites tested included high right atrium, low lateral right atrium, low septal right atrium, proximal coronary sinus, and distal coronary sinus. Atrial stimulation sites with shortest and longest stimulus-delta intervals could be identified in each patient, the shortest stimulus-delta interval in each case ranging from 60 to 80 ms. The difference between the shortest and longest stimulus-delta interval in each case ranged from 60 to 110 ms. It was suggested that the site with the shortest stimulus-delta interval corresponded to a site close to the atrial insertion of the anomalous pathway. This hypothesis was confirmed in all cases (3 with epicardial mapping and 2 with retrograde atrial activation data). In conclusion, atrial pacing at multiple sites is helpful in predicting the site of anterogradely conducting anomalous pathways, and appears particularly useful for differentiation of right posterior, left posterior, and septal pre-excitation. Images PMID:861093

  2. Up-regulation of E2F-1 in Down's syndrome brain exhibiting neuropathological features of Alzheimer-type dementia.

    PubMed

    Motonaga, K; Itoh, M; Hirayama, A; Hirano, S; Becker, L E; Goto, Y; Takashima, S

    2001-06-29

    We studied the expression of the apoptosis-related protein, E2F-1, in Down's syndrome (DS) brains. The immunoreactivity for E2F-1 was detected in the pyramidal neurons of the cerebral cortex from DS brains exhibiting the neuropathological features of dementia of Alzheimer type (DAT), in accordance with the amyloid beta protein (A beta) deposition in the neuron. Therefore, the implication is that A beta deposition may trigger E2F-1-mediated neuronal apoptosis in DS brains with DAT.

  3. Pathological tau burden and distribution distinguishes progressive supranuclear palsy-parkinsonism from Richardson's syndrome.

    PubMed

    Williams, David R; Holton, Janice L; Strand, Catherine; Pittman, Alan; de Silva, Rohan; Lees, Andrew J; Revesz, Tamas

    2007-06-01

    Clinical syndromes associated with progressive supranuclear palsy-tau pathology now include progressive supranuclear palsy-parkinsonism (PSP-P), in addition to classic Richardson's syndrome (RS) and pure akinesia with gait freezing (PAGF). Although pathological heterogeneity of progressive supranuclear palsy (PSP) has also been established, attempts to correlate this with clinical findings have only rarely provided conclusive results. The aim of this study was to investigate whether regional variations in the types of tau lesions or differences in overall tau load may explain the clinical differences between the RS, PSP-P and PAGF. Quantitative tau pathology assessment was performed in 17 brain regions in 42 cases of pathologically diagnosed PSP (22 RS, 14 PSP-P and 6 PAGF). Neurofibrillary tangles, tufted astrocytes, coiled bodies and thread pathology were quantitated and a grading system was developed separately for each region. Using these grades the overall tau load was calculated in each case. To establish a simplified system for grading the severity of tau pathology, all data were explored to identify the minimum number of regions that satisfactorily summarized the overall tau severity. The subthalamic nucleus, substantia nigra and globus pallidus were consistently the regions most severely affected by tau pathology. The mean severity in all regions of the RS group was higher than in PSP-P and PAGF, and the overall tau load was significantly higher in RS than in PSP-P (P = 0.002). Using only the grade of coiled body + thread lesions in the substantia nigra, caudate and dentate nucleus, a reliable and repeatable 12-tiered grading system was established (PSP-tau score: 0, mild tau pathology, restricted distribution; >7, severe, widespread tau pathology). PSP-tau score was negatively correlated with disease duration (Spearman's rho -0.36, P = 0.028) and time from disease onset to first fall (Spearman's rho -0.49, P = 0.003). The PSP-tau score in PSP-P (median 3

  4. Long-term Impact of Parental Well-Being on Adult Outcomes and Dementia Status in Individuals with Down Syndrome

    PubMed Central

    Esbensen, Anna J.; Mailick, Marsha R.; Silverman, Wayne

    2013-01-01

    Parental characteristics were significant predictors of health, functional abilities, and behavior problems in adults with Down syndrome (n = 75) over a 22-year time span, controlling for initial levels and earlier changes in these outcomes. Lower levels of behavior problems were predicted by improvements in maternal depressive symptoms. Higher levels of functional abilities were predicted by prior measures of and improvements in maternal depressive symptoms. Better health was predicted by prior measures of maternal depressive symptoms, paternal positive psychological well-being, relationship quality between fathers and their adult children, and improvements in maternal positive psychological well-being. Dementia status was also predicted by parental characteristics. The study suggests the importance of the family context for healthy aging in adults with Down syndrome. PMID:23937371

  5. [Neuropsychological characteristics of Parkinson's disease].

    PubMed

    Ostrosky-Solis, F

    Form part of the clinical symptoms of Parkinson's disease. These disorders may present in varying degrees: whilst in some patients a clinical picture of dementia is seen, in others there are only specific symptoms. In this article we consider three of the most controversial aspects currently dominating study of the neuropsychology of Parkinson's disease. The first relates to the pathophysiological basis and neurotransmitters involved. The second deals with the distinction between subcortical-type and Alzheimer-type dementia, and the third with the pathophysiological basis underlying the cognitive profile of the subgroups of patients with Parkinson's disease who do not present dementia. The relation between the factors causing the disease, neuropathology, individual variables and the presence of these subgroups requires precise systematic investigation of the neuropsychology shown by patients with Parkinson's disease.

  6. [Obvious or inapparent Wolff-Parkinson-White syndrome associated with duality of nodal conduction. Apropos of 4 cases].

    PubMed

    Motté, G; Belhassen, B; Bodereau, P

    1979-03-01

    In a series of 48 patients undergoing electrophysiological investigation for attacks of reciprocating tachycardia related to concealed or overt Wolff-Parkinson-White syndrome in sinus rhythm, 4 patients were found to have duality of nodal conduction. This association was responsible for several tachycardia circuits: in 2 patients the activation passed constantly retrogradely through the accessory pathway and then either through the slow nodal pathway or the rapid nodal pathway in the anterograde direction. In the other two patients, in addition to classical orthodromic tachycardia, purely intranodal reciprocating rhythms giving rise to sustained tachycardia in one case and to simple echos in the other, were observed.

  7. Ventricular fibrillation development following atrial fibrillation after the ingestion of sildenaphil in a patient with Wolff-Parkinson-White syndrome

    PubMed Central

    Inci, Sinan; Izgu, Ibrahim; Aktas, Halil; Dogan, Pinar; Dogan, Ali

    2015-01-01

    Summary Complications in the accessory pathway in Wolff-Parkinson-White (WPW) syndrome could cause different clinical conditions by inducing different arrhythmias. Atrial fibrillation (AF) is one of these arrhythmias and is important as it causes life-threatening arrhythmias. It is known that some drugs, underlying cardiac diseases, and the number of accessory pathways, cause a predisposition to this condition. In the current report, we presented a patient with WPW who was admitted to the emergency department with AF, wide QRS and a rapid ventricular response that progressed to ventricular fibrillation. PMID:26361569

  8. Reversible Valproate Induced Pisa Syndrome and Parkinsonism in a Neuro-Oncology Patient with Depression and Epilepsy

    PubMed Central

    Botturi, Andrea; Silvani, Antonio; Pravettoni, Gabriella; Paoli, Riccardo Augusto; Lucchiari, Claudio

    2016-01-01

    Neurological and psychiatric conditions frequently overlap in neuro-oncology. This overlapping negatively affects patients’ quality of life and decreases the ability of providers to manage specific symptoms by therapy modulation, especially when psychopharmacotherapy needs to be prescribed. We describe here a patient with recurrent brain tumor, symptomatic epilepsy and depression who developed Pisa syndrome and parkinsonism after several months of valproic acid use. An accurate recognition of symptoms and treatment side effect allowed an appropriate clinical approach so as to rapidly improve both movement disorder and depression without increasing the risk of developing seizure. This has improved the autonomy and quality of life in a patient with poor prognosis. PMID:27462241

  9. Reversible Valproate Induced Pisa Syndrome and Parkinsonism in a Neuro-Oncology Patient with Depression and Epilepsy.

    PubMed

    Botturi, Andrea; Silvani, Antonio; Pravettoni, Gabriella; Paoli, Riccardo Augusto; Lucchiari, Claudio

    2016-01-01

    Neurological and psychiatric conditions frequently overlap in neuro-oncology. This overlapping negatively affects patients' quality of life and decreases the ability of providers to manage specific symptoms by therapy modulation, especially when psychopharmacotherapy needs to be prescribed. We describe here a patient with recurrent brain tumor, symptomatic epilepsy and depression who developed Pisa syndrome and parkinsonism after several months of valproic acid use. An accurate recognition of symptoms and treatment side effect allowed an appropriate clinical approach so as to rapidly improve both movement disorder and depression without increasing the risk of developing seizure. This has improved the autonomy and quality of life in a patient with poor prognosis.

  10. Quantitative Proteomics Identifies Surfactant-Resistant α-Synuclein in Cerebral Cortex of Parkinsonism-Dementia Complex of Guam but Not Alzheimer’s Disease or Progressive Supranuclear Palsy

    PubMed Central

    Yang, Wan; Woltjer, Randall L.; Sokal, Izabela; Pan, Catherine; Wang, Yan; Brodey, Mary; Peskind, Elaine R.; Leverenz, James B.; Zhang, Jing; Perl, Daniel P.; Galasko, Douglas R.; Montine, Thomas J.

    2007-01-01

    Parkinsonism-dementia complex (PDC) remains a significant health burden to the Chamorro population. We tested the hypothesis that quantitative proteomics might provide fresh insight into this enigmatic illness by analyzing proteins resistant to surfactant extraction from patients with Alzheimer’s disease (AD) or PDC and their matched controls using isobaric tags for relative and absolute quantification. In addition to the expected increase in abnormal frontal cortical Aβ peptides, tau, ubiquitin, and apolipoprotein E in AD, and tau in PDC, we identified α-synuclein (SNCA) as a major abnormal protein in PDC but not AD. We confirmed our isobaric tags for relative and absolute quantification findings by enzyme-linked immunosorbent assay in frontal and temporal cortices. We extended our assays to include a limited number of cases of progressive supranuclear palsy (PSP) and dementia with Lewy bodies; we observed increased abnormal tau but not SNCA in PSP, and abnormal SNCA in dementia with Lewy bodies that was quantitatively similar to PDC. Finally, soluble Aβ oligomers were selectively increased in AD but not PDC or PSP. These results show that frontal and temporal cortex in PDC is distinguished from AD and PSP by its accumulation of abnormal SNCA and suggest that PDC be considered a synucleinopathy as well as a tauopathy. PMID:17675576

  11. [Visual hallucinations in Parkinson and Charles Bonnet Syndrome patients. A phenomenological and pathogenetic comparison].

    PubMed

    Diederich, N J; Pieri, V; Goetz, C G

    2000-03-01

    Visual hallucinations (VH) are seen in about a third of all patients with Parkinson's disease (PD) and are usually considered to be an early marker or clinical component of a dopaminergic psychosis. Their peculiar phenomenology has not yet been studied in a systematic manner. A semi-structured interview was performed twice in 62 PD patients. Different motoric and cognitive disease scales were used. The patients were not demented or depressed and had no other psychotic features other than hallucinations. Their visions was at least 0.6. 22 patients (36%) reported complex visual hallucinations or illusions in both interviews. These patients were not different from the non-hallucinating patients in terms of age, duration and stage of the disease, dosage and type of medication and frequency of cataracts. VH were diurnal in 41% of the patients, nocturnal in 18% of the patients and mixed in 41 patients. They were seen at least once weekly in 67% and they lasted always less than an hour. VH most frequently involved adults, children and pets. They were often mobile and had normal size and physiognomy. Notable emotional reactions were only reported by 18% of the patients. The phenomenology of VH in PD differs from VH in exogenous or endogenous psychoses, but is similar to the Charles Bonnet-syndrome (CBS), appearing in elderly patients with different visual deficits. As PD patients suffer regularly from visual deficits of contrast and color perception, a similar pathogenesis to CBS can be hypothesized, with these "minor" and benign VH being due to "release phenomena" in relation to partial visual deprivation. The lack of multimodality hallucinations and of secondary paranoia as well as the clear sensorium are helpful features in distinguishing them from toxic psychosis.

  12. Intermittent versus Persistent Wolff-Parkinson-White Syndrome in Children: Electrophysiologic Properties and Clinical Outcomes.

    PubMed

    Kiger, Michelle E; McCanta, Anthony C; Tong, Suhong; Schaffer, Michael; Runciman, Martin; Collins, Kathryn K

    2016-01-01

    Intermittent Wolff-Parkinson-White (WPW) syndrome is considered to have a lower risk of sudden death. Fewer data exist regarding electrophysiologic (EP) characteristics and the natural history of intermittent WPW in children. All patients with WPW age 1-18 years at a single institution (1996-2013) were reviewed. Patients with intermittent preexcitation were compared to those with loss of preexcitation on Holter/exercise testing and those with persistent preexcitation. High-risk accessory pathway (AP) was defined as AP effective refractory period (APERP), block cycle length, or shortest preexcited RR interval during atrial fibrillation ≤250 ms. A total of 295 patients were included: 226 (76.6%) persistent, 39 (13.2%) intermittent, and 30 (10.2%) loss of preexcitation Holter/exercise. There were no differences in symptoms between groups. Median interquartile range APERP was significantly longer in intermittent WPW (380 [320, 488] ms vs 320 [300, 350] ms persistent, 310 [290, 330] ms loss of preexcitation Holter/exercise; P = 0.0008). At baseline, there was no difference between groups in frequency of high-risk pathways. However, when isoproterenol values were included, high-risk pathways were more frequent among patients with loss of preexcitation on Holter/exercise (54% vs 16% persistent, 11% intermittent; P = 0.005). There was one death in a patient with loss of preexcitation on exercise testing, no EP study, and prior drug use. A second patient with persistent WPW and APERP 270 ms required resuscitation following a methadone overdose. Intermittent preexcitation in children does not connote a lower risk AP by EP criteria or reduced symptoms. The low number of pediatric WPW patients who develop preexcited atrial fibrillation or sudden death warrants larger studies to investigate these outcomes. ©2015 Wiley Periodicals, Inc.

  13. Reflections upon the Development of a Dementia Screening Service for Individuals with Down's Syndrome across the Hyndburn and Ribble Valley Area

    ERIC Educational Resources Information Center

    Cairns, Victoria; Lamb, Isobel; Smith, Esther

    2011-01-01

    The high prevalence of dementia in individuals with Down's syndrome has led learning disability services in the Hyndburn and Ribble Valley (HRV) area to develop a screening service to address this need; this paper offers reflections upon this process by its members after the first 12 months of operation. A multidisciplinary team comprising…

  14. Will General Practitioners Be Adequately Prepared to Meet the Complexities of Enhanced Dementia Screening for People with Learning Disabilities and Down Syndrome: Key Considerations

    ERIC Educational Resources Information Center

    Rowe, Michelle

    2016-01-01

    This article provides a timely response in regard to the Department of Health's current initiative to financially reward GPs to prioritise and undertake dementia screening for people with learning disabilities over the age of 50 years and for people with Down syndrome over the age of 40 years. Whilst GPs are becoming increasingly aware of their…

  15. Will General Practitioners Be Adequately Prepared to Meet the Complexities of Enhanced Dementia Screening for People with Learning Disabilities and Down Syndrome: Key Considerations

    ERIC Educational Resources Information Center

    Rowe, Michelle

    2016-01-01

    This article provides a timely response in regard to the Department of Health's current initiative to financially reward GPs to prioritise and undertake dementia screening for people with learning disabilities over the age of 50 years and for people with Down syndrome over the age of 40 years. Whilst GPs are becoming increasingly aware of their…

  16. Reflections upon the Development of a Dementia Screening Service for Individuals with Down's Syndrome across the Hyndburn and Ribble Valley Area

    ERIC Educational Resources Information Center

    Cairns, Victoria; Lamb, Isobel; Smith, Esther

    2011-01-01

    The high prevalence of dementia in individuals with Down's syndrome has led learning disability services in the Hyndburn and Ribble Valley (HRV) area to develop a screening service to address this need; this paper offers reflections upon this process by its members after the first 12 months of operation. A multidisciplinary team comprising…

  17. Efficacy and safety of donepezil in the treatment of executive dysfunction in Parkinson disease: a pilot study.

    PubMed

    Linazasoro, Gurutz; Lasa, Asier; Van Blercom, Nadége

    2005-01-01

    Cognitive disturbances in Parkinson's disease (PD) are dominated by troubles in executive functions which affects to a vast majority of parkinsonian patients since the onset of the disease. A common clinical observation is that parkinsonian patients, who eventually develop dementia, exhibit subtle cognitive disturbances quite earlier. The main biochemical substrate of cognitive dysfunction in PD, even of the early dysexecutive syndrome, might be a cholinergic deficiency. The aim of this pilot study was to determine the efficacy and safety of donepezil in the treatment of 10 patients with PD and dysexecutive alterations without dementia. All the items of the Clinical Global Impression were significantly improved. An improvement on both the modified Wisconsin Card Sorting Test and DIGIT Span was found. Parkinsonism remained unchanged during the study. Only 1 out of 10 patients experienced transient and mild gastrointestinal side effects. This study suggests that donepezil may be useful in the treatment of the dysexecutive syndrome associated with PD.

  18. Wolff-Parkinson-White syndrome in the era of catheter ablation: insights from a registry study of 2169 patients.

    PubMed

    Pappone, Carlo; Vicedomini, Gabriele; Manguso, Francesco; Saviano, Massimo; Baldi, Mario; Pappone, Alessia; Ciaccio, Cristiano; Giannelli, Luigi; Ionescu, Bogdan; Petretta, Andrea; Vitale, Raffaele; Cuko, Amarild; Calovic, Zarko; Fundaliotis, Angelica; Moscatiello, Mario; Tavazzi, Luigi; Santinelli, Vincenzo

    2014-09-02

    The management of Wolff-Parkinson-White is based on the distinction between asymptomatic and symptomatic presentations, but evidence is limited in the asymptomatic population. The Wolff-Parkinson-White registry was an 8-year prospective study of either symptomatic or asymptomatic Wolff-Parkinson-White patients referred to our Arrhythmology Department for evaluation or ablation. Inclusion criteria were a baseline electrophysiological testing with or without radiofrequency catheter ablation (RFA). Primary end points were the percentage of patients who experienced ventricular fibrillation (VF) or potentially malignant arrhythmias and risk factors. Among 2169 enrolled patients, 1001 (550 asymptomatic) did not undergo RFA (no-RFA group) and 1168 (206 asymptomatic) underwent ablation (RFA group). There were no differences in clinical and electrophysiological characteristics between the 2 groups except for symptoms. In the no-RFA group, VF occurred in 1.5% of patients, virtually exclusively (13 of 15) in children (median age, 11 years), and was associated with a short accessory pathway antegrade refractory period (P<0.001) and atrioventricular reentrant tachycardia initiating atrial fibrillation (P<0.001) but not symptoms. In the RFA group, ablation was successful in 98.5%, and after RFA, no patients developed malignant arrhythmias or VF over the 8-year follow-up. Untreated patients were more likely to experience malignant arrhythmias and VF (log-rank P<0.001). Time-dependent receiver-operating characteristic curves for predicting VF identified an optimal anterograde effective refractory period of the accessory pathway cutoff of 240 milliseconds. The prognosis of the Wolff-Parkinson-White syndrome essentially depends on intrinsic electrophysiological properties of AP rather than on symptoms. RFA performed during the same procedure after electrophysiological testing is of benefit in improving the long-term outcomes. © 2014 American Heart Association, Inc.

  19. Should We Refer for a Dementia Assessment? A Checklist to Help Know when to Be Concerned about Dementia in Adults with Down Syndrome and Other Intellectual Disabilities

    ERIC Educational Resources Information Center

    Whitwham, Sarah; McBrien, Judith; Broom, Wendy

    2011-01-01

    The aim of this research was to develop a simple screening checklist to help carers and professionals know when to make a referral for a dementia assessment. A checklist was completed for all new referrals to a dementia service for people with intellectual disabilities. The obtained scores were compared to the diagnostic outcome of a comprehensive…

  20. Should We Refer for a Dementia Assessment? A Checklist to Help Know when to Be Concerned about Dementia in Adults with Down Syndrome and Other Intellectual Disabilities

    ERIC Educational Resources Information Center

    Whitwham, Sarah; McBrien, Judith; Broom, Wendy

    2011-01-01

    The aim of this research was to develop a simple screening checklist to help carers and professionals know when to make a referral for a dementia assessment. A checklist was completed for all new referrals to a dementia service for people with intellectual disabilities. The obtained scores were compared to the diagnostic outcome of a comprehensive…

  1. Utility of Exercise Testing and Adenosine Response for Risk Assessment in Children with Wolff-Parkinson-White Syndrome.

    PubMed

    Ergul, Yakup; Ozturk, Erkut; Ozyilmaz, Isa; Unsal, Serkan; Carus, Hayat; Tola, Hasan Tahsin; Tanidir, Ibrahim Cansaran; Guzeltas, Alper

    2015-01-01

    We aimed to determine the correlation between noninvasive testing (exercise stress testing [EST] and adenosine responsiveness of accessory pathway [AP] ) and invasive electrophysiology study (EPS) for assessment antegrade conduction of the AP in Wolff-Parkinson-White syndrome. This prospective, observational study enrolled 40 children (58% male children, median age of 13 years, and median weight of 47.5 kg) with Wolff-Parkinson-White syndrome. Conduction through the AP to a cycle length of ≤250 ms was considered rapid or high-risk; otherwise, patients were nonrapid or low-risk. The sudden disappearance of the delta-wave was seen in 10 cases (25%) during EST. Accessory pathway was found to be high-risk in 13 cases (13/40, 32.5%) while the accessory path was identified as low-risk in 27 cases; however, six patients (15%) had blocked AP conduction with adenosine during EPS. Low-risk classification by EST alone to identify patients with nonrapid conduction in baseline EPS had a specificity of 93% and a positive predictive value of 90% (accuracy 54%). Blocked AP conduction with adenosine as a marker of nonrapid baseline AP conduction had a specificity of 93% and a positive predictive value of 84%. Finally, AP was adenosine nonresponsive in the majority of patients (28/30, 93%) with persistent delta-waves, 40% of those who had a sudden disappearance of delta-waves had an adenosine-responsive AP (P value: .028). Abrupt loss of preexcitation during EST and blocked AP conduction with adenosine had high specificity and positive predictive value for nonrapid and low-risk antegrade conduction during baseline invasive EPS. Successful risk stratification of pediatric patients with Wolff-Parkinson-White is possible through the use of EST and the adenosine responsiveness of AP. © 2015 Wiley Periodicals, Inc.

  2. A randomized study of prophylactic catheter ablation in asymptomatic patients with the Wolff-Parkinson-White syndrome.

    PubMed

    Pappone, Carlo; Santinelli, Vincenzo; Manguso, Francesco; Augello, Giuseppe; Santinelli, Ornella; Vicedomini, Gabriele; Gulletta, Simone; Mazzone, Patrizio; Tortoriello, Valter; Pappone, Alessia; Dicandia, Cosimo; Rosanio, Salvatore

    2003-11-06

    Young age and inducibility of atrioventricular reciprocating tachycardia or atrial fibrillation during invasive electrophysiological testing identify asymptomatic patients with a Wolff-Parkinson-White pattern on the electrocardiogram as being at high risk for arrhythmic events. We tested the hypothesis that prophylactic catheter ablation of accessory pathways would provide meaningful and durable benefits as compared with no treatment in such patients. From 1997 to 2002, among 224 eligible asymptomatic patients with the Wolff-Parkinson-White syndrome, patients at high risk for arrhythmias were randomly assigned to radio-frequency catheter ablation of accessory pathways (37 patients) or no treatment (35 patients). The end point was the occurrence of arrhythmic events over a five-year follow-up period. Patients assigned to ablation had base-line characteristics that were similar to those of the controls. Two patients in the ablation group (5 percent) and 21 in the control group (60 percent) had arrhythmic events. One control patient had ventricular fibrillation as the presenting arrhythmia. The five-year Kaplan-Meier estimates of the incidence of arrhythmic events were 7 percent among patients who underwent ablation and 77 percent among the controls (P<0.001 by the log-rank test); the risk reduction with ablation was 92 percent (relative risk, 0.08; 95 percent confidence interval, 0.02 to 0.33; P<0.001). Prophylactic accessory-pathway ablation markedly reduces the frequency of arrhythmic events in asymptomatic patients with the Wolff-Parkinson-White syndrome who are at high risk for such events. Copyright 2003 Massachusetts Medical Society

  3. Phase analysis in the Wolff-Parkinson-White syndrome with surgically proven accessory conduction pathways: concise communication

    SciTech Connect

    Nakajima, K.; Bunko, H.; Tada, A.; Taki, J.; Tonami, N.; Hisada, K.; Misaki, T.; Iwa, T.

    1984-01-01

    Twenty-one patients with the Wolff-Parkinson-White (WPW) syndrome who underwent surgical division of the accessory conduction pathway (ACP) were studied by gated blood-pool scintigraphy. In each case, a functional image of the phase was generated, based on the fundamental frequency of the Fourier transform. The location of the ACP was confirmed by electrophysiologic study, epicardial mapping, and surgery. Phase analysis identified the side of preexcitation correctly in 16 out of 20 patients with WPW syndrome with a delta wave. All patients with right-cardiac type (N=9) had initial contraction in the right ventricle (RV). In patients with left-cardiac type (N=10), six had initial movement in the left ventricle (LV); but in the other four the ACPs in the anterior or lateral wall of the left ventricle (LV) could not be detected. In patients with multiple ACPs (N=2), one right-cardiac type had initial contraction in the RV, while in the other (with an intermittent WPW syndrome) the ACP was not detected. These observations indicate that abnormal wall motion is associated with the conduction anomalies of the WPW syndrome. We conclude that phase analysis can correctly identify the side of initial contraction in the WPW syndrome before and after surgery. However, as a method of preoperative study, it seems difficult to determine the precise site of the ACP by phase analysis alone.

  4. [The natural history of 270 cases of Wolff-Parkinson-White syndrome in a survey of the general population].

    PubMed

    Soria, R; Guize, L; Chretien, J M; Le Heuzey, J Y; Lavergne, T; Desnos, M; Hagege, A; Guerre, Y

    1989-03-01

    Among 226,464 ambulatory subjects who underwent medical check-ups over a 15-year period, 270 were found to have Wolff-Parkinson-White syndrome (1.2 case in 1,000). The syndrome was more frequent in men (181 cases, 1.4 p. 1,000) than in women (89 cases, 0.9 p. 1,000). 222 subjects were aged from 20 to 49 years (1.4 p. 1,000) and only 48 were between 50 and 80 years of age (0.7 p. 1,000). 197 subjects were re-evaluated: 119 (60.4 p. 100) complained of palpitations and 78 (39.6 p. 100) were asymptomatic. Palpitations began at all ages, even after 50 years, and usually proceeded in short attacks lasting a few seconds or minutes, with a mean recurrence rate of 5 attacks per annum (76.4 p. 100). This constant pattern sometimes was interrupted for months or years. Conversely, in a minority of cases (23.5 p. 100) an unexpected accentuation occurred which lasted for hours or days. As years went by, palpitations tented to decrease and disappear. The pre-excitation area and its degree of fusion with the normal ventricular activation had no influence on the origin and frequency of palpitations. In contrast, sustained tachycardia seemed to be more frequent in cases with lateral and posterior left pre-excitation. Among 270 subjects with pre-excitation syndrome, 7 died including 4 whose death was not due to a cardiac disease, 2 who died suddenly and 1 who succumbed to ventricular tachycardia after a road accident. None of these patients had an associated heart disease. These last 3 cases might contribute to alter the usually favourable prognosis of Wolff-Parkinson-White syndrome.

  5. Dural arteriovenous fistula as a treatable dementia

    PubMed Central

    Enofe, Ikponmwosa; Thacker, Ike

    2017-01-01

    Dementia is a chronic loss of neurocognitive function that is progressive and irreversible. Although rare, dural arteriovenous fistulas (DAVFs) could present with a rapid decline in neurocognitive function with or without Parkinson-like symptoms. DAVFs represent a potentially treatable and reversible cause of dementia. Here, we report the case of an elderly woman diagnosed with a DAVF after presenting with new-onset seizures, deteriorating neurocognitive function, and Parkinson-like symptoms. PMID:28405088

  6. Politics of science: Progress toward prevention of the dementia-Alzheimer's syndrome.

    PubMed

    Khachaturian, Zaven S; Khachaturian, Ara S

    2015-01-01

    There exist many challenges hampering the discovery and development of effective interventions to prevent dementia. Three major trends have now intersected to influence the emerging interest in disease modifying therapies that may delay or halt dementia. The three crucial factors shaping this current focus are: (1) the emergence of the longevity revolution and the impact of a aging society, (2) the effects of the US Federal investment in research in advancing knowledge about the neurobiology of aging and dementia, and (3) the problem of US legislators and health policy makers to balance the allocation of evermore scarce research funding resources. The purpose of this essay is to provide a survey of the politics of science and to describe efforts to correctly manage the high level of expectations of both the patient and research communities. The perspective offered reviews the history and evolution of the ideas to treat or prevent dementia and Alzheimer's disease as a national strategic goal. The aim is to evaluate the interplay between science and formulation of public policy for setting research priority. We use the history of developing US National Institute of Aging's extramural research programs on brain aging and Alzheimer's disease (Khachaturian, 2006; 2007) as an initial case study. Copyright © 2015 Elsevier Ltd. All rights reserved.

  7. Alaska Native Parkinson’s Disease Registry

    DTIC Science & Technology

    2011-06-01

    Investigator Parkinsonism (PS) is a syndrome characterized by tremor , rigidity, slowness of movement, and problems with walking and balance...2. Developing an identification protocol. The primary source of parkinsonism cases will be the Indian Health Service (IHS) provider database, called...of parkinsonism among Alaska Natives. Status: Complete 3. Developing a secure Alaska Native parkinsonism registry database. Status: The database

  8. A Four-Year Longitudinal Study on Restless Legs Syndrome in Parkinson Disease.

    PubMed

    Moccia, Marcello; Erro, Roberto; Picillo, Marina; Santangelo, Gabriella; Spina, Emanuele; Allocca, Roberto; Longo, Katia; Amboni, Marianna; Palladino, Raffaele; Assante, Roberta; Pappatà, Sabina; Pellecchia, Maria Teresa; Barone, Paolo; Vitale, Carmine

    2016-02-01

    Restless legs syndrome (RLS) prevalence estimates range from 0% to 52% in Parkinson disease (PD), but the causal relationship between the two disorders is still debated. The present study aims to evaluate RLS prevalence in de novo PD subjects, its incidence during the first 4 years from diagnosis, and possible relationships with clinical, laboratory, and neuroradiological data. One hundred nine newly diagnosed, drug-naïve PD subjects were evaluated at the time of PD diagnosis, and after 2- and 4-years. RLS diagnosis was performed with the RLS Diagnostic Index at each visit. Motor features, additional non-motor symptoms (NMS), and concomitant dopaminergic and nondopaminergic treatments were also gathered. Moreover, at baseline, 65 subjects were randomly selected to undergo a FP-CIT SPECT to study dopamine transporter availability. RLS prevalence rose from 4.6% at baseline evaluation to 6.5% after 2 years and to 16.3% after 4 years (P = 0.007). A multinomial logistic stepwise regression model selected NMS Questionnaire items more likely to be associated with RLS at diagnosis (insomnia, OR = 15.555; P = 0.040) and with occurrence of RLS during follow-up (dizziness, OR = 1.153; P = 0.022; and daytime sleepiness; OR = 9.557; P = 0.001), as compared to patients without RLS. Older age was more likely associated to increased RLS occurrence during follow-up in a random effect logistic regression model (OR = 1.187; P = 0.036). A multinomial logistic stepwise model found increased dopaminergic transporter availability of affected caudate and putamen to be more likely associated with RLS presence at diagnosis (n = 5; OR = 75.711; P = 0.077), and RLS occurrence during follow-up (n = 16; OR = 12.004; P = 0.059), respectively, as compared to patients without RLS (n = 88). RLS is present since PD diagnosis, and increases in prevalence during the course of PD. PD subjects with RLS have higher age at PD onset, more preserved dopaminergic pathways, and worse sleep and cardiovascular

  9. [Prevalence and electrocardiographic forms of the Wolff-Parkinson-White syndrome].

    PubMed

    Soria, R; Guize, L; Fernandez, F; Chaouat, J C; Chrétien, J M

    1982-12-01

    In a routine electrocardiographic study of 133929 subjects aged from 20 to 73, 136 cases of the Wolff-Parkinson-White syndrome were detected, 6 with intermittent pre-excitation. In this study, the prevelance of WPW was about 1 in a 1000, the highest incidence being in the 20-40 year age group with an equal sex ratio. The ECG analysis of the 136 cases consisted in determining the orientation of the delta wave in the precordial leads to establish the right or left ventricular origin of the pre-excitation, calculating the direction of the delta wave vector in the frontal plane to find out the anterior, lateral or posterior origin of the pre-excitation and analyse the position of the QRS axis to assess the appearances of the latest ventricular activity. The 136 ECGs were then classified according to electrophysiological criteria and the results of mapping: 1. Left ventricular pre-excitation; 74 cases characterised by a dominant delta wave in the right precordial leads. These cases were subdivided into: - 30 cases with posterior paraseptal pre-excitation, axis of the delta wave deviated superiorly and to the left, between -30 degrees and -60 degrees; - 20 cases of lateral pre-excitation with the vector of the delta wave deviated inferiorly and to the right between +100 degrees and +120 degrees; - 24 cases of anterior paraseptal pre-excitation with high amplitude delta and QRS deflections in all precordial leads and a delta wave axis between +50 degrees and +80 degrees. 2. Right ventricular pre-excitation; 62 cases characterised by a negative or isoelectric delta wave in the right precordial leads, including: - 14 posterior paraseptal pre-excitation with significant delta wave axis deviation between -30 degrees and -60 degrees; - 33 lateral pre-excitation with the delta and QRS axis pointing directly to the left at about 0 degrees; - 15 cases of anterior paraseptal pre-excitation with the delta wave axis between +50 degrees and +80 degrees. The cases with terminal forces

  10. Patterns of Performance on the Modified Cued Recall Test in Spanish Adults With Down Syndrome With and Without Dementia.

    PubMed

    Benejam, Bessy; Fortea, Juan; Molina-López, Rafael; Videla, Sebastià

    2015-11-01

    The assessment of memory decline in people with intellectual disability (ID) is more difficult than in the general population, due to a lack of appropriate instruments and to preexisting cognitive impairment. The aim of this study was to describe performance of healthy adults with Down syndrome (healthy-DS; prospectively cohort) on a Spanish version of the modified Cued Recall Test (mCRT). We also recruited retrospectively a cohort of DS subjects with Dementia of the Alzheimer's Type (DS-DAT). Healthy-DS obtained higher scores on free recall and total score than DS-DAT. Age was the main factor associated with decreasing mCRT scores. The mCRT was useful in DS subjects with ID at the upper end of the spectrum or ID in the middle range of the spectrum, and discriminated well between DS subjects with and without DAT.

  11. [Delirium and dementia].

    PubMed

    Marín Carmona, José Manuel

    2008-01-01

    Delirium and dementia are highly prevalent neurocognitive syndromes in the elderly. These syndromes are defined by level of consciousness, clinical onset, and potential reversibility, etc. Frequently, both syndromes coincide in the elderly patient and share many epidemiologic, pathogenic and clinical features, which are reviewed in this article. There is no solid scientific evidence that explains the association between delirium and dementia. The present article proposes a change of paradigm in the diagnostic, preventive and therapeutic approach to delirium in the elderly that recognizes the inherent complexity of this geriatric syndrome and, unlike dichotomic models, explores the complex interrelations between both geriatric syndromes. Delirium is viewed as an important model to investigate cognitive disorders and dementia.

  12. [A case of Parkinson syndrome secondary to combined amineptine and bromazepam abuse].

    PubMed

    Lapaz, A G; Pozo, P; Romero, H; Barcia, D

    1998-01-01

    A case report of parkinsonism secondary to chronic abuse of amineptine (3 gr/day) and bromazepam (35 mg/day) in a patient diagnosed of borderline personality disorder is presented. The patient did not take any other drugs and he was not recently on neuroleptic treatment; he recognized the abuse of amineptine, as a stimulant, and the bromazepam abuse, looking for a relief to the excessive anxiety secondary to amineptine. The parkinsonism improved after removing both drugs and taking biperiden and diazepam; lastly the patient was discharged without any medication. The patient did not suffer from other complications associated with amineptine or bromazepam abuse. There are some cases reported of parkinsonism secondary to benzodiazepines but there is none secondary to amineptine. We present a short review of the possible responsible mechanisms, thinking on a complex interaction of both drugs on dopamine and its modulatory systems.

  13. Inflammation and Parkinson's disease.

    PubMed

    Wersinger, Christophe; Sidhu, Anita

    2002-09-01

    Numerous recent findings indicate the possible involvement of an immune mechanism in the pathogenesis of neurodegeneration. The immune reaction could either act as a primary event, generating changes leading to cell death, or could be a secondary response to neuronal injury. In various neurodegenerative disorders such as Alzheimer's, Huntington's or Pick's disease, Down's syndrome, multiple sclerosis and the AIDS-dementia complex, the inflammatory pathomechanism is strongly supported by experimental and clinical studies. Such inflammatory mechanisms have also been postulated in Parkinson's disease (PD). This review summarizes some generalities about inflammation and immune reactions in the context of the brain, and provides clinical, epidemiological and experimental data showing that inflammation and immunity, or even auto-immunity, could be implicated in PD, either in its initial step or in its progression. Different experimental models useful for studying the role(s) of inflammation and (auto)immunity in the neurodegenerative process of the dopaminergic neurons in PD are examined. The major similarities and differences between PD and other neurodegenerative disorders are discussed.

  14. Genetic impact on cognition and brain function in newly diagnosed Parkinson's disease: ICICLE-PD study.

    PubMed

    Nombela, Cristina; Rowe, James B; Winder-Rhodes, Sophie E; Hampshire, Adam; Owen, Adrian M; Breen, David P; Duncan, Gordon W; Khoo, Tien K; Yarnall, Alison J; Firbank, Michael J; Chinnery, Patrick F; Robbins, Trevor W; O'Brien, John T; Brooks, David J; Burn, David J; Barker, Roger A

    2014-10-01

    Parkinson's disease is associated with multiple cognitive impairments and increased risk of dementia, but the extent of these deficits varies widely among patients. The ICICLE-PD study was established to define the characteristics and prevalence of cognitive change soon after diagnosis, in a representative cohort of patients, using a multimodal approach. Specifically, we tested the 'Dual Syndrome' hypothesis for cognitive impairment in Parkinson's disease, which distinguishes an executive syndrome (affecting the frontostriatal regions due to dopaminergic deficits) from a posterior cortical syndrome (affecting visuospatial, mnemonic and semantic functions related to Lewy body pathology and secondary cholinergic loss). An incident Parkinson's disease cohort (n = 168, median 8 months from diagnosis to participation) and matched control group (n = 85) were recruited to a neuroimaging study at two sites in the UK. All participants underwent clinical, neuropsychological and functional magnetic resonance imaging assessments. The three neuroimaging tasks (Tower of London, Spatial Rotations and Memory Encoding Tasks) were designed to probe executive, visuospatial and memory encoding domains, respectively. Patients were also genotyped for three polymorphisms associated with cognitive change in Parkinson's disease and related disorders: (i) rs4680 for COMT Val158Met polymorphism; (ii) rs9468 for MAPT H1 versus H2 haplotype; and (iii) rs429358 for APOE-ε2, 3, 4. We identified performance deficits in all three cognitive domains, which were associated with regionally specific changes in cortical activation. Task-specific regional activations in Parkinson's disease were linked with genetic variation: the rs4680 polymorphism modulated the effect of levodopa therapy on planning-related activations in the frontoparietal network; the MAPT haplotype modulated parietal activations associated with spatial rotations; and APOE allelic variation influenced the magnitude of activation

  15. Parkinson's Disease

    MedlinePlus

    ... Emergency Room? What Happens in the Operating Room? Parkinson's Disease KidsHealth > For Kids > Parkinson's Disease A A ... symptoms of something called Parkinson's disease. What Is Parkinson's Disease? You may have seen the actor Michael ...

  16. Dopamine dysregulation syndrome and levodopa-induced dyskinesias in Parkinson disease: common consequences of anomalous forms of neural plasticity.

    PubMed

    Linazasoro, Gurutz

    2009-01-01

    Four to 10% of patients with Parkinson disease and chronically treated with levodopa undergo an addictionlike behavioral disturbance named dopamine dysregulation syndrome (DDS). This article suggests that patients with Parkinson disease could be especially prone to develop DDS due to the dopamine deficiency and the "priming" of neural networks by the chronic use of drugs with a short half-life, such as levodopa. These suggestions are based on the clinical and molecular similarities between levodopa-induced dyskinesias and behavioral alterations seen in DDS and addiction to illegal drugs. Motor and behavioral abnormalities can be seen as the consequence of common mechanisms involving anomalous forms of neural plasticity. These forms affect parts of the cortical-basal ganglia-thalamocortical circuits that are topographically organized to differently modulate emotional and motor functions. Recent evidence using positron emission tomography provides support to this idea. By contrast, molecular data suggest that functional segregation may be lost in addiction, DDS, and dyskinesias. The existence of common pathogenic mechanisms for both phenomena could provide the basis for common therapeutic strategies.

  17. Wolff-Parkinson-White syndrome type B and left bundle-branch block: electrophysiologic and radionuclide study

    SciTech Connect

    Rakovec, P.; Kranjec, I.; Fettich, J.J.; Jakopin, J.; Fidler, V.; Turk, J.

    1985-01-01

    Coinciding left bundle-branch block and Wolff-Parkinson-White syndrome type B, a very rare electrocardiographic occurrence, was found in a patient with dilated cardiomyopathy. Electrophysiologic study revealed eccentric retrograde atrial activation during ventricular pacing, suggesting right-sided accessory pathway. At programmed atrial pacing, effective refractory period of the accessory pathway was 310 ms; at shorter pacing coupling intervals, normal atrioventricular conduction with left bundle-branch block was seen. Left bundle-branch block was seen also with His bundle pacing. Radionuclide phase imaging demonstrated right ventricular phase advance and left ventricular phase delay; both right and left ventricular phase images revealed broad phase distribution histograms. Combined electrophysiologic and radionuclide investigations are useful to disclose complex conduction abnormalities and their mechanical correlates.

  18. A 6.4MB duplication of the alpha-synuclein locus causing fronto-temporal dementia and parkinsonism - phenotype-genotype correlations

    PubMed Central

    Kara, Eleanna; Kiely, Aoife P; Proukakis, Christos; Giffin, Nicola; Love, Seth; Hehir, Jason; Rantell, Khadija; Pandraud, Amelie; Hernandez, Dena G; Nacheva, Elizabeth; Pittman, Alan M; Nalls, Mike A; Singleton, Andrew B; Revesz, Tamas; Bhatia, Kailash P; Quinn, Niall; Hardy, John; Holton, Janice L; Houlden, Henry

    2015-01-01

    Importance SNCA locus duplications are associated with variable clinical features and reduced penetrance but the reasons underlying this variability are unknown. Objective 1) To report a novel family carrying a heterozygous 6.4Mb duplication of the SNCA locus with an atypical clinical presentation strongly reminiscent of frontotemporal dementia (FTD) and late-onset pallidopyramidal syndromes. 2) To study phenotype-genotype correlations in SNCA locus duplications. Design, Setting, Participants and Data sources We report the clinical and neuropathologic features of a family carrying a 6.4Mb duplication of the SNCA locus. To identify candidate disease modifiers, we undertake a genetic analysis in the family and conduct statistical analysis on previously published cases carrying SNCA locus duplication using regression modelling with robust standard errors to account for clustering at the family level. Main outcome measures To assess whether length of the SNCA locus duplication influences disease penetrance and severity, and whether extra-duplication factors have a disease-modifying role. Results We identified a large 6.4Mb duplication of the SNCA locus in this family. Neuropathological analysis showed extensive α-synuclein pathology with minimal phospho-tau pathology. Genetic analysis showed an increased burden of PD-related risk factors and the disease-predisposing H1/H1 MAPT haplotype. Statistical analysis of previously published cases suggested that there is a trend towards increasing disease severity and disease penetrance with increasing duplication size. The corresponding odds ratios (95% CI) from the univariate analyses were 1.17 (0.81 to 1.68) and 1.34 (0.78 to 2.31) respectively. Gender was significantly associated with both disease risk and severity; males compared to females had increased disease risk and severity and the corresponding odds ratios (95% CI) from the univariate analyses were 8.36 (1.97 to 35.42) and 5.55 (1.39 to 22.22) respectively

  19. Comparison of the accuracy of three algorithms in predicting accessory pathways among adult Wolff-Parkinson-White syndrome patients.

    PubMed

    Maden, Orhan; Balci, Kevser Gülcihan; Selcuk, Mehmet Timur; Balci, Mustafa Mücahit; Açar, Burak; Unal, Sefa; Kara, Meryem; Selcuk, Hatice

    2015-12-01

    The aim of this study was to investigate the accuracy of three algorithms in predicting accessory pathway locations in adult patients with Wolff-Parkinson-White syndrome in Turkish population. A total of 207 adult patients with Wolff-Parkinson-White syndrome were retrospectively analyzed. The most preexcited 12-lead electrocardiogram in sinus rhythm was used for analysis. Two investigators blinded to the patient data used three algorithms for prediction of accessory pathway location. Among all locations, 48.5% were left-sided, 44% were right-sided, and 7.5% were located in the midseptum or anteroseptum. When only exact locations were accepted as match, predictive accuracy for Chiang was 71.5%, 72.4% for d'Avila, and 71.5% for Arruda. The percentage of predictive accuracy of all algorithms did not differ between the algorithms (p = 1.000; p = 0.875; p = 0.885, respectively). The best algorithm for prediction of right-sided, left-sided, and anteroseptal and midseptal accessory pathways was Arruda (p < 0.001). Arruda was significantly better than d'Avila in predicting adjacent sites (p = 0.035) and the percent of the contralateral site prediction was higher with d'Avila than Arruda (p = 0.013). All algorithms were similar in predicting accessory pathway location and the predicted accuracy was lower than previously reported by their authors. However, according to the accessory pathway site, the algorithm designed by Arruda et al. showed better predictions than the other algorithms and using this algorithm may provide advantages before a planned ablation.

  20. [Sleep disturbances in Alzheimer's disease and other dementias].

    PubMed

    Vecchierini, Marie-Françoise

    2010-03-01

    Sleep in dementias has been mainly studied in Alzheimer disease (AD). Sleep disturbances are found in 25 to 35% of subjects with AD. Subjective and objective disturbances are described. Long nocturnal awakenings disrupt sleep; total sleep time and sleep efficiency are reduced. Slow wave sleep is decreased and sometimes disappears. REM sleep percentage is also reduced and at a later stage of the disease, REM latency is increased. Sleep fragmentation can be associated with excessive daytime napping and sleepiness, and with other behavioral symptoms such as the sundowning syndrome and nocturnal agitation. Sleep abnormalities closely parallel the level of severity of dementia. The rest/activity ratio and the sleep-wake rhythms are more and more disturbed; the phase delay of the temperature rhythm is associated with the severity of the sundowning syndrome. Sleep disturbances and behavioral symptoms are the main reasons to institutionalize the patient. Sleep disturbances are related to multiple factors. Pathophysiological changes resulting of the disease itself, such as damage to the cholinergic pathways and to the circadian pacemaker in the suprachiasmatic nucleus, contribute to sleep changes in AD. Associated medical and psychiatric illness and their different treatments as well as environmental factors also induced sleep disturbances. Sleep-disordered breathing is a highly prevalent condition in AD patients and restless leg syndrome may account for nocturnal agitation. In Parkinson and in Lewy body dementias, sleep disturbances are more severe than in DA and REM sleep behavior disorder can precede by several years these diseases. Sleep attacks and sleepiness are very frequent in Parkinson disease. Specific etiologies should drive specific treatment. Several non pharmacologic treatments are usually associated to treat sleep disturbances in AD: information, increased daytime physical, social activities to minimize daytime naps and exposure to bright light. Some studies

  1. Cognitive impairment in degenerative parkinsonisms: role of radionuclide brain imaging.

    PubMed

    Arnaldi, D; Morbelli, S; Morrone, E; Campus, C; Nobili, Flavio

    2012-02-01

    Cognitive impairment in Parkinson's disease (PD) and atypical parkinsonian syndromes is gaining increased clinical significance. The neurochemical and neuropathological basis in the various parkinsonian forms and even in an individual patient are not fully elucidated yet and could be heterogeneous. Loss of dopaminergic, cholinergic and noradrenergic innervation has been suggested to be the underlying neurochemical deficits for cognitive impairment and dementia in PD, but the onset of cognitive impairment and the progression to dementia may not share the same underlying neurochemical basis. Similarly, pathological evidence is also heterogeneous, ranging from subcortical pathology, limbic or cortical Lewy body type degeneration, and Alzheimer's type pathology that can be found even in the same patient with PD dementia (PDD). Typically, the prototype of early cognitive deficit in PD is a dysexecutive syndrome, but other cognitive domains are more involved when dementia develops, mainly including visuospatial, language and memory dysfunction. Functional radionuclide neuroimaging, by means of single-photon emission computed tomography and positron emission tomography, are contributing to characterize the topographic cortical pattern of cognitive impairment, as well as to define the underlying neurochemical deficit. Lastly, the advent of amyloid PET may help clarifying the meaning of amyloid load in diffuse Lewy body disease and PDD. Knowing the neurochemical and pathophysiological substrate of cognitive deficit in patients with PD or other degenerative Parkinsonisms may help the clinician in understanding the clinical condition of an individual patient in order to plan pharmacological and non-pharmacological intervention. The introduction of acetylcholinesterase inhibitors for therapy of PDD is an example of information integration between clinical-neuropsychological and pathophysiological-neurochemical aspects obtained also with the key contribution of functional

  2. Dementia in intellectual disability.

    PubMed

    Sheehan, Rory; Ali, Afia; Hassiotis, Angela

    2014-03-01

    Dementia is emerging as a significant condition in the population with intellectual disability. This review is aimed at clinicians working in the field. We revisit what is known on the subject and expand on this with results from recent research. The emphasis of this review is on the clinical research rather than laboratory or molecular research. Research has encompassed all aspects of dementia in intellectual disability, from epidemiology, assessment and diagnosis, through to management. There remains a lack of evidence concerning both pharmacological and nonpharmacological treatment of dementia in people with intellectual disability. Recent research has tended to focus on dementia in Down syndrome. More research is necessary in order to translate improvements in the understanding of the neuropathology of intellectual disability and dementia into effective treatments. There is also a need to investigate the optimum environment in which to provide holistic care for individuals affected.

  3. Validation of the Lille's Apathy Rating Scale in Very Mild to Moderate Dementia.

    PubMed

    Fernández-Matarrubia, Marta; Matías-Guiu, Jordi A; Moreno-Ramos, Teresa; Valles-Salgado, María; Marcos-Dolado, Alberto; García-Ramos, Rocío; Matías-Guiu, Jorge

    2016-07-01

    Apathy is one of the most common and disabling syndromes of dementia and presents at all stages of the disease. Comprehensive and structured methods to assess apathy in dementia are still needed. Lille's Apathy Rating Scale (LARS) has shown good psychometric properties for apathy evaluation in Parkinson disease but has not been validated in dementia. The aim of this study was to validate the LARS in a cohort of patients with very mild to moderate dementia. 101 patients with cognitive impairment (Clinical Dementia Rating ≤ 2) and 50 healthy subjects were recruited. Patient diagnoses included 43 individuals with Alzheimer disease, 41 frontotemporal dementia, and 17 primary progressive aphasia. In addition to LARS, the following assessments were administered: Clinical Dementia Rating, Interview for Deterioration in Daily Living Activities in Dementia, Functional Activities Questionnaire, Frontal Behavioral Inventory, Neuropsychiatric Inventory (NPI), and Hamilton Depression Rating Scale. Internal consistency for LARS (Cronbach's alpha) was 0.940. Test-retest intraclass correlation coefficient (ICC) was 0.940 and inter-rater ICC was 0.987. The correlation among LARS and NPI apathy scores (concurrent validity) was 0.834. Receiver operating characteristic analysis estimated an area under the curve of 0.987. The optimal cutoff point was -10. Although total LARS score was influenced by the presence of depression, this disorder was independent with respect to apathy. LARS is reliable and valid for detecting and quantifying apathy in patients with dementia, even in very early stages of the disease. Copyright © 2016 American Association for Geriatric Psychiatry. Published by Elsevier Inc. All rights reserved.

  4. [Artistic creativity and dementia].

    PubMed

    Sellal, François; Musacchio, Mariano

    2008-03-01

    Artistic creativity can be defined as the ability to produce both innovative and esthetic works. Though most dementias result in a loss of instrumental functions and a deterioration in artistic production, for some established artists, dementia, most often Alzheimer's disease, changed their style and technique but preserved their creativity and prolific artistic drive. Moreover, in some cases, mainly frontotemporal dementia, Parkinson's disease, and very occasionally strokes, the disease may favour the emergence of de novo artistic talent. This phenomenon has been conceptualized as a paradoxical facilitation, a disinhibition of brain areas devoted to visuospatial processing, greater freedom in a patient who becomes less bound by social conventions, enhancement of motivation and pleasure, etc. These neurological cases provide an opportunity to shed some light on the roots of artistic creation.

  5. Clinical presentation and differential diagnosis of dementia with Lewy bodies: a review.

    PubMed

    Morra, L F; Donovick, P J

    2014-06-01

    Dementia with Lewy bodies is one of the most prevalent dementia diagnoses. However, differential diagnosis between dementia with Lewy bodies, Alzheimer's disease, and Parkinson's disease with dementia can still be very difficult given the overlap in neuropathology, clinical presentation, cognitive, and neuroanatomical changes. A literature review of dementia with Lewy bodies, Alzheimer's disease, and Parkinson's disease with dementia was conducted using PubMed. Accurate diagnosis of dementia with Lewy bodies is crucial in order to more accurately predict the progression of the disease and negative side effects from pharmacological treatment. The differences and similarities between dementia with Lewy bodies, Alzheimer's disease, and Parkinson's disease with dementia are highlighted in order to aid clinicians in differential diagnosis. Copyright © 2013 John Wiley & Sons, Ltd.

  6. Mixed Dementia

    MedlinePlus

    ... community Use our Virtual Library Treatment and outcomes back to top Because most people with mixed dementia are diagnosed with a single type of dementia, physicians often base their prescribing decisions on the type of dementia ...

  7. Magnetic Resonance Imaging and Magnetic Resonance Spectroscopy in Dementias

    PubMed Central

    Hsu, Yuan-Yu; Du, An-Tao; Schuff, Norbert; Weiner, Michael W.

    2007-01-01

    This article reviews recent studies of magnetic resonance imaging and magnetic resonance spectroscopy in dementia, including Alzheimer's disease, frontotemporal dementia, dementia with Lewy bodies, idiopathic Parkinson's disease, Huntington's disease, and vascular dementia. Magnetic resonance imaging and magnetic resonance spectroscopy can detect structural alteration and biochemical abnormalities in the brain of demented subjects and may help in the differential diagnosis and early detection of affected individuals, monitoring disease progression, and evaluation of therapeutic effect. PMID:11563438

  8. The prevalence of frontal variant frontotemporal dementia and the frontal lobe syndrome in a population based sample of 85 year olds

    PubMed Central

    Gislason, T; Sjogren, M; Larsson, L; Skoog, I

    2003-01-01

    Objectives: To investigate the prevalence of the frontal lobe syndrome (FLS) and the frontal variant of frontotemporal dementia (fvFTD) in a population based sample of 85 year olds. Methods: A representative sample of 85 year olds (n = 451) in Gothenburg, Sweden was examined with a neuropsychiatric examination and a key informant interview performed by an experienced psychiatrist. A subsample underwent computed tomography (CT) of the head. The Lund-Manchester research criteria were used as a basis for a symptom algorithm to identify individuals with FLS and fvFTD. These were diagnosed blindly to the diagnosis of dementia according to DSM-III-R. Results: A total of 86 individuals (19%) fulfilled the criteria for FLS, and 14 of them fulfilled criteria for fvFTD. There were no differences between men and women. Among those with FLS, 75 (87%) fulfilled DSM-III-R criteria for other types of dementia, mainly Alzheimer's disease and vascular dementia. Among the 14 fvFTD cases, only five were demented according to DSM-III-R. Moderate to severe frontal atrophy was found in 93% of those with FLS (and in all cases with fvFTD), but also in 49% of those without FLS. FLS was found in 35% of those with moderate to severe frontal atrophy, and in 3% of those without these changes. Conclusions: The prevalence of fvFTD was 3% in 85 year olds, which is higher than previously expected in this age group. Only a minority of those with fvFTD were detected by the DSM-III-R criteria for dementia. FLS was even more common, especially in those diagnosed with a dementia disorder. PMID:12810769

  9. Physiological Expression of AMPKγ2RG Mutation Causes Wolff-Parkinson-White Syndrome and Induces Kidney Injury in Mice.

    PubMed

    Yang, Xiaodong; Mudgett, John; Bou-About, Ghina; Champy, Marie-France; Jacobs, Hugues; Monassier, Laurent; Pavlovic, Guillaume; Sorg, Tania; Herault, Yann; Petit-Demoulière, Benoit; Lu, Ku; Feng, Wen; Wang, Hongwu; Ma, Li-Jun; Askew, Roger; Erion, Mark D; Kelley, David E; Myers, Robert W; Li, Cai; Guan, Hong-Ping

    2016-11-04

    Mutations of the AMP-activated kinase gamma 2 subunit (AMPKγ2), N488I (AMPKγ2(NI)) and R531G (AMPKγ2(RG)), are associated with Wolff-Parkinson-White (WPW) syndrome, a cardiac disorder characterized by ventricular pre-excitation in humans. Cardiac-specific transgenic overexpression of human AMPKγ2(NI) or AMPKγ2(RG) leads to constitutive AMPK activation and the WPW phenotype in mice. However, overexpression of these mutant proteins also caused profound, non-physiological increase in cardiac glycogen, which might abnormally alter the true phenotype. To investigate whether physiological levels of AMPKγ2(NI) or AMPKγ2(RG) mutation cause WPW syndrome and metabolic changes in other organs, we generated two knock-in mouse lines on the C57BL/6N background harboring mutations of human AMPKγ2(NI) and AMPKγ2(RG), respectively. Similar to the reported phenotypes of mice overexpressing AMPKγ2(NI) or AMPKγ2(RG) in the heart, both lines developed WPW syndrome and cardiac hypertrophy; however, these effects were independent of cardiac glycogen accumulation. Compared with AMPKγ2(WT) mice, AMPKγ2(NI) and AMPKγ2(RG) mice exhibited reduced body weight, fat mass, and liver steatosis when fed with a high fat diet (HFD). Surprisingly, AMPKγ2(RG) but not AMPKγ2(NI) mice fed with an HFD exhibited severe kidney injury characterized by glycogen accumulation, inflammation, apoptosis, cyst formation, and impaired renal function. These results demonstrate that expression of AMPKγ2(NI) and AMPKγ2(RG) mutations at physiological levels can induce beneficial metabolic effects but that this is accompanied by WPW syndrome. Our data also reveal an unexpected effect of AMPKγ2(RG) in the kidney, linking lifelong constitutive activation of AMPK to a potential risk for kidney dysfunction in the context of an HFD. © 2016 by The American Society for Biochemistry and Molecular Biology, Inc.

  10. [Wolff-Parkinson-White syndrome with orthodromic supraventricular tachycardia associated with a "hyper-conductor" atrio-ventricular node. A therapeutic challenge].

    PubMed

    Hernández González, D; Iturralde Torres, P; Romero, L; Colín, L; Villarreal, A; González Hermosillo, J A

    1990-01-01

    One case of Wolff-Parkinson-White Syndrome with paroxysmal supraventricular tachycardia related to orthodromic atrioventricular reentry using an accessory pathway for retrograde conduction an a rapidly conducting AV node for anterograde conduction is present. The pharmacological therapy with Digoxin, Propranolol, Quinidine, Disopyramide and Propafenone was not effective. An electrophysiologic study showed a reciprocating tachycardia induced by spontaneous ventricular beats. Both the effective refractory period of the AV node and the anterograde effective refractory period of the accessory pathway were minor or equal to 220 msec which made the control of the arrhythmia difficult. Amiodarone was able to suppress the premature ventricular beats, depress conduction and prolong refractoriness in both, the AV node and accessory pathway to prevent recurrences of atrioventricular reentry. In this patient a false positive test with ajmaline was documented. The electrophysiologic study showed the association of Wolff-Parkinson-White Syndrome with an enhanced atrioventricular nodal-conduction and allowed the selection of an appropriate antiarrhythmic agent.

  11. Overexpression of G100S mutation in PRKAG2 causes Wolff-Parkinson-White syndrome in zebrafish.

    PubMed

    Zhang, B L; Ye, Z; Xu, R L; You, X H; Qin, Y W; Wu, H; Cao, J; Zhang, J L; Zheng, X; Zhao, X X

    2014-09-01

    The Wolff-Parkinson-White (WPW) syndrome was believed to be associated with PRKAG2 gene mutations. In this study, we verified the pathopoiesis of G100S mutation, a novel mutation only discovered in Chinese patients with WPW, in cardiac disorder. Similar to R302Q, when overexpressed PRKAG2 G100S mutant in zebrafish, we observed a thicker heart wall, detected a decreased AMPK enzymatic activity by tissue AMPK kinase activity colorimetric technique, as well as examined an increased glycogen storage in heart wall using the method for periodic acid-Schiff staining, in comparison with the zebrafish without exogenous PRKAG2 (mock) or with wild-type PRKAG2 (WT). Taken together, we concluded PRKAG2 G100S mutation might contribute to impair the AMP-activated protein kinase function, which resulted in increased cardiac glycogen storage, serving as a pathogenesis for WPW syndrome in Chinese. © 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  12. Parametric imaging of experimentally simulated Wolff-Parkinson-White syndrome conduction abnormalities in dogs: a concise communication

    SciTech Connect

    Weismueller, P.H.; Henze, E.; Adam, W.E.; Roth, J.; Bitter, F.; Stauch, M.

    1986-01-01

    In order to test the diagnostic potential of phase analysis of radionuclide ventriculography (RNV) for localizing accessory bundles in Wolff-Parkinson-White (WPW) syndrome, 24 experimental runs were performed in three open chest instrumented dogs. After a baseline study, WPW syndrome was simulated by stimulation at seven different sites around the base of the ventricles, and RNV's were obtained. Subsequent data processing including Fourier transformation allowed the localization of the site of the first inward motion of the ventricles by an isophasic wave display. In sinus rhythm, the septum contracted first. During ectopic premature ventricular stimulation by triggering the atrial signal, the phase scan was altered only when the stimulus was applied earlier than 20 ms before the expected QRS complex during sinus rhythm. During stimulation with fixed frequency, only the left lateral positions of the premature stimulation were detected by phase analysis with a sensitivity of 86%. Neither the antero- or posteroseptal nor the right ventricular premature contraction pattern could be exactly localized.

  13. [Wolff-Parkinson-White syndrome. Outcome of patients treated with anti-arrhythmia agents from data of electrophysiological examinations].

    PubMed

    Cointe, R; Lévy, S; Metge, M; Bru, P; Bricaud, H; Gérard, R

    1988-02-01

    Seventy-two consecutive patients with electrocardiographic evidence of Wolff-Parkinson-White syndrome underwent electrophysiological study (EPS). Fifty-five of these patients (76 p. 100) had episodes of tachycardia, 11 experienced palpitations or syncopes and 6 were asymptomatic. The decision to prescribe an antiarrhythmic agent was reached on the basis of the patients' symptoms and EPS data. One patient was treated by surgery before the medical treatment was tried; 17 patients were discharged without treatment, 4 were discharged with an episodic and 50 with a preventive antiarrhythmic treatment. Among these 50 patients, 46 (92 p. 100) could be followed up for a mean period of 45.7 +/- 28 months. One died of lung cancer; 43 presented with spontaneous episodes of tachycardia, 4 were able to discontinue treatment at the end of the follow-up period since they had very few symptoms and 2 were lost sight of. Among the 37 patients under antiarrhythmic treatment followed up, 29 (78 p. 100) are well controlled, while 8 (22 p. 100) still present with episodes of tachycardia. A tachycardia-reducing pacemaker was implanted in 5 of these 8 patients. It therefore appears that 78 p. 100 of patients presenting with spontaneous episodes of tachycardia associated with WPW syndrome can be controlled with an antiarrhythmic treatment. This result was obtained after trying at least two types of antiarrhythmic agents in 86 p. 100 of the cases.(ABSTRACT TRUNCATED AT 250 WORDS)

  14. [Difficulties of persistent repolarization following normalization of the QRS wave in the Wolff-Parkinson-White syndrome].

    PubMed

    Laham, J; Frank, R; Fontaine, G; Artigou, Y; Heller, J; Gerbaux, A; Grosgogeat, Y

    1982-06-01

    The normalisation of the ventricular complex in the Wolff-Parkinson-White syndrome is often accompanied by changes in the repolarisation phase with a deep, symmetric and pointed T wave suggestive of coronary artery disease. In order to study this phenomenon we examined 29 cases of intermittent WPW, 13 of which had abnormalities of the normalised complex. Normalisation occurred spontaneously on 10 occasions, twice on exercise and once after Ajmaline. In the majority of cases (9/13) the preexcitation was a right (4 cases) or left (5 cases) posterior pathway and the T waves were abnormal in the posterior leads (II, III and AVF). In left lateral preexcitation the T waves were negative in lead 1 and AVL. The T wave changes seem to be related to the topography of the preexcitation pathway. They gradually disappeared in the 3 cases in which preexcitation had not recurred. The age of the patients (11 to 45 years, average 31 years) or normal coronary angiography, performed in 3 cases, excluded coronary pathology as did the close relationship between the topography of the preexcitation and the T wave changes and the gradual disappearance of the abnormalities in the cases where preexcitation did not recur. This phenomenon, related to abnormal ventricular activation, seems to be comparable to the changes in ventricular repolarisation observed on termination of ventricular pacing, on the regression of certain intermittent left bundle branch blocks and perhaps, in some cases, of the post-ventricular tachycardia syndrome.

  15. Visual hallucinations in the differential diagnosis of parkinsonism

    PubMed Central

    Bertram, Kelly

    2012-01-01

    Visual hallucinations (VH) occur commonly in Parkinson's disease (PD) and dementia with Lewy bodies (DLB) but are reported much less frequently in other neurodegenerative causes of parkinsonism, such as progressive supranuclear palsy, multiple system atrophy and corticobasal degeneration syndrome. This clinical sign may be helpful when considering the differential diagnosis of patients with parkinsonism. The observation that VH may be specific to Lewy body pathology probably reflects a greater vulnerability of the visual systems to PD and DLB neurodegeneration compared with other diseases. Topographic differences in pathology are probably the major factor producing VH in Lewy body diseases, rather than neurophysiological changes that are specific to α-synuclein protein accumulation. VH correlate with pathology in the limbic system and more specifically the amygdale that is frequently affected in PD and DLB but relatively preserved in other forms of parkinsonism often misdiagnosed as PD. In this review, the published frequencies of VH in these different conditions are compared to put into context the notion of VH as a clinical clue to underlying Lewy body pathology. PMID:22228724

  16. [The current diagnostic approach for chronic progressive dementia].

    PubMed

    Bartels, C; Wallesch, C W

    2007-05-01

    This review presents diagnostic criteria for the different dementia syndromes and mild cognitive impairment. It is being claimed that in view of the current pharmacological interventions and after exclusion of symptomatic dementia, a controlled trial with cholinesterase inhibitors or memantine is more efficient than elaborate diagnostics. Efficiency of differential diagnosis will increase when drugs are available that specifically improve the different degenerative dementias and vascular dementia. Clinical pictures of the various dementia syndromes are briefly described.

  17. An Unusual Type of Localized Hypertrophic Cardiomyopathy With Wolf Parkinson White Syndrome Presenting With Pulmonary Edema

    PubMed Central

    Vatan, Mehmet Bulent; Gunduz, Huseyin; Gurel, Safiye; Kocayigit, Ibrahim; Vural, Ahmet; Demirtas, Saadet; Cakar, Mehmet Akif; Gunduz, Yasemin

    2012-01-01

    Hypertrophic cardiomyopathy (HCM) is an autosomal dominant heart disease that is the most common genetic cardiac disorder. The disease is characterized by excessive thickening of the left ventricular myocardium. The anterior portion of the interventricular ventricular septum is often involved. Asymmetric hypertrophy of apical site, left ventricular free wall, and right ventricle are less common in hypertrophic cardiomyopathy that occur in 1% cases. We report a case of a patient with an unusual type of hypertrophic cardiomyopathy and Wolf Parkinson White (WPW) presenting with pulmonary edema.

  18. The utility of cerebral blood flow imaging in patients with the unique syndrome of progressive dementia with motor neuron disease

    SciTech Connect

    Ohnishi, T.; Hoshi, H.; Jinnouchi, S.; Nagamachi, S.; Watanabe, K.; Mituyama, Y. )

    1990-05-01

    Two patients presenting with progressive dementia coupled with motor neuron disease underwent brain SPECT using N-isopropyl-p iodine-123-iodoamphetamine (({sup 123}I)IMP). The characteristic clinical features of progressive dementia and motor neuron disease were noted. IMP SPECT also revealed reduced uptake in the bilateral frontal and temporal regions, with no reduction of uptake in the parietal, parietal-occipital regions. We conclude that IMP SPECT has potential for the evaluation of progressive dementia with motor neuron disease.

  19. Contributions of the Instituto Nacional de Cardiología in the diagnosis and treatment of the Wolff-Parkinson - White syndrome.

    PubMed

    Iturralde-Torres, Pedro; Márquez, Manlio F

    2010-01-01

    Since the first description of the disease now known as Wolff-Parkinson-White syndrome, much knowledge has been gained through several experimental and clinical studies all over the world. The Instituto Nacional de Cardiología Ignacio Chávez in Mexico City has not been the exception. In this report, we describe the clinical, electrocardiographic and electrophysiologic contributions of past and present researchers at the Institute, as well as the experience in the diagnosis and treatment of the W-P-W syndrome at this Instituto Nacional de Cardiología Ignacio Chávez.

  20. [Wolff-Parkinson-White syndrome. Value of intravenous flecainide for detecting Kent's pathways with short refractory period].

    PubMed

    Talard, P; Cointe, R; Bru, P; Moyal, C; Lacombe, P; Bremondy, M; Levy, S; Gerard, R

    1990-04-01

    The aim of this study was to assess the value of a non-invasive test in detecting accessory pathways with short anterograde effective refractory periods (AERP) (less than or equal to 270 ms) in patients with the Wolff-Parkinson-White syndrome. An intravenous injection of Flecainide acetate was administered to 19 consecutive patients referred for electrophysiological investigation of a WPW syndrome with permanent pre-excitation of the surface electrocardiogram. The first 8 patients (Group I) received a dose of 1.5 mg/kg over 5 minutes and the following 11 patients (Group II) were given 2 mg/kg in 5 minutes. In Group I, preexcitation disappeared in 3 patients (37.5%) who all had accessory pathways with AERP greater than 270 ms. It persisted in the other 5 patients (62.5%) of whom 4 had AERP less than or equal to 270 ms and 1 an AERP greater than 270 ms (false negative). In Group II, preexcitation disappeared in 8 patients (72.2%) of whom 4 had AERP greater than 270 ms and 4 had AERP less than 270 ms (false positives). Preexcitation persisted in the 3 other patients (27.3%); the AERP was less than or equal to 270 ms in 2 patients and greater than 270 ms in the other patients. These results suggest that intravenous Flecainide acetate at the dose of 1.5 mg/kg could be useful in differentiating WPW syndromes with long refractory periods (greater than 270 ms) from those with short refractory periods (less than or equal to 270 ms) with a satisfactory sensitivity and specificity, and that further studies on larger numbers of patients are required to confirm this hypothesis.

  1. Weight Loss in Adults with Down Syndrome and with Dementia in Alzheimer's Disease

    ERIC Educational Resources Information Center

    Prasher, V. P.; Metseagharun, T.; Haque, S.

    2004-01-01

    An association between weight loss and Alzheimer's disease has been established in the general population but little information is available regarding this association in people with intellectual disabilities. A 4-year longitudinal study of adults with Down syndrome with and without Alzheimer's disease was undertaken. Age-associated weight loss…

  2. Weight Loss in Adults with Down Syndrome and with Dementia in Alzheimer's Disease

    ERIC Educational Resources Information Center

    Prasher, V. P.; Metseagharun, T.; Haque, S.

    2004-01-01

    An association between weight loss and Alzheimer's disease has been established in the general population but little information is available regarding this association in people with intellectual disabilities. A 4-year longitudinal study of adults with Down syndrome with and without Alzheimer's disease was undertaken. Age-associated weight loss…

  3. Ageing and Dementia in a Longitudinal Study of a Cohort with Down Syndrome

    ERIC Educational Resources Information Center

    Carr, Janet; Collins, Suzanne

    2014-01-01

    Background: A population sample of people with Down syndrome has been studied from infancy and has now been followed up again at age 47 years. Methods: Intelligence and language skills were tested and daily living skills assessed. Memory/cognitive deterioration was examined using two test instruments. Results: Scores on verbal tests of…

  4. Ageing and Dementia in a Longitudinal Study of a Cohort with Down Syndrome

    ERIC Educational Resources Information Center

    Carr, Janet; Collins, Suzanne

    2014-01-01

    Background: A population sample of people with Down syndrome has been studied from infancy and has now been followed up again at age 47 years. Methods: Intelligence and language skills were tested and daily living skills assessed. Memory/cognitive deterioration was examined using two test instruments. Results: Scores on verbal tests of…

  5. Chronic stress-like syndrome as a consequence of medial site subthalamic stimulation in Parkinson's disease.

    PubMed

    Růžička, Filip; Jech, Robert; Nováková, Lucie; Urgošík, Dušan; Bezdíček, Ondřej; Vymazal, Josef; Růžička, Evžen

    2015-02-01

    Considering the functional organization of the subthalamic nucleus (STN), we hypothesized that subthalamic deep brain stimulation (STN-DBS) in Parkinson's disease might have a differential impact on the hypothalamic-pituitary-adrenal axis in relation to the position of active stimulating contact within the STN. In addition, we searched for any STN-DBS-related morning plasma cortisol changes in association with postoperative anxiety and weight gain. A plasma cortisol measurement was performed on the day of initiation of bilateral STN-DBS and repeated after 1 and 17 months in twenty patients with advanced Parkinson's disease. The body weight change and anxiety scores following the implantation were assessed as well. The electrode positions in the STN were determined on T1-weighted magnetic resonance images. After initiation of stimulation, cortisol levels significantly decreased and the cortisol changes after 1 and 17 months strongly correlated with the position of active contact in the subthalamic area. Patients with at least one contact located more medially in the STN experienced a significantly greater decrease of cortisol than those with one or both active contacts more laterally. Furthermore, the lower cortisol levels were strongly associated with higher trait anxiety and weight gain. These changes mimicked the effects of chronic stress and suggest the disturbing impact of STN-DBS on limbic and motivational systems.

  6. James Parkinson: Parkinson's disease.

    PubMed

    Ellis, Harold

    2013-11-01

    Parkinson's disease is a condition that anyone with a modicum of medical knowledge can recognise in the street--as indeed how it was studied by James Parkinson himself. Its three characteristic features are: 1. Increase in the tone of the voluntary muscles (rigidity). 2. Slowness of movement (bradykinesis). 3. Tremor (the characteristic 'pill rolling' movements of the fingers).

  7. Serum NGAL is Associated with Distinct Plasma Amyloid-β Peptides According to the Clinical Diagnosis of Dementia in Down Syndrome.

    PubMed

    Naudé, Petrus J W; Dekker, Alain D; Coppus, Antonia M W; Vermeiren, Yannick; Eisel, Ulrich L M; van Duijn, Cornelia M; Van Dam, Debby; De Deyn, Peter P

    2015-01-01

    The majority of people with Down syndrome (DS) develop dementia due to Alzheimer's disease (AD). Neuropathological features are characterized by an accumulation of amyloid-β (Aβ) deposits and the presence of an activated immune response. Neutrophil Gelatinase-Associated Lipocalin (NGAL) is a newly identified (neuro)inflammatory constituent in AD. This study examines NGAL as an inflammatory marker in DS and its associations with plasma Aβ peptides according to the follow-up clinical diagnosis of dementia. Baseline serum NGAL and plasma Aβ40, Aβ42, Aβ(n40), and Aβ(n42) were quantified in 204 people with DS. The diagnosis of dementia in DS was established by follow-up clinical assessments. The following study groups were characterized: DS with AD at baseline (n = 67), DS without AD (n = 53), and non-demented DS individuals that converted to AD (n = 84). Serum NGAL was analyzed in 55 elderly non-DS, non-demented people. Serum NGAL levels were significantly increased in DS subjects compared to non-DS people. Serum NGAL levels were not associated with clinical dementia symptoms in DS. However, NGAL was positively associated with Aβ42 and Aβ(n42) in demented DS individuals and with Aβ40 and Aβ(n40) in the non-demented DS group. NGAL was negatively associated with Aβ42/Aβ40 and Aβ(n42)/Aβ(n40) ratios in converted DS subjects. These associations persisted for Aβ(n40), Aβ42/Aβ40, and Aβ(n42)/Aβ(n40) after adjusting for demographics measures, apolipoprotein E ε4 allele, platelets, and anti-inflammatory medication. Serum NGAL levels are increased in DS and associated with distinct species of Aβ depending on the progression of dementia as diagnosed by baseline and follow-up clinical assessments.

  8. Phenotypic Heterogeneity of Monogenic Frontotemporal Dementia

    PubMed Central

    Benussi, Alberto; Padovani, Alessandro; Borroni, Barbara

    2015-01-01

    Frontotemporal dementia (FTD) is a genetically and pathologically heterogeneous disorder characterized by personality changes, language impairment, and deficits of executive functions associated with frontal and temporal lobe degeneration. Different phenotypes have been defined on the basis of presenting clinical symptoms, i.e., the behavioral variant of FTD, the agrammatic variant of primary progressive aphasia, and the semantic variant of PPA. Some patients have an associated movement disorder, either parkinsonism, as in progressive supranuclear palsy and corticobasal syndrome, or motor neuron disease (FTD–MND). A family history of dementia is found in 40% of cases of FTD and about 10% have a clear autosomal-dominant inheritance. Genetic studies have identified several genes associated with monogenic FTD: microtubule-associated protein tau, progranulin, TAR DNA-binding protein 43, valosin-containing protein, charged multivesicular body protein 2B, fused in sarcoma, and the hexanucleotide repeat expansion in intron 1 of the chromosome 9 open reading frame 72. Patients often present with an extensive phenotypic variability, even among different members of the same kindred carrying an identical disease mutation. The objective of the present work is to review and evaluate available literature data in order to highlight recent advances in clinical, biological, and neuroimaging features of monogenic frontotemporal lobar degeneration and try to identify different mechanisms underlying the extreme phenotypic heterogeneity that characterizes this disease. PMID:26388768

  9. Young-Onset Dementia

    PubMed Central

    Kuruppu, Dulanji K; Matthews, Brandy R

    2014-01-01

    Young-onset dementia (YOD) is an neurological syndrome that affects behavior and cognition of patients younger than 65 years of age. Although frequently misdiagnosed, a systematic approach, reliant upon attainment of detailed medical history, collateral history from an informant, neuropsychological testing, laboratory studies, and neuroimaging, may facilitate earlier and more accurate diagnosis with subsequent intervention. The differential diagnosis of YOD is extensive and includes early-onset forms of adult neurodegenerative conditions including Alzheimer's disease, vascular dementia, frontotemporal dementia, Lewy body dementias, Huntington's disease, and prion disease. Late-onset forms of childhood neurodegenerative conditions may also present as YOD and include mitochondrial disorders, lysosomal storage disorders, and leukodystrophies. Potentially reversible etiologies including inflammatory disorders, infectious diseases, toxic/metabolic abnormalities, transient epileptic amnesia, obstructive sleep apnea, and normal pressure hydrocephalus also represent important differential diagnostic considerations in YOD. This review will present etiologies, diagnostic strategies, and options for management of YOD with comprehensive summary tables for clinical reference. PMID:24234358

  10. Biochemical evaluations in skeletal muscles of primates with MPTP Parkinson-like syndrome.

    PubMed

    Pastoris, O; Dossena, M; Foppa, P; Catapano, M; Ferrari, R; Dagani, F

    1995-06-01

    The toxic effects of the neurotoxin MPTP (1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine) in primates can be exploited for investigating the physiopathology of Parkinson's disease which may also cause functional alterations of skeletal muscles, whose biochemical modifications have been studied very little. Some enzyme activities related to energy transduction in skeletal muscles were evaluated (gastrocnemius, soleus and biceps) from MPTP-treated monkeys. Systemically administered MPTP altered the enzyme activities related to: (i) the anaerobic glycolytic pathway (decrease in hexokinase and phosphofructokinase activities; increase in lactate dehydrogenase activity); (ii) the tricarboxylic acid cycle (decrease in malate dehydrogenase activity); (iii) the electron transfer chain (decrease in cytochrome oxidase activity related to complex IV). No alteration in mitochondrial Complex I was observed. Treatment with an ergot alkaloid derivative (dihydroergocryptine) modified some alterations in the muscle enzyme activities and reduced the rigidity and some autonomic dysfunction.

  11. Retention of the cyanobacterial neurotoxin beta-N-methylamino-l-alanine in melanin and neuromelanin-containing cells--a possible link between Parkinson-dementia complex and pigmentary retinopathy.

    PubMed

    Karlsson, Oskar; Berg, Cecilia; Brittebo, Eva B; Lindquist, Nils Gunnar

    2009-02-01

    beta-N-methylamino-l-alanine (BMAA), a neurotoxic amino acid produced by cyanobacteria, has been suggested to be involved in the etiology of a neurodegenerative disease complex which includes Parkinson-dementia complex (PDC). In PDC, neuromelanin-containing neurons in substantia nigra are degenerated. Many PDC patients also have an uncommon pigmentary retinopathy. The aim of this study was to investigate the distribution of (3)H-BMAA in mice and frogs, with emphasis on pigment-containing tissues. Using autoradiography, a distinct retention of (3)H-BMAA was observed in melanin-containing tissues such as the eye and neuromelanin-containing neurons in frog brain. Analysis of the binding of (3)H-BMAA to Sepia melanin in vitro demonstrated two apparent binding sites. In vitro-studies with synthetic melanin revealed a stronger interaction of (3)H-BMAA with melanin during synthesis than the binding to preformed melanin. Long-term exposure to BMAA may lead to bioaccumulation in melanin- and neuromelanin-containing cells causing high intracellular levels, and potentially changed melanin characteristics via incorporation of BMAA into the melanin polymer. Interaction of BMAA with melanin may be a possible link between PDC and pigmentary retinopathy.

  12. A novel cholinesterase and brain-selective monoamine oxidase inhibitor for the treatment of dementia comorbid with depression and Parkinson's disease.

    PubMed

    Weinstock, Marta; Gorodetsky, Elena; Poltyrev, Tatyana; Gross, Aviva; Sagi, Yotam; Youdim, Moussa

    2003-06-01

    Degeneration of cholinergic cortical neurons is one of the main reasons for the cognitive deficit in dementia of the Alzheimer type (AD) and in dementia with Lewy bodies (DLB). Many subjects with AD and DLB have extrapyramidal dysfunction and depression resulting from degeneration of dopaminergic, noradrenergic and serotoninergic neurons. We prepared a novel drug, TV-3326 (N-propargyl-3R-aminoindan-5yl)-ethyl methylcarbamate), with both cholinesterase (ChE) and monoamine oxidase (MAO) inhibitory activity, as potential treatment of AD and DLB. TV-3326 inhibits brain acetyl and butyrylcholinesterase (BuChE) in rats after oral doses of 10-100 mg/kg. After chronic but not acute treatment, it inhibits MAO-A and -B in the brain by more than 70% but has almost no effect on these enzymes in the small intestine in rats and rabbits. The brain selectivity results in minimal potentiation of the pressor response to oral tyramine. TV-3326 acts like other antidepressants in the forced swim test in rats, indicating a potential for antidepressant activity. Chronic treatment of mice with TV-3326 (26 mg/kg) prevents the destruction of nigrostriatal neurons by the neurotoxin MPTP (N-methyl-4-phenyl-1,2,3,6-tetrahydropyridine). In addition to ChE and MAO inhibition, the propargylamine moiety of TV-3326 confers neuroprotective activity against cytotoxicity induced by ischemia and peroxynitrite in cultured neuronal cells that results from prevention of the fall in mitochondrial membrane potential and antiapoptotic activity. These unique multiple actions of TV-3326 make it a potentially useful drug for the treatment of dementia with Parkinsonian-like symptoms and depression.

  13. [Influence of exercise on the permeability of accessory pathways and supraventricular arrhythmia in Wolff-Parkinson-White syndrome].

    PubMed

    Mabo, P; Kermarrec, A; Gras, D; Paillard, F; Bédossa, M; Daubert, C

    1992-10-01

    The influence of adrenergic stimulation on the effective anterograde refractory period of the accessory pathways and on supraventricular arrhythmias, was studied in 20 patients (average age 38 +/- 16 years) with an untreated permanent Wolff-Parkinson-White syndrome and a resting anterograde refractory period < or = 400ms. Repeated electrophysiological studies with a single endocavity catheter positioned near the atrial pole of the accessory pathway were performed under basal conditions and during a standardised exercise test on a bicycle ergometer. The effective anterograde refractory period of the accessory pathway, the length of the tachycardia cycle during reciprocating orthodromic tachycardia, the average heart rate, the percentage of preexcited QRS complexes during induced atrial fibrillation, were measured in all patients under basal conditions and at the peak of exercise. Exercise significantly reduced the anterograde refractory period of the accessory pathway (287 +/- 49 ms at rest versus 238 +/- 24 ms on exercise: p < 0.001), the cycle of orthodromic tachycardia (302 +/- 32 vs 260 +/- 22 ms p < 0.001), the minimal R-R interval (270 +/- 65 vs 227 +/- 46 ms: p < 0.05) and % of preexcited QRS complexes (75 +/- 33 vs 51 +/- 39: p < 0.05) in atrial fibrillation whilst increasing the average heart rate (165 +/- 42 vs 202 +/- 39 bpm: p < 0.02). Adrenergic stimulation significantly improves anterograde conduction in the accessory pathway. The reduction in the % of preexcited QRS complexes in atrial fibrillation could indicate a preferential action of catecholamines on the nodo-hisian pathway.(ABSTRACT TRUNCATED AT 250 WORDS)

  14. [Influence of age on the presumed cause of syncope in patients with the Wolff-Parkinson-White syndrome].

    PubMed

    Chometon, F; Brembilla-Perrot, B

    2007-01-01

    The aim of this study was to assess the causes of syncope in patients with the Wolff-Parkinson-White syndrome (WPW) and to determine whether the age of the patients was a significant factor. Forty-seven patients with a WPW, aged 11 to 72 years, underwent electrophysiological study by the oesophageal approach because of an unexplained syncope. Nineteen patients were under 20 years of age (16 +/- 3 years: group I) and 28 were over 20 years of age (40 +/- 13 years: group II). Junctional tachycardia was induced in 8 patients of group I (42%) and in 13 of group II (46%) (NS); atrial fibrillation was induced in 8 patients of group I (42%) and in 9 of group II (35%) (NS). A potentially malignant form of WPW was identified in 8 patients of group I (42%) and in 11 of group II (39%) (NS); Syncope was directly attributed to the WPW in 14 patients of group I (74%) and in 19 of group II (78%), either after identification of a serious form or induction of junctional tachycardia (6 patients of group I and 8 of group II). The rest of the syncopal episodes had various causes. There were no deaths. The authors conclude that oesophageal electrophysiological investigations enable rapid identification of a high incidence of tachycardias probably responsible for syncope in WPW. The causes of syncope and incidence of potentially severe forms of WPW were not significantly influenced by the age of the patients.

  15. Characterizing restless legs syndrome and leg motor restlessness in patients with Parkinson's disease: A multicenter case-controlled study.

    PubMed

    Suzuki, Keisuke; Okuma, Yasuyuki; Uchiyama, Tomoyuki; Miyamoto, Masayuki; Sakakibara, Ryuji; Shimo, Yasushi; Hattori, Nobutaka; Kuwabara, Satoshi; Yamamoto, Toshimasa; Kaji, Yoshiaki; Hirano, Shigeki; Numao, Ayaka; Hirata, Koichi

    2017-08-14

    We investigated the prevalence and impact of restless legs syndrome (RLS) and leg motor restlessness (LMR) in patients with Parkinson's disease (PD) in a multicenter study. A total of 436 PD patients and 401 age- and sex-matched controls were included in this study. RLS was diagnosed based on four essential features. LMR was diagnosed when a participant exhibited the urge to move his or her legs but did not meet the four essential features of RLS. The RLS prevalence did not differ between PD patients and controls (3.4% vs. 2.7%), while LMR prevalence was significantly higher in PD patients than in controls (12.8% vs. 4.5%). PD patients with RLS or LMR had a higher prevalence of excessive daytime sleepiness (EDS) (50.7%, vs. 6.9%), probable REM sleep behavior disorder (38.0% vs. 3.4%) and PD-related sleep problems (49.3% vs. 20.7%) than controls with RLS or LMR. RLS/LMR preceding PD onset was related to an older age of PD onset. Our study revealed an increased prevalence of LMR but not RLS in PD patients. LMR could be an early manifestation of PD; however, whether LMR is within the range of RLS or whether LMR and RLS constitute different entities in PD requires further studies. Copyright © 2017 Elsevier Ltd. All rights reserved.

  16. [An electrophysiologic and electropharmacological study of functional properties of the bundle of Kent in Wolff-Parkinson-White syndrome].

    PubMed

    Costantini, M; Chimienti, M; Zardini, M; Klersy, C; Guasti, L; Salerno, J A

    1989-04-01

    The aim of this report is to attempt a definition of functional properties of Kent bundle on the basis of electrophysiologic and electropharmacologic data obtained from 89 cases of Wolff-Parkinson-White syndrome selected among a total number of 114 consecutive cases of WPW syndrome that underwent electrophysiologic intracavitary study. In 36 cases anterograde (ant) and retrograde (retr) effective refractory period (ERP) of accessory pathway were evaluated with premature (atrial and ventricular) stimulation at the same driven cycle length. The ant-ERP was longer than retr-ERP in 28/36 patients, shorter in 5 and equal in 2. This strong discrepancy between ant- and retr- ERP suggests an important role of "impedance mismatch" in the activation of ventricular (or atrial) muscle through an anomalous muscular bundle. In 11 cases an intermittent pattern of ventricular preexcitation was observed; in all these patients an anterograde supernormal conduction through the accessory pathway was observed. This aspect could be related to the activation of ventricular muscle, beyond Kent bundle, in its supernormal phase of excitability, suggesting the critical role played by ventricular activation for the appearance of preexcitation. Isoproterenol, injected in 11 cases (1 among them with intermittent ventricular preexcitation in basal conditions), produced a reduction of ant-ERP in all these cases, in spite of its well known poor effect on refractoriness of myocardial fibers. Ajmaline, injected in 32 patients, was able to block ventricular preexcitation in 81% of the cases, in spite of its poor effect on refractoriness of normal tissues. It is very likely that the disappearance of ventricular preexcitation is in this instance expression of lack of ventricular excitation (distal to Kent bundle) consequent to a drug-induced reduction of membrane responsiveness of ventricular cells. In conclusion, all these aspects strongly suggest that the appearance of ventricular (or atrial

  17. mTOR in Down syndrome: Role in Aß and tau neuropathology and transition to Alzheimer disease-like dementia.

    PubMed

    Di Domenico, Fabio; Tramutola, Antonella; Foppoli, Cesira; Head, Elizabeth; Perluigi, Marzia; Butterfield, D Allan

    2017-08-12

    The mammalian target of rapamycin (mTOR) is a serine/threonine protein kinase involved in the regulation of protein synthesis and degradation, longevity and cytoskeletal formation. The mTOR pathway represents a key growth and survival pathway involved in several diseases such as cancer, obesity, cardiovascular disease and neurodegenerative diseases. Numerous studies linked the alterations of mTOR pathway to age-dependent cognitive decline, pathogenesis of Alzheimer disease (AD) and AD-like dementia in Down syndrome (DS). DS is the most frequent chromosomal abnormality that causes intellectual disability. The neuropathology of AD in DS is complex and involves impaired mitochondrial function, defects in neurogenesis, increased oxidative stress, altered proteostasis and autophagy networks as a result of triplication of chromosome 21(chr 21). The chr21 gene products are considered a principal neuropathogenic moiety in DS. Several genes involved respectively in the formation of senile plaques and neurofibrillary tangles (NFT), two main pathological hallmarks of AD, are mapped on chr21. Further, in subjects with DS the activation of mTOR signaling contributes to Aβ generation and the formation of NFT. This review discusses recent research highlighting the complex role of mTOR associated with the presence of two hallmarks of AD pathology, senile plaques (composed mostly of fibrillar Aß peptides), and NFT (composed mostly of hyperphosphorylated tau protein). Oxidative stress, associated with chr21-related Aβ and mitochondrial alterations, may significantly contribute to this linkage of mTOR to AD-like neuropathology in DS. Copyright © 2017. Published by Elsevier Inc.

  18. Music perception in dementia

    PubMed Central

    Nicholas, Jennifer M; Cohen, Miriam H; Slattery, Catherine F; Paterson, Ross W; Foulkes, Alexander J M; Schott, Jonathan M; Mummery, Catherine J; Crutch, Sebastian J; Warren, Jason D

    2017-01-01

    Despite much recent interest in music and dementia, music perception has not been widely studied across dementia syndromes using an information processing approach. Here we addressed this issue in a cohort of 30 patients representing major dementia syndromes of typical Alzheimer’s disease (AD, n=16), logopenic aphasia (LPA, an Alzheimer variant syndrome; n=5) and progressive nonfluent aphasia (PNFA; n=9) in relation to 19 healthy age-matched individuals. We designed a novel neuropsychological battery to assess perception of musical patterns in the dimensions of pitch and temporal information (requiring detection of notes that deviated from the established pattern based on local or global sequence features) and musical scene analysis (requiring detection of a familiar tune within polyphonic harmony). Performance on these tests was referenced to generic auditory (timbral) deviance detection and recognition of familiar tunes and adjusted for general auditory working memory performance. Relative to healthy controls, patients with AD and LPA had group-level deficits of global pitch (melody contour) processing while patients with PNFA as a group had deficits of local (interval) as well as global pitch processing. There was substantial individual variation within syndromic groups. No specific deficits of musical temporal processing, timbre processing, musical scene analysis or tune recognition were identified. The findings suggest that particular aspects of music perception such as pitch pattern analysis may open a window on the processing of information streams in major dementia syndromes. The potential selectivity of musical deficits for particular dementia syndromes and particular dimensions of processing warrants further systematic investigation. PMID:27802226

  19. Music Perception in Dementia.

    PubMed

    Golden, Hannah L; Clark, Camilla N; Nicholas, Jennifer M; Cohen, Miriam H; Slattery, Catherine F; Paterson, Ross W; Foulkes, Alexander J M; Schott, Jonathan M; Mummery, Catherine J; Crutch, Sebastian J; Warren, Jason D

    2017-01-01

    Despite much recent interest in music and dementia, music perception has not been widely studied across dementia syndromes using an information processing approach. Here we addressed this issue in a cohort of 30 patients representing major dementia syndromes of typical Alzheimer's disease (AD, n = 16), logopenic aphasia (LPA, an Alzheimer variant syndrome; n = 5), and progressive nonfluent aphasia (PNFA; n = 9) in relation to 19 healthy age-matched individuals. We designed a novel neuropsychological battery to assess perception of musical patterns in the dimensions of pitch and temporal information (requiring detection of notes that deviated from the established pattern based on local or global sequence features) and musical scene analysis (requiring detection of a familiar tune within polyphonic harmony). Performance on these tests was referenced to generic auditory (timbral) deviance detection and recognition of familiar tunes and adjusted for general auditory working memory performance. Relative to healthy controls, patients with AD and LPA had group-level deficits of global pitch (melody contour) processing while patients with PNFA as a group had deficits of local (interval) as well as global pitch processing. There was substantial individual variation within syndromic groups. Taking working memory performance into account, no specific deficits of musical temporal processing, timbre processing, musical scene analysis, or tune recognition were identified. The findings suggest that particular aspects of music perception such as pitch pattern analysis may open a window on the processing of informa