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Sample records for parkinson dementia syndromes

  1. Imaging Amyloidopathy in Parkinson Disease and Parkinsonian Dementia Syndromes

    PubMed Central

    Frey, Kirk A.; Petrou, Myria

    2015-01-01

    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. 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. PMID:27502301

  3. 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

  4. Parkinson's Disease Dementia

    MedlinePlus

    ... Is Dementia Types of Dementia Chronic Traumatic Encephalopathy (CTE) Creutzfeldt-Jakob Disease Dementia with Lewy Bodies Down ... Research Traumatic Brain Injury and Chronic Traumatic Encephalopathy (CTE) Awardees Year Researcher Study Name 2015 Jesse Mez ...

  5. Cognitive dysfunction and dementia in Parkinson disease.

    PubMed

    Caballol, Nuria; Martí, Maria J; Tolosa, Eduardo

    2007-09-01

    Impairment in different cognitive domains such as executive functions, language, memory, and visuospatial skills occurs frequently in Parkinson disease (PD) even in the early stages of the disease. Although frank dementia (Parkinson disease dementia, PDD) is less frequent, risk for developing dementia is two to six times greater than the prevalence rate in general population and it increases in relation to disease duration. Clinically, dementia in PD is characterized by uninsidious onset and slowly progressive cognitive decline, with a predominant dysexecutive syndrome accompanied frequently by a variety of behavioral symptoms such as hallucinations, depression, anxiety, and excessive daytime sleepiness. Although the exact pathophysiology and neurobiological basis of PDD is not known, dementia in PD probably develops as a result of progressive involvement of subcortical and cortical structures by Lewy-type pathology and associated Alzheimer-like histological changes. Dysfunction of different monoamine transmitter has also been implicated in the cognitive deterioration of PD but reduced cholinergic activity in the cortex is thought to account for the strongest mechanism in the development of dementia. Recent evidence suggests that cholinesterase inhibitors are effective in the treatment of dementia and accompanying behavioral symptoms in PD. PMID:18175397

  6. Parkinsonism and frontotemporal dementia: the clinical overlap.

    PubMed

    Espay, Alberto J; Litvan, Irene

    2011-11-01

    Frontotemporal dementia is commonly associated with parkinsonism in several sporadic (i.e., progressive supranuclear palsy, corticobasal degeneration) and familial neurodegenerative disorders (i.e., frontotemporal dementia associated with parkinsonism and MAPT or progranulin mutations in chromosome 17). The clinical diagnosis of these disorders may be challenging in view of overlapping clinical features, particularly in speech, language, and behavior. The motor and cognitive phenotypes can be viewed within a spectrum of clinical, pathologic, and genetic disorders with no discrete clinicopathologic correlations but rather lying within a dementia-parkinsonism continuum. Neuroimaging and cerebrospinal fluid analysis can be helpful, but the poor specificity of clinical and imaging features has enormously challenged the development of biological markers that could differentiate these disorders premortem. This gap is critical to bridge in order to allow testing of novel biological therapies that may slow the progression of these proteinopathies. PMID:21892619

  7. Wolff-Parkinson-White syndrome

    MedlinePlus

    Wolff-Parkinson-White syndrome is a condition in which there is an extra electrical pathway of the heart. The ... to periods of rapid heart rate ( tachycardia ). Wolff-Parkinson-White syndrome is one of the most common ...

  8. Dementia in Down's syndrome.

    PubMed

    Ballard, Clive; Mobley, William; Hardy, John; Williams, Gareth; Corbett, Anne

    2016-05-01

    Down's syndrome is the most common genetic cause of learning difficulties, and individuals with this condition represent the largest group of people with dementia under the age of 50 years. Genetic drivers result in a high frequency of Alzheimer's pathology in these individuals, evident from neuroimaging, biomarker, and neuropathological findings, and a high incidence of cognitive decline and dementia. However, cognitive assessment is challenging, and diagnostic methods have not been fully validated for use in these patients; hence, early diagnosis remains difficult. Evidence regarding the benefits of cholinesterase inhibitors and other therapeutic options to treat or delay progressive cognitive decline or dementia is very scarce. Despite close similarities with late-onset Alzheimer's disease, individuals with Down's syndrome respond differently to treatment, and a targeted approach to drug development is thus necessary. Genetic and preclinical studies offer opportunities for treatment development, and potential therapies have been identified using these approaches. PMID:27302127

  9. Adult-onset phenylketonuria with rapidly progressive dementia and parkinsonism.

    PubMed

    Tufekcioglu, Zeynep; Cakar, Arman; Bilgic, Basar; Hanagasi, Hasmet; Gurvit, Hakan; Emre, Murat

    2016-06-01

    Phenylketonuria (PKU) is an autosomal recessive metabolic disorder due to mutations in the phenylalanine hydroxylase (PAH) gene, which converts phenylalanine (PHE) to tyrosine. Although it is principally a childhood disorder, in rare cases, the first signs of PKU may develop in late adulthood resembling common neurological diseases. Here we report a 59-year-old, previously normal functioning man who was admitted with blurred vision, cognitive problems, and gait difficulty that began 8 months before. He had brisk reflexes and left side dominant parkinsonism. His Mini-Mental State Examination (MMSE) score was 25/30, and neuropsychological evaluation revealed a dysexecutive syndrome with simultanagnosia and constructional apraxia. His Clinical Dementia Rating score (CDR) was 1. Cranial MRI revealed bilateral diffuse hyperintense lesions in parietal and occipital white matter in T2, fluid-attenuated inversion recovery, and diffusion weighted images. Diagnostic workup for rapidly progressive dementias was all normal except PHE level which was found to be highly elevated (1075 μmol/L, normal 39-240 μmol/L) with normal tyrosine level (61.20 μmol/L, normal 35-100 μmol/L). Three months after PHE-restricted diet, his cognitive impairment and signs of parkinsonism significantly improved, with MRI scan unchanged. This case demonstrates that late-onset PKU is a rare, treatable cause of rapidly progressive dementia and parkinsonism with certain constellations such as consanguinity and white matter abnormalities (WMAs) in imaging. PMID:26962957

  10. Aluminium and calcium in soil and food from Guam, Palau and Jamaica: Implications for amyotrophic lateral sclerosis and parkinsonism-dementia syndromes of Guam.

    PubMed

    Crapper McLachlarf, D R; McLachlan, C D; Krishnan, B; Krishnan, S S; Dalton, A J; Steele, J C

    1989-06-01

    Low calcium and high aluminium concentrations in the soils, waters and native foods have been hypothesised as environmental factors contributing to the unusually high incidence of amyotrophic lateral sclerosis and parkinsonism with dementia (ALS-PD) found on the island of Guam. The amounts of elemental aluminium and calcium were measured in foods of the native diet of the Chamorro people of Guam. The amount of aluminium eluted from topsoil by water at pH 7 at 22 °C was also measured. For comparison, food, water and soil samples were collected from two islands which have not reported a high incidence of ALS-PD syndromes: Palau and Jamaica.Compared with agricultural soils of Jamaica or Palau, the agricultural soils of Guam averaged 42-fold higher yield of elutable aluminium. The food data, however, do not indicate a differentially high exposure to elemental aluminium or low calcium intake in the diet of any one population. While this study did not detect an unusually high dietary aluminium or low dietary calcium content, the soils and possibly the dusts of Guam may be a major source of aluminium entering the body of the native people, particularly through the respiratory epithelium. Since iipid soluble organic ligands of aluminium more readily penetrate epithelial membranes, further study of soil aluminium ligands is required. PMID:24202289

  11. Diagnosis and management of Parkinson's disease dementia

    PubMed Central

    Poewe, W; Gauthier, S; Aarsland, D; Leverenz, J B; Barone, P; Weintraub, D; Tolosa, E; Dubois, B

    2008-01-01

    Parkinson's disease (PD) has long been considered predominantly a motor disorder. However, its frequent association with dementia, which contributes significantly to the morbidity and mortality of the condition, is gaining increasing recognition. PD dementia (PDD) has a unique clinical profile and neuropathology, distinct from Alzheimer's disease (AD). Cholinergic deficits, a feature of both AD and PDD, underlie the rationale for cholinesterase inhibitor therapy in both conditions. In clinical practice, it is important that PDD should be recognised and appropriately treated. This review aims to outline the recently proposed clinical diagnostic criteria for PDD and to summarise the guidelines/recommendations published since 2006 on the use of cholinesterase inhibitors in the management of PDD. Although the cholinesterase inhibitor rivastigmine has recently been approved for the management of PDD, there remains a need for the development of novel therapies that can affect key mechanisms of the disease or prevent/delay patients with PD and mild cognitive impairment from progressing to PDD. PMID:18822028

  12. Clinical aspects of familial forms of frontotemporal dementia associated with parkinsonism.

    PubMed

    Fujioka, Shinsuke; Wszolek, Zbigniew K

    2011-11-01

    Frontotemporal dementia is the second most common dementia among people under the age of 65. Fifty percent of affected patients have an associated family history. Several pathogenic genes have been identified for frontotemporal dementia associated with parkinsonism, including microtubule-associated protein tau, progranulin, and chromatin modifying protein 2B, and fused in sarcoma. It has also been reported that frontotemporal dementia associated with parkinsonism can be linked to chromosome 9p. In addition, there are families with frontotemporal dementia associated with a parkinsonian phenotype but unknown genetic status. Some of these kindreds have been diagnosed clinically as familial progressive supranuclear palsy, hereditary diffuse leukoencephalopathy with axonal spheroids, "overlap" syndrome, and others. Clinical presentation of frontotemporal dementia associated with parkinsonism is variable at age of symptomatic disease onset, disease duration, symptoms, and their occurrence during the disease course. Clinically, it is often difficult to sort out the different genetic forms of frontotemporal dementia associated with parkinsonism. However, with available clinical genetic testing for known genes, the precise diagnosis can be accomplished in some cases. PMID:21656039

  13. [Paraneoplastic Neurological Syndrome with Dementia].

    PubMed

    Tanaka, Keiko

    2016-04-01

    Paraneoplastic neurological syndrome with limbic encephalopathy tends to progress rapidly, presenting with physical symptoms such as ataxia or sensory disturbance. However, some affected patients demonstrate amnesia, inactivity, or abnormal behavior, which lead to the diagnosis of dementia. It is important to perform an extensive differential diagnosis with autoantibody-examination and tumor survey, so as not to overlook potentially treatable dementia. PMID:27056857

  14. Rivastigmine: new indication. Dementia and Parkinson's disease: no thank you!

    PubMed

    2007-04-01

    In patients with both Parkinson's disease and dementia, a placebo-controlled trial has confirmed that the adverse effects of rivastigmine outweigh its benefits. It is better to carefully adjust ongoing antiparkinsonian treatments than to add an anticholinesterase. PMID:17458048

  15. 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. PMID:27193487

  16. 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

  17. Wolff-Parkinson-White syndrome.

    PubMed

    Morscher, J H

    1992-02-01

    Wolff-Parkinson-White (WPW) syndrome is a cardiac conduction disorder that presents with potentially life-threatening consequences. Wolff-Parkinson-White syndrome-induced dysrhythmias account for 20% of all supraventricular tachycardias that occur in the general population. Clinical presentations range from no symptoms to a sudden cardiac arrest. The risk of sudden death is always present with WPW syndrome, and it is the motivating force in the evaluation and treatment of this syndrome. Current diagnostic modalities are accurate in identifying patients with WPW syndrome, but lack the sensitivity to predict sudden cardiac death. This article reviews the history of WPW syndrome, as well as its general characteristics, diagnostic criteria, treatment modalities, and nursing implications. PMID:1554559

  18. [Treatment for dementia in parkinsonian syndromes. Efficacy of cholinesterase inhibitors].

    PubMed

    Liepelt, I; Maetzler, W; Blaicher, H-P; Gasser, T; Berg, D

    2008-01-01

    In parkinsonian syndromes dementia frequently occurs in the disease progress. The cholinergic system has been proposed as playing a key role in cognitive disturbances. Therefore the application of cholinesterase inhibitors (ChEI) is also hotly argued for dementia associated with parkinsonian syndromes. This review focuses on the specific symptoms of dementia in Parkinson's disease (PDD), dementia with Lewy bodies (DLB), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD). The effect of cholinergic treatment on cognition and behaviour is reported and critically discussed. There is evidence that medication with some ChEIs reduces cognitive disturbances and to a lesser extent improves activities of daily living in PDD. Behavioural symptoms also seem to be positively influenced by treatment with ChEIs in both PDD and DLB. The effect of treatment with cholinesterase inhibitors in PSP and CBD warrants more carefully designed studies including sufficient numbers of patients. PMID:17687535

  19. Dementia syndromes: evaluation and treatment

    PubMed Central

    Scott, Kevin R; Barrett, Anna M

    2008-01-01

    As our population ages, diseases affecting memory and daily functioning will affect an increasing number of individuals, their families and the healthcare system. The social, financial and economic impacts will be profound. This article provides an overview of current dementia syndromes to assist clinicians in evaluating, educating and treating these patients. PMID:17425495

  20. COTARD SYNDROME IN SEMANTIC DEMENTIA

    PubMed Central

    Mendez, Mario F.; Ramírez-Bermúdez, Jesús

    2011-01-01

    Background Semantic dementia is a neurodegenerative disorder characterized by the loss of meaning of words or concepts. semantic dementia can offer potential insights into the mechanisms of content-specific delusions. Objective The authors present a rare case of semantic dementia with Cotard syndrome, a delusion characterized by nihilism or self-negation. Method The semantic deficits and other features of semantic dementia were evaluated in relation to the patient's Cotard syndrome. Results Mrs. A developed the delusional belief that she was wasting and dying. This occurred after she lost knowledge for her somatic discomforts and sensations and for the organs that were the source of these sensations. Her nihilistic beliefs appeared to emerge from her misunderstanding of her somatic sensations. Conclusion This unique patient suggests that a mechanism for Cotard syndrome is difficulty interpreting the nature and source of internal pains and sensations. We propose that loss of semantic knowledge about one's own body may lead to the delusion of nihilism or death. PMID:22054629

  1. Genetics Home Reference: Wolff-Parkinson-White syndrome

    MedlinePlus

    ... Home Health Conditions Wolff-Parkinson-White syndrome Wolff-Parkinson-White syndrome Enable Javascript to view the expand/ ... Download PDF Open All Close All Description Wolff-Parkinson-White syndrome is a condition characterized by abnormal ...

  2. 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. PMID:21953737

  3. 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

  4. International validation of a behavioral scale in Parkinson's disease without dementia.

    PubMed

    Rieu, Isabelle; Martinez-Martin, Pablo; Pereira, Bruno; De Chazeron, Ingrid; Verhagen Metman, Leo; Jahanshahi, Marjan; Ardouin, Claire; Chéreau, Isabelle; Brefel-Courbon, Christine; Ory-Magne, Fabienne; Klinger, Helene; Peyrol, Fleur; Schupbach, Michael; Dujardin, Kathy; Tison, François; Houeto, Jean Luc; Krack, Paul; Durif, Franck

    2015-04-15

    The "Ardouin Scale of Behavior in Parkinson's Disease" is a new instrument specifically designed for assessing mood and behavior with a view to quantifying changes related to Parkinson's disease, to dopaminergic medication, and to non-motor fluctuations. This study was aimed at analyzing the psychometric attributes of this scale in patients with Parkinson's disease without dementia. In addition to this scale, the following measures were applied: the Unified Parkinson's Disease Rating Scale, the Montgomery and Asberg Depression Rating Scale, the Lille Apathy Rating Scale, the Bech and Rafaelsen Mania Scale, the Positive and Negative Syndrome Scale, the MacElroy Criteria, the Patrick Carnes criteria, the Hospital Anxiety and Depression Scale, and the Mini-International Neuropsychiatric Interview. Patients (n=260) were recruited at 13 centers across four countries (France, Spain, United Kingdom, and United States). Cronbach's alpha coefficient for domains ranged from 0.69 to 0.78. Regarding test-retest reliability, the kappa coefficient for items was higher than 0.4. For inter-rater reliability, the kappa values were 0.29 to 0.81. Furthermore, most of the items from the Ardouin Scale of Behavior in Parkinson's Disease correlated with the corresponding items of the other scales, depressed mood with the Montgomery and Asberg Depression Rating Scale (ρ=0.82); anxiety with the Hospital Anxiety and Depression Scale-anxiety (ρ=0.56); apathy with the Lille Apathy Rating Scale (ρ=0.60). The Ardouin Scale of Behavior in Parkinson's disease is an acceptable, reproducible, valid, and precise assessment for evaluating changes in behavior in patients with Parkinson's disease without dementia. PMID:25809278

  5. Clinical and Neuropsychological Differences between Mild Parkinson's Disease Dementia and Dementia with Lewy Bodies

    PubMed Central

    Petrova, Mariya; Mehrabian-Spasova, Shima; Aarsland, Dag; Raycheva, Margarita; Traykov, Latchezar

    2015-01-01

    Background The specific profile of dementia in Parkinson's disease (PDD) and dementia with Lewy bodies (DLB) in the earliest stages of dementia is still unclear and subject of considerable controversy. Methods We investigated 27 PDD patients and 24 DLB patients with parkinsonism in the early stage of dementia, i.e. with a Mini-Mental State Examination score of ≥24. Results Compared to PDD, patients with DLB demonstrated significantly lower scores when testing attention and executive functions [modified card sorting test (p < 0.001) and digit span backward (p < 0.02)], as well as when testing constructive abilities [copy of complex designs (p = 0.001) and pentagon (p < 0.001)]. Using logistic regression analysis, diagnosis was predicted from the cognitive profile, with an overall accuracy of 88.2%. In addition, PDD patients showed a significantly higher Unified Parkinson's Disease Rating Scale (UPDRS) motor subscore (p < 0.001) as well as higher UPDRS motor item scores [tremor at rest (p = 0.01) and bradykinesia (p = 0.001)]. Conclusions The cognitive profile in PDD differs from that in DLB in the early stage of dementia, with worse performance on tests of attention and executive functions and constructive abilities in DLB compared to PDD patients. In contrast, motor symptoms are more severe in PDD than in DLB. PMID:26195977

  6. A controlled, longitudinal study of dementia in Parkinson's disease.

    PubMed Central

    Biggins, C A; Boyd, J L; Harrop, F M; Madeley, P; Mindham, R H; Randall, J I; Spokes, E G

    1992-01-01

    Serial assessments of cognition, mood, and disability were carried out at nine month intervals over a 54 month period on a cohort of 87 patients with Parkinson's disease (PD) and a matched cohort of 50 control subjects. Dementia was diagnosed from data by rigorously applying DSM-III-R criteria. Initially, 6% (5/87) PD patients were demented, compared with none of the 50 control subjects. A further 10 PD patients met the dementia criteria during the follow up period; this was equivalent, with survival analysis, to a cumulative incidence of 19%. With the number of person years of observation as the denominator, the incidence was 47.6/1000 person years of observation. None of the control subjects fulfilled dementia criteria during the follow up period. The patients with PD who became demented during follow up were older at onset of Parkinson's disease than patients who did not become demented, had a longer duration of Parkinson's disease, and were older at inclusion to the study. PMID:1640232

  7. Dural arteriovenous fistula presenting with progressive dementia and parkinsonism

    PubMed Central

    Fujii, Hiroki; Nagano, Yoshito; Hosomi, Naohisa; Matsumoto, Masayasu

    2014-01-01

    We report a rare case of progressive parkinsonism and cognitive dysfunction due to dural arteriovenous fistula (DAVF). A 69-year-old man, with a history of hypertension and diabetes, was admitted to our hospital because of parkinsonism and dementia. Susceptibility-weighted imaging (SWI) revealed a thrombus in the superior sagittal sinus (SSS) and marked dilation of the medullary vein suggestive of the presence of comorbid DAVF. A single-photon emission CT (SPECT) showed widespread hypoperfusion in the bilateral frontal lobes. Selective cerebral angiography revealed a DAVF in SSS. These symptoms were significantly ameliorated following transvenous embolisation of the venous sinus at the shunting point. Reversible parkinsonism and dementia after embolisation was correlated with decreased dilation of medullary vein on SWI and improved cerebral blood flow on SPECT in the frontal lobes. Differentiation of parkinsonian and dementia symptoms due to DAVF from those associated with neurodegenerative disease is of great importance because DAVF-associated deficits may be reversed by endovascular therapy. PMID:24891481

  8. 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

  9. 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. PMID:21442661

  10. 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

  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. Dementia with Lewy bodies in Meige Syndrome

    PubMed Central

    Ho, Ho Yin; Yu, Chi Shing

    2012-01-01

    Meige syndrome is a rare form of segmental dystonia characterized by blepharospasm and oromandibular dystonia. A few case reports of Meige syndrome have been associated with Lewy body pathologies, and the syndrome was also proposed for inclusion in the spectrum of Lewy body disease. We report a case of an elderly gentleman with a history of Meige syndrome for more than 10 years who developed dementia with Lewy bodies. Updated clinical and pathological evidence of linkages between these two conditions is also presented. PMID:22984651

  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. [Early diagnosis of dementia with the help of MR-morphometry in patients with Parkinson's disease].

    PubMed

    Trufanov, A G; Odinak, M M; Litvinenko, I V; Rezvantsev, M V; Voronkov, L V

    2013-09-01

    54 patients with idiopathic Parkinson's disease were examined. 1,5 Tesla MRI with T1 gradient-echo protocol and following calculating by FreeSurfer software was performed. Dementia was revealed in 23 patients. Significant changes of different zones of brain-cortex were revealed in patients with Parkinson's disease accompanied by dementia. Changes were revealed in the hemispheres, particularly in frontal, temporal and occipital lobes. Thickness of lingual medial occipitotemporal gyrus can be used as a criterion for dementia prognosis. Individual patient monitoring and cortex alteration evaluation allow prognosticating increasing risk of cognitive disorders development and prescribing proper therapy. PMID:24341200

  15. DEVELOPMENT OF CLINICAL DEMENTIA RATING SCALE CUTOFF SCORES FOR PATIENTS WITH PARKINSON'S DISEASE

    PubMed Central

    Wyman-Chick, Kathryn A.; Scott, BJ

    2015-01-01

    Background The purpose of this study was to explore validity of the Clinical Dementia Rating Scale in measuring cognitive impairment among individuals with Parkinson's disease. The scale was created for use in patients with Alzheimer's disease and, to date, there have been no published studies examining if this tool is appropriate for patients with Parkinson's disease. Methods The data were obtained from the National Alzheimer's Coordinating Center database and included 490 subjects diagnosed with Parkinson's disease, further categorized as having Parkinson's disease dementia (n= 151), mild cognitive impairment (n= 186), or normal cognition (n = 153) by a treating physician. Sensitivity, specificity, positive predictive value and negative predictive values were calculated for the Clinical Dementia Rating Scale Global Score as well as the Sum of Boxes Score using existing cutoff scores. Finally, new cutoff scores were calculated using sensitivity and specificity values derived using Receiver Operating Characteristic curves. Results Sensitivity and specificity of the published Global Score cutoff scores for patients with dementia were .34 and .10, respectively. The newly calculated cutoff scores for patients with dementia yielded a sensitivity of .79 and a specificity of .96. The area under the curve was 0.92 (95% CI = 0.90-0.95). Conclusion The CDR is a useful tool in identifying dementia in patients with Parkinson's disease when the cutoff scores are adjusted. PMID:26660076

  16. Identifying Fallers among Home Care Clients with Dementia and Parkinson's Disease.

    PubMed

    Bansal, Symron; Hirdes, John P; Maxwell, Colleen J; Papaioannou, Alexandra; Giangregorio, Lora M

    2016-09-01

    Few studies have focused on falls among home care (HC) clients with neurological conditions. This study identified factors that increase risk of falling among HC clients with no recent history of falls, and explored whether risk profiles varied among those with dementia or parkinsonism compared to those without selected neurological conditions. A retrospective cohort design was used and analysis of data from community-based HC clients across Ontario was conducted on a sample of ambulatory clients with dementia, parkinsonism, or none of the selected neurological conditions. Data were obtained from the Resident Assessment Instrument for HC (RAI-HC) assessment. The outcome used in multivariable analyses was whether clients fell during follow-up. Unsteady gait was a strong predictor of falls across all three groups. Co-morbid parkinsonism most strongly predicted falls in the dementia group. Clients with borderline intact to mild cognitive impairment had higher odds of falling within the parkinsonism and comparison groups. PMID:27426223

  17. A Comparative White Matter Study with Parkinson's disease, Parkinson's Disease with Dementia and Alzheimer's Disease

    PubMed Central

    Perea, Rodrigo D; Rada, Rebecca C; Wilson, Jessica; Vidoni, Eric D; Morris, Jill K; Lyons, Kelly E; Pahwa, Rajesh; Burns, Jeffrey M; Honea, Robyn A

    2014-01-01

    Alzheimer's disease (AD) and Parkinson's disease (PD) are among the most common neurodegenerative disorders affecting older populations. AD is characterized by impaired memory and cognitive decline while the primary symptoms of PD include resting tremor, bradykinesia and rigidity. In PD, mild cognitive changes are frequently present, which could progress to dementia (PD dementia (PDD)). PDD and AD dementias are different in pathology although the difference in microstructural changes remains unknown. To further understand these diseases, it is essential to understand the distinct mechanism of their microstructural changes. We used diffusion tensor imaging (DTI) to investigate white matter tract differences between early stage individuals with AD (n=14), PD (n=12), PDD (n=9), and healthy non-demented controls (CON) (n=13). We used whole brain tract based spatial statistics (TBSS) and a region of interest (ROI) analysis focused on the substantia nigra (SN). We found that individuals with PDD had more widespread white matter degeneration compared to PD, AD, and CON. Individuals with AD had few regional abnormalities in the anterior and posterior projections of the corpus callosum while PD and CON did not appear to have significant white matter degeneration when compared to other groups. ROI analyses showed that PDD had the highest diffusivity in the SN and were significantly different from CON. There were no significant ROI differences between CON, PD, or AD. In conclusion, global white matter microstructural deterioration is evident in individuals with PDD, and DTI may provide a means with which to tease out pathological differences between AD and PD dementias. PMID:24724042

  18. 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

  19. Clinical correlates of pathology in the claustrum in Parkinson's disease and dementia with Lewy bodies.

    PubMed

    Kalaitzakis, M E; Pearce, R K B; Gentleman, S M

    2009-09-11

    Dementia and visual hallucinations are common complications of Parkinson's disease (PD), yet their patho-anatomical bases are poorly defined. We studied alpha-synuclein (alphaSyn), tau and amyloid-beta (Abeta) pathology in the claustrum of 20 PD cases without dementia, 12 PD cases with dementia (PDD) and 7 cases with dementia with Lewy bodies (DLB). alphaSyn positivity was observed in 75% of PD cases without dementia and in 100% of PDD and DLB cases. Abeta was observed in the claustrum in 25% of PD, 58% of PDD and 100% of DLB cases. Tau was negligible in all cases restricting further analysis. Compared to PD cases without dementia, PDD cases demonstrated a significantly greater alphaSyn burden in the claustrum (p=0.0003). In addition, DLB cases showed a significantly increased alphaSyn deposition when compared to PDD (p=0.02) and PD without dementia (p=0.0002). A similar hierarchy, PDdementia in Parkinson's disease and DLB. PMID:19523504

  20. 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

  1. Evidence for modest familial co-aggregation between dementia and parkinsonism.

    PubMed

    Feldman, Adina L; Wirdefeldt, Karin; Johansson, Anna L V; Gatz, Margaret; Pedersen, Nancy L

    2014-01-01

    To investigate the contribution of shared familial risk to the co-occurrence of dementia and parkinsonism by studying familial co-aggregation of Alzheimer's disease (AD) and Parkinson's disease (PD). Using Swedish population-based registers we constructed two cohorts; a first-degree relative cohort of persons born 1932-1960 (n = 2,775,332) and a spouse cohort of persons born 1890-1960 (n = 4,736,006). Study persons were followed up between 1969 and 2009 in the National Patient and Cause of Death Registers. We modeled the association between incidence of disease and having at least one affected relative using Cox proportional hazard regression that estimated hazard ratios (HRs) with 95% confidence intervals (CIs) adjusted for age, sex and number of relatives. Within each disorder; dementia, AD, parkinsonian disorders and PD, there was a strong association between risk of disease and having at least one affected sibling or parent. There was also a modest shared familial risk between the diseases; risk of parkinsonian disorders was associated with having a sibling with AD (HR 1.35, 95% CI 1.11-1.65) and risk of dementia was associated with having a sibling with PD (HR 1.20, 95% CI 1.02-1.41). There were no meaningful familial risks among spouses. The risk of co-occurring dementia in PD was considerably increased (HR 2.83, 95% CI 2.76-2.89). There is strong familial aggregation within dementia, AD, parkinsonian disorders and PD, and modest familial co-aggregation between dementia and parkinsonism. Thus, co-occurrence of dementia and parkinsonism is not primarily caused by shared familial risk between AD and PD. PMID:24248476

  2. 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). PMID:27446628

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

    PubMed Central

    Roberts, Julie; Lloyd-Williams, Huw; 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). PMID:27446628

  4. [Overlooked Wolff-Parkinson-White syndrome].

    PubMed

    Nielsen, Trine Skov; Dalager, Søren; Larsen, Maiken Kudahl; Jensen, Henrik Kjaerulf; Lundemose, Jytte Banner

    2010-09-13

    An autopsy in a 28-year-old man did not explain the cause of sudden unexpected death. However, a history of episodes with tachycardia and dizziness and a reassessed previous electrocardiogram exhibiting ventricular pre-excitation was consistent with Wolff-Parkinson-White (WPW) syndrome. In this patient we believe that the occurrence of atrial fibrillation caused sudden cardiac death from ventricular fibrillation due to a short refractory period of an accessory atrioventricular pathway and a very rapid ventricular rate in atrial fibrillation. PMID:20836960

  5. Validation of Neuropsychological Tests to Screen for Dementia in Chinese Patients With Parkinson's Disease.

    PubMed

    Qiao, Jin; Wang, Xiaoyan; Lu, Wenhui; Cao, Hongmei; Qin, Xing

    2016-06-01

    To compare the accuracy of different neuropsychological tests and their combinations for deriving reliable cognitive indices for dementia diagnosis in Parkinson's disease (PD). One hundred forty consecutive patients with PD were recruited and administrated an extensive battery of neuropsychological tests. Discriminant analysis and receiver-operator characteristic curve were used to evaluate their correct classifications and validity. Patients with PD having dementia (PDD; 23.5%) performed significantly worse in all tests than patients without dementia. Age of onset, disease duration, Hoehn-Yahr grade, Unified Parkinson's Disease Rating Scale part III scores, and education were associated with dementia in patients with PD. Mini-Mental State Examination (MMSE), Montreal Cognitive Assessment, and Block Design (BD) showed better specificity and sensitivity when used alone, and combined use of MMSE and BD further increased the validity. Our results indicated that the accuracy of MMSE was better in dementia diagnosis of Chinese patients with PD, and combined use of MMSE and BD could further increase the validity of dementia diagnosis. PMID:26646116

  6. 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. PMID:23026537

  7. 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

  8. 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

  9. 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.

  10. Dementia and cognitive impairment in patients with Parkinson's disease from India: a 7-year prospective study.

    PubMed

    Sanyal, Jaya; Banerjee, Tapas Kumar; Rao, Vadlamudi Raghavendra

    2014-11-01

    Depression and cognitive impairment are frequent manifestations in Parkinson's disease (PD). Although a few longitudinal studies have reported on depression and dementia in PD, there is a yet a lack of such studies in India. This 7-year longitudinal study is a hospital-based prospective case (n = 250)-control (n = 280) study. In all, 36.8% had PD with no cognitive impairment (PD-Normal), 27.2% of the patients with PD were affected by dementia (PDD), and 36% of the remaining patients with PD had mild cognitive impairment (PD-MCI) at baseline. After 7 years of evaluation, 32 new patients, 12 patients from the PD-MCI group and 9 patients from the PD-Normal group, were diagnosed with dementia. The 7-year prevalence rate for dementia was estimated to be 49.28%. In the Indian population, an early onset of dementia is noted among patients with PD, with the age of onset being less than 55 years. Patients with early-onset PDD showed depression symptoms that differed significantly from the controls of the same age-group. There was a major difference in verbal fluency, word list recall, constructional praxis and recall, word list recognition, abridged Boston Naming Test, word list memory with repetition, and Mini-Mental State Examination between PD-MCI and PDD groups. Hallucinations before baseline (odds ratio [OR] = 4.427, 95% confidence interval [CI] = 2.122-9.373), akinetic/tremor dominancy (OR = 0.380, 95%CI = 0.149-0.953), and asymmetrical disease onset (OR = 0.3285, 95%CI = 0.1576-0.685) can be considered as risk factors for patients with dementia. Patients with early-onset PD might be more prone to complex depression and dementia. As the disease progresses, akinetic-dominant PD, early hallucinations, and asymmetrical disease onset are the potential risk factors for the development of dementia in patients with PD. PMID:24771763

  11. 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…

  12. 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…

  13. Parkinson's disease dementia – A diminished role for the Lewy body

    PubMed Central

    Libow, Leslie S.; Frisina, Pasquale G.; Haroutunian, Vahram; Perl, Daniel P.; Purohit, Dushyant P.

    2010-01-01

    The literature currently views Lewy bodies as central in the pathogenesis of Parkinson's disease dementia (PDD) when Alzheimer's disease (AD) or vascular pathology is not present. Because the neuropathology of PDD is not well understood, the pathological features of PDD were characterized in eighteen PD brain specimens using published criteria for AD, Diffuse Lewy Body Disease (DLBD), and Vascular Disease as a framework. Among the PD dementia (n = 16) subjects, 3 (19%) did not have LBs outside of the brain stem, nor AD or vascular pathology. In two additional cases, one did have rare LBs in the neocortex and cingulate gyrus. However, these two cases did not meet the diagnostic criteria for DLBD. Beyond these 5 cases, the remaining PD dementia subjects fitted a classical pathological profile consistent with AD (38%), vascular disease (12.5%), DLBD (6%), or a combination of these pathologies (12.5%). The findings from this study do not support the hypothesis that LBs are the main substrate for dementia in PD. More research with a larger sample size is needed to determine whether the LB may be a secondary phenomenon and/or an “innocent-bystander”. The entire role of the LB in PD dementia is again brought into question. PMID:19346154

  14. 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…

  15. [An autopsy case of senile dementia with pathological features of Parkinson's disease].

    PubMed

    Oshima, Kenichi; Tsuchiya, Kuniaki; Iritani, Shuji; Niizato, Kazuhiro; Akiyama, Haruhiko; Ikeda, Kenji; Arai, Heii

    2004-07-01

    We report an autopsy case of Parkinson's disease mimicking senile dementia of the Alzheimer type. A Japanese man developed memory disturbance and visual hallucination at age 70. Although he died from pneumonia at age of 74, he had no neurological signs throughout the clinical course. The weight of his brain was 1,420 g. Macroscopic examination of the brain revealed prominent depigmentation of the substantia nigra and locus ceruleus. Histological examination disclosed neuronal loss with astrocytosis and the appearance of the Lewy bodies in the nucleus basalis of Meynert, substantia nigra, locus ceruleus, and dorsal vagal nucleus. There were widespread senile plaques in the brain, including the precentral gyrus, which was compatible with Braak stage C. A small number of neurofibrillary changes were present in the limbic areas, consistent with Braak stage III. This case is consistent with brain stem dominance with the pathological diagnosis of the Consortium on Dementia with Lewy Bodies International Workshop. That is, it is compatible with Parkinson's disease. We postulate that the clinical features of Parkinson's disease are more widespread than previously considered. PMID:15379289

  16. 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

  17. [Future standards in diagnosing Parkinson syndrome].

    PubMed

    Reichmann, H

    2010-03-01

    So far, the diagnosis of an idiopathic Parkinson-syndrome was based on the British Brain Bank Criteria, that is the occurrence of bradykinesia together with at least one more of the cardinal symptoms, i. e. resting tremor, rigidity or postural instability. The latter symptom is not useful, since it occurs only in the Hoehn and Yahr stage III which is seen in advanced PD patients. Thus, this symptom is certainly not useful for EARLY diagnosis. Early signs for PD are hyposmia, constipation, REM sleep behaviour disorder and depression. Early diagnosis is still a clinical one, which can be supported by a levodopa test. It can be expected that in the near future gene chips will be available for patients with a positive family history for PD or with an early onset. As long as a blood test for PD is not available, methods such as SPECT and PET are extremely useful in patients with an unclear clinical symptomatology. In my own view, each PD patient should receive once in his career a cranial CT or MRI. PMID:20195942

  18. 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

  19. Parkinsonism and dementia are negative prognostic factors for the outcome of subdural hematoma.

    PubMed

    Arca, Roberta; Ricchi, Valeria; Murgia, Daniela; Melis, Marta; Floris, Francesco; Mereu, Alessandra; Contu, Paolo; Marrosu, Francesco; Melis, Maurizio; Cossu, Giovanni

    2016-08-01

    To determine, among a population with subdural hematoma (SH), whether patients affected by neurodegenerative disorders (parkinsonism and dementia) have a worse clinical outcome. We reviewed the data of patients diagnosed with fall-related SH discharged from the Departments of Neurology/Stroke unit, Neurosurgery, Intensive Care Unit at Brotzu General Hospital (Cagliari, Italy) between January 2010 and December 2013. Patients with severe traumatisms, evidence of spontaneous intracerebral bleeding or aged less than 50 were excluded. 332 patients were selected: 69 with a neurodegenerative parkinsonism or dementia (N-group), 217 with history of chronic non-neurological medical conditions with significant disability, previous falls and/or balance problems (NND-group) and 46 with a history of "minor" chronic non-neurological disorder. (NN-group). The clinical status at admission and discharge was assessed by modified Rankin Scale (mRS). The time-span between trauma and hospital admission was also calculated. At hospital admission we found a significantly longer delay in SH's diagnosis (χ (2) test p < 0.001) and a worse mRS score (Kruskal Wallis p < 0.001) in the N-group compared to both NN and NND-groups. During hospital stay we observed the lack of significant variation in mRS score in N-group (Wilcoxon test p = 0.86), in contrast with NN and NND-groups who significantly improved (Wilcoxon test p < 0.001). Our results demonstrate that the consequences of SH are more severe in the N-group compared to NN and NND-groups. The longer interval between trauma and hospital admittance plays a critical role in worsening the outcome of patients with parkinsonism and dementia compared to subjects without neurodegenerative disorders. PMID:27120071

  20. 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. PMID:27247605

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

    PubMed Central

    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

    2016-01-01

    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. PMID:27247605

  2. Long Leukocyte Telomere Length at Diagnosis Is a Risk Factor for Dementia Progression in Idiopathic Parkinsonism

    PubMed Central

    Degerman, Sofie; Domellöf, Magdalena; Landfors, Mattias; Linder, Jan; Lundin, Mathias; Haraldsson, Susann; Elgh, Eva; Roos, Göran; Forsgren, Lars

    2014-01-01

    Telomere length (TL) is regarded as a marker of cellular aging due to the gradual shortening by each cell division, but is influenced by a number of factors including oxidative stress and inflammation. Parkinson's disease and atypical forms of parkinsonism occur mainly in the elderly, with oxidative stress and inflammation in afflicted cells. In this study the relationship between blood TL and prognosis of 168 patients with idiopathic parkinsonism (136 Parkinson's disease [PD], 17 Progressive Supranuclear Palsy [PSP], and 15 Multiple System Atrophy [MSA]) and 30 controls was investigated. TL and motor and cognitive performance were assessed at baseline (diagnosis) and repeatedly up to three to five years follow up. No difference in TL between controls and patients was shown at baseline, nor any significant difference in TL stability or attrition during follow up. Interestingly, a significant relationship between TL at diagnosis and cognitive phenotype at follow up in PD and PSP patients was found, with longer mean TL at diagnosis in patients that developed dementia within three years. PMID:25501556

  3. Parkinsonism and impaired axonal transport in a mouse model of frontotemporal dementia

    PubMed Central

    Ittner, Lars M.; Fath, Thomas; Ke, Yazi D.; Bi, Mian; van Eersel, Janet; Li, Kong M.; Gunning, Peter; Götz, Jürgen

    2008-01-01

    Frontotemporal dementia (FTD) is characterized by cognitive and behavioral changes and, in a significant subset of patients, Parkinsonism. Histopathologically, FTD frequently presents with tau-containing lesions, which in familial cases result from mutations in the MAPT gene encoding tau. Here we present a novel transgenic mouse strain (K3) that expresses human tau carrying the FTD mutation K369I. K3 mice develop a progressive histopathology that is reminiscent of that in human FTD with the K369I mutation. In addition, K3 mice show early-onset memory impairment and amyotrophy in the absence of overt neurodegeneration. Different from our previously generated tau transgenic strains, the K3 mice express the transgene in the substantia nigra (SN) and show an early-onset motor phenotype that reproduces Parkinsonism with tremor, bradykinesia, abnormal gait, and postural instability. Interestingly, motor performance of young, but not old, K3 mice improves upon L-dopa treatment, which bears similarities to Parkinsonism in FTD. The early-onset symptoms in the K3 mice are mechanistically related to selectively impaired anterograde axonal transport of distinct cargos, which precedes the loss of dopaminergic SN neurons that occurs in aged mice. The impaired axonal transport in SN neurons affects, among others, vesicles containing the dopamine-synthesizing enzyme tyrosine hydroxylase. Distinct modes of transport are also impaired in sciatic nerves, which may explain amyotrophy. Together, the K3 mice are a unique model of FTD-associated Parkinsonism, with pathomechanistic implications for the human pathologic process. PMID:18832465

  4. RAB39B gene mutations are not a common cause of Parkinson's disease or dementia with Lewy bodies.

    PubMed

    Hodges, Kyndall; Brewer, Sheridan S; Labbé, Catherine; Soto-Ortolaza, Alexandra I; Walton, Ronald L; Strongosky, Audrey J; Uitti, Ryan J; van Gerpen, Jay A; Ertekin-Taner, Nilüfer; Kantarci, Kejal; Lowe, Val J; Parisi, Joseph E; Savica, Rodolfo; Graff-Radford, Jonathan; Jones, David T; Knopman, David S; Petersen, Ronald C; Murray, Melissa E; Graff-Radford, Neill R; Ferman, Tanis J; Dickson, Dennis W; Wszolek, Zbigniew K; Boeve, Bradley F; Ross, Owen A; Lorenzo-Betancor, Oswaldo

    2016-09-01

    Mutations in Ras-related protein Rab-39B (RAB39B) gene have been linked to X-linked early-onset Parkinsonism with intellectual disabilities. The aim of this study was to address the genetic contribution of RAB39B to Parkinson's disease (PD), dementia with Lewy bodies (DLB), and pathologically confirmed Lewy body dementia (pLBD) cases. A cohort of 884 PD, 399 DLB, and 379 pLBD patients were screened for RAB39B mutations, but no coding variants were found, suggesting RAB39B mutations are not a common cause of PD, DLB, or pLBD in Caucasian population. PMID:27459931

  5. 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.

  6. Wolff-Parkinson-white syndrome mimics a conduction disease.

    PubMed

    Marrakchi, S; Kammoun, I; Kachboura, S

    2014-01-01

    Background. It is important to recognise Wolff-Parkinson-White (WPW) syndrome in electrocardiograms (ECG), as it may mimic ischaemic heart disease, ventricular hypertrophy, and bundle branch block. Recognising WPW syndrome allows for risk stratification, the identification of associated conditions, and the institution of appropriate management. Objective. The present case showed that electrophysiological study is indicated in patients with abnormal ECG and syncope. Case Report. A 40-year-old man with Wolff-Parkinson-White syndrome was presented to emergency with syncope. A baseline ECG was a complete right branch block and posterior left hemiblock. He was admitted to the cardiac care unit for pacemaker implantation. The atypical figure of complete right branch block and posterior left hemiblock was thought to be a "false positive" of conduction abnormality. But the long anterograde refractory period of the both accessory pathway and atrioventricular conduction may cause difficulty in diagnosing Wolff-Parkinson-White syndrome, Conclusion. A Wolff-Parkinson-White Syndrome may mimic a conduction disease. No reliable algorithm exists for making an ECG diagnosis of a preexcitation syndrome with conduction disorders. This can lead to diagnostic and therapeutic dilemmas in the context of syncope. PMID:25114686

  7. Wolff-Parkinson-White Syndrome Mimics a Conduction Disease

    PubMed Central

    Marrakchi, S.; Kammoun, I.; Kachboura, S.

    2014-01-01

    Background. It is important to recognise Wolff-Parkinson-White (WPW) syndrome in electrocardiograms (ECG), as it may mimic ischaemic heart disease, ventricular hypertrophy, and bundle branch block. Recognising WPW syndrome allows for risk stratification, the identification of associated conditions, and the institution of appropriate management. Objective. The present case showed that electrophysiological study is indicated in patients with abnormal ECG and syncope. Case Report. A 40-year-old man with Wolff-Parkinson-White syndrome was presented to emergency with syncope. A baseline ECG was a complete right branch block and posterior left hemiblock. He was admitted to the cardiac care unit for pacemaker implantation. The atypical figure of complete right branch block and posterior left hemiblock was thought to be a “false positive” of conduction abnormality. But the long anterograde refractory period of the both accessory pathway and atrioventricular conduction may cause difficulty in diagnosing Wolff-Parkinson-White syndrome, Conclusion. A Wolff-Parkinson-White Syndrome may mimic a conduction disease. No reliable algorithm exists for making an ECG diagnosis of a preexcitation syndrome with conduction disorders. This can lead to diagnostic and therapeutic dilemmas in the context of syncope. PMID:25114686

  8. 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.

  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. 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…

  12. 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…

  13. [Familial juvenile parkinsonism with dementia and autonomic failure--a case report].

    PubMed

    Nitta, E; Ishikawa, A; Ishiguro, H; Baba, H; Fukuhara, N

    1993-01-01

    A case of familial juvenile parkinsonism with dementia, orthostatic hypotension, neurogenic bladder and constipation was reported. He had been in a good health until the age of 28 when a finger tremor occurred on effort to hold hands in a definite position, and disturbances in gait and speech were noted. These symptoms were relieved by levodopa treatment followed by dyskinesia and motor fluctuations. Three years later, he complained of faintness, constipation and urinary frequency. The neurological examination revealed mentally sound male with masked face, tremor and rigidity in his extremities, and short step gait with lateropulsion. Urodynamic study showed uninhibited bladder. In the following years, orthostatic hypotension, dysuria and urinary retention developed gradually. He became mentally loose and was unable to take medicines appropriately. When in the Nishiojiya Byoin National Sanatorium, he tried to snake out the hospital many times. His parents and a brother suffered from Parkinson's disease and juvenile parkinsonism, respectively, suggesting an autosomal dominant inheritance. On admission to our hospital, he was apathetic. He had masked face, bilateral postural tremor, frozen gait and dyskinesia in the right lower extremity. Little bradykinesia or rigidity was noted. His muscle tone and deep tendon reflexes were decreased but neither muscular wasting, weakness, ataxia nor sensory disturbance was observed. Laboratory data including ceruloplasmin, copper, dopamine-beta-hydroxylase and lysosomal enzyme activities were normal except for mild anemia. A cranial CT scan revealed mild cortical atrophy in the frontal and temporal lobes, but nerve conduction study and cortical evoked potentials showed no abnormality. While in the hospital, his mental functions deteriorated to the state of dementia and orthostatic hypotension became apparent.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:8334779

  14. 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…

  15. Converging mediators from immune and trophic pathways to identify Parkinson disease dementia

    PubMed Central

    Schmitz, Christopher T.; Snyder, Noelle L.; Chen, Kewei; Walker, Douglas G.; Davis, Kathryn J.; Belden, Christine; Caviness, John N.; Driver-Dunckley, Erika; Adler, Charles H.; Sabbagh, Marwan N.; Shill, Holly A.

    2016-01-01

    Objective: To identify a panel of peripheral inflammatory/immune mediators that could discriminate Parkinson disease with dementia (PDD) from Parkinson disease (PD) without dementia. Methods: Plasma samples from 52 patients with PD and 22 patients with PDD were prepared from freshly collected blood following an institutional review board–approved protocol. A total of 160 proteins were measured using a multiplex antibody array. Plasma α-synuclein levels were analyzed by an electrochemiluminescence immunoassay. The main objective of the statistical analyses was to identify PDD discriminants using the plasma protein profile alone or in combination with age. Results: The PD and PDD groups differed significantly in cognitive measurements (Mini-Mental State Examination, Auditory Verbal Learning Test-A7, and Clinical Dementia Rating) and age. The age-adjusted levels of thymus and activation-regulated chemokine (TARC) and platelet-derived growth factor (PDGF)-AA were significantly different between disease groups. The levels of plasma α-synuclein significantly correlated with 26 proteins; among them, PDGF-BB, TARC, PDGF-AA, and epidermal growth factor were the highest. Linear discriminant analysis with leave-one-out cross-validation identified a 14-protein panel with age as discriminants of PDD (96% sensitivity, 89% specificity, area under the curve = 0.9615). Conclusions: We showed that multiple proteins that are mediators of growth/trophic and immune response-related pathways had discriminatory power for identifying PDD in patients with PD. Validation of this discovery-based study in longitudinal population-based studies is warranted. Classification of evidence: This study provides Class III evidence that a 14-protein panel plasma assay combined with age has a sensitivity of 96% and a specificity of 89% for PDD. PMID:26848485

  16. A comprehensive review of proton magnetic resonance spectroscopy studies in dementia and Parkinson's disease.

    PubMed

    Firbank, M J; Harrison, R M; O'Brien, J T

    2002-01-01

    We reviewed the literature of proton magnetic resonance spectroscopy (MRS) in dementia and Parkinson's disease (PD) and quantitatively compared the reported values of the markers N-acetyl aspartate (NAA), choline, and myo-Inositol between control and disease groups. We analysed a total of 27 reports in dementia. Combining the quantitative data from these showed a relative decrease of 15% in NAA level in the temporal lobe tissue in patients with Alzheimer's disease (AD) compared with controls. The rest of the brain showed a seemingly uniform 10% decrease in NAA levels in AD compared with controls. myo-Inositol was raised by about 15%, again uniformly throughout the brain, but there was no evidence for changed levels of choline. We found 15 reports of MRS in PD, which show a small decrease (5%) in the NAA level in the lentiform nucleus compared with controls. In progressive supranuclear palsy (PSP), there is a greater decrease in NAA levels in the frontal region and the lentiform nucleus. This may aid in the diagnosis of PSP. Further research is needed to determine spectroscopic changes in other dementias, to monitor how markers change with disease progression and to establish clinical utility. PMID:12145453

  17. 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. PMID:23948919

  18. 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. PMID:26003251

  19. Pisa syndrome in Parkinson's disease and parkinsonism: clinical features, pathophysiology, and treatment.

    PubMed

    Barone, Paolo; Santangelo, Gabriella; Amboni, Marianna; Pellecchia, Maria Teresa; Vitale, Carmine

    2016-09-01

    Pisa syndrome is defined as a reversible lateral bending of the trunk with a tendency to lean to one side. It is a frequent and often disabling complication of Parkinson's disease, and has also been described in several atypical forms of parkinsonism and in neurodegenerative and psychiatric disorders after drug exposure and surgical procedures. Although no consistent diagnostic criteria for Pisa syndrome are available, most investigations have adopted an arbitrary cutoff of at least 10° of lateral flexion for the diagnosis of the syndrome. Pathophysiological mechanisms underlying Pisa syndrome have not been fully explained. One hypothesis emphasises central mechanisms, whereby Pisa syndrome is thought to be caused by alterations in sensory-motor integration pathways; by contrast, a peripheral hypothesis emphasises the role of anatomical changes in the musculoskeletal system. Furthermore, several drugs are reported to induce Pisa syndrome, including antiparkinsonian drugs. As Pisa syndrome might be reversible, clinicians need to be able to recognise this condition early to enable prompt management. Nevertheless, further research is needed to determine optimum treatment strategies. PMID:27571158

  20. The patient with Wolff-Parkinson-White syndrome.

    PubMed

    Berry, V A

    1994-03-01

    Wolff-Parkinson-White (WPW) syndrome is characterized by a classic ECG pattern and a history of palpitations, syncope, pre-syncope, or tachyarrhythmias. An electrophysiology study is an integral part of the diagnosis and treatment of this disease, and with the advent of catheter ablative techniques, WPW syndrome is now considered a curable disease. The critical care nurse needs to be aware of the acute and chronic treatment of this syndrome, the complications of therapy, and the educational needs of the patient and family. PMID:8192882

  1. Dementia

    MedlinePlus

    PATIENT / FAMILY TEACHING SHEET Dementia What is dementia? Dementia is a result of diseases that affect how the brain works. Alzheimer’s disease is the most common cause of dementia. Symptoms occur ...

  2. Dementia

    MedlinePlus

    ... Awards Enhancing Diversity Find People About NINDS NINDS Dementia Information Page Condensed from Dementia: Hope Through Research ... en Español Additional resources from MedlinePlus What is Dementia? Dementia is not a specific disease. It is ...

  3. 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. PMID:26643944

  4. Are all subcortical dementias alike? Verbal learning and memory in Parkinson's and Huntington's disease patients.

    PubMed

    Massman, P J; Delis, D C; Butters, N; Levin, B E; Salmon, D P

    1990-10-01

    The utility of the concept of 'subcortical dementia' was investigated by comparing the verbal learning and memory abilities of Parkinson's disease (PD) patients with those of Huntington's disease (HD) patients. Many similarities between the PD and HD groups emerged, including impaired immediate memory spans, inconsistency of recall across learning trials, deficient use of a semantic clustering learning strategy, elevated intrusion rates on delayed recall, impaired recognition memory performance, normal retention of information over delay periods, normal vulnerability to proactive or retroactive interference, and normal types of intrusion errors. The HD subjects, however, displayed inferior free recall, deficient improvement across learning trials, abnormal serial position recall effects, higher perseveration rates, and supranormal improvement on recognition testing compared with free recall. Implications of these results for characterizing memory deficits associated with subcortical system dysfunction are discussed. PMID:2147923

  5. 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

  6. Frontotemporal dementia and parkinsonism linked to chromosome 17 - the first Polish family

    PubMed Central

    Narożańska, Ewa; Jasińska-Myga, Barbara; Sitek, Emilia J.; Robowski, Piotr; Brockhuis, Bogna; Lass, Piotr; Dubaniewicz, Mirosława; Wieczorek, Dariusz; Baker, Matt; Rademakers, Rosa; Wszolek, Zbigniew K.; Sławek, Jarosław

    2010-01-01

    Background Frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17) is a neurodegenerative disorder with various clinical phenotypes. We present the first Central-Eastern European family (Gdansk Family) with FTDP-17 due to a P301L mutation in MAPT. Methods We have studied a family consisting of 82 family members, 39 of whom were genetically evaluated. The proband and her affected brother underwent detailed clinical and neuropsychological examinations. Results P301L mutation in MAPT was identified in two affected and five asymptomatic family members. New features included hemispatial neglect and unilateral resting tremor not previously reported for P301L MAPT mutation. Low blood folic acid levels were also detected. Conclusions Our report suggests that FTDP-17 affects patients worldwide, but due to its heterogenous clinical presentation remains underrecognized. PMID:20561037

  7. Tau pathology involves protein phosphatase 2A in parkinsonism-dementia of Guam.

    PubMed

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

    2014-01-21

    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 Tyr(307) 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 Tyr(307) 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 Tyr(307). 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 Tyr(307) by Src. PMID:24395787

  8. 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

  9. Arrestins and two receptor kinases are upregulated in Parkinson's disease with dementia.

    PubMed Central

    ER, Bychkov; VV, Gurevich; JN, Joyce; JL, Benovic; EV, Gurevich

    2008-01-01

    Arrestins and G proteins-coupled receptor kinases (GRKs) regulate signaling and trafficking of G protein-coupled receptors. We investigated changes in the expression of arrestins and GRKs in the striatum of patients with Parkinson's disease without (PD) or with dementia (PDD) at post mortem using Western blotting and ribonuclease protection assay. Both PD and PDD groups had similar degree of dopamine depletion in all striatal regions. Arrestin proteins and mRNAs were increased in the PDD group throughout striatum. Protein and mRNA of GRK5, the major subtype in the human striatum, and GRK3 were also upregulated, whereas GRK2 and 6 were mostly unchanged. The PD group had lower concentration of arrestins and GRKs than the PDD group. There was no statistical link between the load of Alzheimer's pathology and the expression of these signaling proteins. Upregulation of arrestins and GRK in PDD may confer resistance to the therapeutic effects of levodopa often observed in these patients. In addition, increased arrestin and GRK concentrations may lead to dementia via perturbation of multiple signaling mechanisms. PMID:17125886

  10. Parkinson's syndrome after closed head injury: a single case report.

    PubMed

    Doder, M; Jahanshahi, M; Turjanski, N; Moseley, I F; Lees, A J

    1999-03-01

    A 36 year old man, who sustained a skull fracture in 1984, was unconscious for 24 hours, and developed signs of Parkinson's syndrome 6 weeks after the injury. When assessed in 1995, neuroimaging disclosed a cerebral infarction due to trauma involving the left caudate and lenticular nucleus. Parkinson's syndrome was predominantly right sided, slowly progressive, and unresponsive to levodopa therapy. Reaction time tests showed slowness of movement initiation and execution with both hands, particularly the right. Recording of movement related cortical potentials suggested bilateral deficits in movement preparation. Neuropsychological assessment disclosed no evidence of major deficits on tests assessing executive function or working memory, with the exception of selective impairments on the Stroop and on a test of self ordered random number sequences. There was evidence of abulia. The results are discussed in relation to previous literature on basal ganglia lesions and the effects of damage to different points of the frontostriatal circuits. PMID:10084539

  11. Ambulatory Gait Behavior in Patients With Dementia: A Comparison With Parkinson's Disease.

    PubMed

    Yoneyama, Mitsuru; Mitoma, Hiroshi; Sanjo, Nobuo; Higuma, Maya; Terashi, Hiroo; Yokota, Takanori

    2016-08-01

    Accelerometry-based gait analysis is a promising approach in obtaining insightful information on the gait characteristics of patients with neurological disorders such as dementia and Parkinson's disease (PD). In order to improve its practical use outside the laboratory or hospital, it is required to design new metrics capable of quantifying ambulatory gait and their extraction procedures from long-term acceleration data. This paper presents a gait analysis method developed for such a purpose. Our system is based on a single trunk-mounted accelerometer and analytical algorithm for the assessment of gait behavior that may be context dependent. The algorithm consists of the detection of gait peaks from acceleration data and the analysis of multimodal patterns in the relationship between gait cycle and vertical gait acceleration. A set of six new measures can be obtained by applying the algorithm to a 24-h motion signal. To examine the performance and utility of our method, we recorded acceleration data from 13 healthy, 26 PD, and 26 mild cognitive impairment or dementia subjects. Each patient group was further classified into two, comprising 13 members each, according to the severity of the disease, and the gait behavior of the five groups was compared. We found that the normal, PD, and MCI/dementia groups show characteristic walking patterns which can be distinguished from one another by the developed gait measure set. We also examined conventional parameters such as gait acceleration, gait cycle, and gait variability, but failed to reproduce the distinct differences among the five groups. These findings suggest that the proposed gait analysis may be useful in capturing disease-specific gait features in a community setting. PMID:26372429

  12. 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

  13. 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

  14. 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…

  15. 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…

  16. Specificity of transcranial sonography in parkinson spectrum disorders in comparison to degenerative cognitive syndromes

    PubMed Central

    2012-01-01

    Background Hyperechogenicity of the substantia nigra (SN+), detected by transcranial sonography (TCS), was reported as a characteristic finding in Parkinson's disease (PD), with high diagnostic accuracy values, when compared mainly to healthy controls or essential tremor (ET) group. However, some data is accumulating that the SN + could be detected in other neurodegenerative and even in non-neurodegenerative disorders too. Our aim was to estimate the diagnostic accuracy of TCS, mainly focusing on the specificity point, when applied to a range of the parkinsonian disorders, and comparing to the degenerative cognitive syndromes. Methods A prospective study was carried out at the Hospital of Lithuanian University of Health Sciences from January until September 2011. Initially, a TCS and clinical examination were performed on 258 patients and 76 controls. The General Electric Voluson 730 Expert ultrasound system was used. There were 12.8% of cases excluded with insufficient temporal bones, and 4.3% excluded with an unclear diagnosis. The studied sample consisted of the groups: PD (n = 71, 33.2%), ET (n = 58, 27.1%), PD and ET (n = 10, 4.7%), atypical parkinsonian syndromes (APS) (n = 3, 1.4%), hereditary neurodegenerative parkinsonism (HDP) (n = 3, 1.4%), secondary parkinsonism (SP) (n = 23, 10.8%), mild cognitive impairment (MCI) (n = 33, 15.4%), dementia (n = 13, 6.1%), and control (n = 71). Results There were 80.3% of PD patients at stages 1 & 2 according to Hoehn and Yahr. At the cut-off value of 0.20 cm2 of the SN+, the sensitivity for PD was 94.3% and the specificity - 63.3% (ROC analysis, AUC 0.891), in comparison to the rest of the cohort. At the cut-off value of 0.26 cm2, the sensitivity was 90% and the specificity 82.4%. The estimations for the lowest specificity for PD, in comparison to the latter subgroups (at the cut-off values of 0.20 cm2 and 0.26 cm2, respectively) were: 0% and 33.3% to APS, 33.3% and 66.7% to HDP, 34.8% and 69.6% to SP, 55.2% and 82

  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. PMID:22674352

  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. 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

  20. C9orf72 Hexanucleotide Repeat Expansion and Guam Amyotrophic Lateral Sclerosis–Parkinsonism-Dementia Complex

    PubMed Central

    Dombroski, Beth A.; Galasko, Douglas R.; Mata, Ignacio F.; Zabetian, Cyrus P.; Craig, Ulla-Katrina; Garruto, Ralph M.; Oyanagi, Kiyomitsu; Schellenberg, Gerard D.

    2013-01-01

    Importance High-prevalence foci of amyotrophic lateral sclerosis (ALS) and parkinsonism-dementia complex (PDC) exist in Japanese on the Kii Peninsula of Japan and in the Chamorros of Guam. Clinical and neuropathologic similarities suggest that the disease in these 2 populations may be related. Recent findings showed that some of the Kii Peninsula ALS cases had pathogenic C9orf72 repeat expansions, a genotype that causes ALS in Western populations. Objectives To perform genotyping among Guam residents to determine if the C9orf72 expanded repeat allele contributes to ALS-PDC in this population and to evaluate LRRK2 for mutations in the same population. Design and Setting Case-control series from neurodegenerative disease research programs on Guam that screened residents for ALS, PDC, and dementia. Participants Study participants included 24 with ALS and 22 with PDC and 43 older control subjects with normal cognition ascertained between 1956 and 2006. All but one participant were Chamorro, the indigenous people of Guam. A single individual of white race/ethnicity with ALS was ascertained on Guam during the study. Main Outcomes and Measures Participants were screened for C9orf72 hexanucleotide repeat length. Participants with repeat numbers in great excess of 30 were considered to have pathogenic repeat expansions. LRRK2 was screened for point mutations by DNA sequencing. Results We found a single individual with an expanded pathogenic hexanucleotide repeat. This individual of white race/ethnicity with ALS was living on Guam at the time of ascertainment but had been born in the United States. All Chamorro participants with ALS and PDC and control subjects had normal repeats, ranging from 2 to 17 copies. No pathogenic LRRK2 mutations were found. Conclusions and Relevance Unlike participants with ALS from the Kii Peninsula, C9orf72 expansions do not cause ALS-PDC in Chamorros. Likewise, LRRK2 mutations do not cause Guam ALS-PDC. PMID:23588498

  1. Cognitive impairment and dementia in Parkinson's disease: practical issues and management.

    PubMed

    Emre, Murat; Ford, Paul J; Bilgiç, Başar; Uç, Ergun Y

    2014-04-15

    Cognitive impairment and dementia pose particular challenges in the management of patients with Parkinson's disease (PD). Decision-making capacity can render patients vulnerable in a way that requires careful ethical considerations by clinicians with respect to medical decision making, research participation, and public safety. Clinicians should discuss how future decisions will be made as early in the disease course as possible. Because of cognitive, visual, and motor impairments, PD may be associated with unsafe driving, leading to early driving cessation in many. DBS of the STN and, to a lesser degree, globus pallidus interna (GPi) has consistently been associated with decreased verbal fluency, but significant global cognitive decline is usually not observed in patients who undergo rigorous selection. There are some observations suggesting lesser cognitive decline in GPi DBS than STN DBS, but further research is required. Management of PD dementia (PDD) patients involves both pharmacological and nonpharmacological measures. Patients with PDD should be offered treatment with a cholinesterase inhibitor taking into account expected benefits and potential risks. Treatment with neuroleptics may be necessary to treat psychosis; classical neuroleptics, as well as risperidone and olanzapine, should be avoided. Quetiapine might be considered first-line treatment because it does not need special monitoring, although the strongest evidence for efficacy exists for clozapine. Evidence from randomized, controlled studies in the PDD population is lacking; selective serotonin reuptake inhibitors or serotonin-norepinephrine reuptake inhibitors may be used to treat depressive features. Clonazepam or melatonin may be useful in the treatment of rapid eye movement behavior disorder. PMID:24757114

  2. A neurophysiological profile in Parkinson's disease with mild cognitive impairment and dementia in China.

    PubMed

    Wang, Ya-qin; Tang, Bei-sha; Yan, Xin-xiang; Chen, Zhi-heng; Xu, Qian; Liu, Zhen-hua; Li, Kai; Wang, Kai; Guo, Ji-feng

    2015-06-01

    Mild cognitive impairment (MCI) and dementia (D) are frequent features of Parkinson's disease (PD) but widely disparate criteria have been used. Our understanding of the prevalence and cognitive profile of Chinese PD patients remains limited. In order to determine the frequency and pattern of cognitive dysfunction and identify risk factors for cognitive dysfunction in the Chinese Han PD population we performed a cross-sectional study in a cohort of 330 PD patients and 163 healthy controls. Five cognitive domains (executive function, attention, praxis and visuospatial function, memory, and language) and mood/behavior were evaluated. According to the Movement Disorder Society Task Force consensus criteria, up to 29.1% of PD patients were classified as PD-MCI and 32.1% as PD-D. Impairments occur in a range of cognitive domains with dysexecutive profile predominating. Healthy controls also outperformed cognitively preserved PD patients in tasks of executive function and attention. Logistic regression indicated that PD-MCI may be predicted by lower educational level and apathy. Additionally, later disease onset, longer disease duration, more severe motor symptoms and higher neuropsychiatric inventory score were associated with a faster transition from PD-MCI to PD-D. These findings suggest that all PD patients should undergo routine cognitive screening. For high-risk patients early recognition and therapeutic intervention is imperative. PMID:25890772

  3. Tau and α-Synuclein Pathology in Amygdala of Parkinsonism-Dementia Complex Patients of Guam

    PubMed Central

    Forman, Mark S.; Schmidt, M. Luise; Kasturi, Sanjay; Perl, Daniel P.; Lee, Virginia M.-Y.; Trojanowski, John Q.

    2002-01-01

    Amyotrophic lateral sclerosis/parkinsonism-dementia complex (ALS/PDC) is a progressive neurodegenerative disorder of Chamorro residents of Guam and the Mariana Islands, characterized by abundant neuron loss and tau neurofibrillary pathology similar to that observed in Alzheimer’s disease (AD). A variety of neurodegenerative diseases with tau pathology including ALS/PDC also have α-synuclein positive pathology, primarily in the amygdala. We further characterized the tau and α-synuclein pathology in the amygdala of a large series of 30 Chamorros using immunohistochemical and biochemical techniques. Tau pathology was readily detected in both affected and unaffected Chamorros. In contrast, α-synuclein pathology was detected in 37% of patients with PDC but not detected in Chamorros without PDC or AD. The α-synuclein aggregates often co-localized within neurons harboring neurofibrillary tangles suggesting a possible interaction between the two proteins. Tau and α-synuclein pathology within the amygdala is biochemically similar to that observed in AD and synucleinopathies, respectively. Thus, the amygdala may be selectively vulnerable to developing both tau and α-synuclein pathology or tau pathology may predispose it to synuclein aggregation. Furthermore, in PDC, tau and α-synuclein pathology occurs independent of β-amyloid deposition in amygdala thereby implicating the aggregation of these molecules in the severe neurodegeneration frequently observed in this location. PMID:12000724

  4. Asymptomatic Wolff-Parkinson-White Syndrome Should be Ablated.

    PubMed

    Pappone, Carlo; Santinelli, Vincenzo

    2012-09-01

    Wolff-Parkinson-White syndrome (WPW) is associated with a small but lifetime risk of cardiac arrest and/or sudden cardiac death (SCD). However, the exact risk is not well defined, particularly in asymptomatic persons. Over recent years the authors have collected and reported new follow-up data among a large number of asymptomatic WPW patients, particularly children, intensively followed. These data have significantly contributed to the knowledge and definition of the natural history of WPW from childhood to adulthood. The risk of SCD is higher in asymptomatic children than in adults, and early ablation can be offered only to selected subjects after electrophysiologic testing. PMID:26939947

  5. Occurrence of Parkinson's syndrome in type I Gaucher disease.

    PubMed

    Neudorfer, O; Giladi, N; Elstein, D; Abrahamov, A; Turezkite, T; Aghai, E; Reches, A; Bembi, B; Zimran, A

    1996-09-01

    Gaucher disease, the most prevalent glycolipid storage disorder, is classically subdivided into types according to the presence or absence of neurological involvement. Type I has hitherto been considered non-neuronopathic. We present six cases and a review of the literature of Parkinsonian symptoms in type I Gaucher disease patients. The hallmark of this atypical Parkinsonian syndrome is a relatively severe clinical course with early appearance of neurological signs in the 4th to 6th decade of life, aggressive progression of the signs and refractoriness to conventional anti-Parkinson therapy. We discuss the implications of these findings in the light of enzyme replacement therapy for Gaucher disease. PMID:8917744

  6. [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. PMID:11565363

  7. [Clinical and medicine characteristics of patients with Parkinson's syndrome].

    PubMed

    Liu, Huan; Xie, Yan-Ming; Yi, Dan-Hui; Wang, Yong-Yan

    2014-09-01

    This study analyze the characteristics and clinical medicine in 17 hospitals all over China, based on hospital information system diagnostic information database, including 4 497 cases of hospitalized patients with Parkinson's syndrome. Results indicate, the most common comorbidities are infarction, hypertension, coronary heart disease, diabetes and lung infections, including cerebral infarction, the combined incidence of hypertension in men reached 33.46% and 30.05%, respectively, it is slightly lower in the females. Men with coronary heart disease are more than women, women with diabetes and bone disease are more than men. Combined incidence of the disease increases with age, vascular factors occupy an important position. The most common combined diseases in patients with 90 years of age or older are coronary heart disease, lung infection, and often accompanied by metabolic disorders and nutritional emergency, critical care. Constipation, depression, anxiety, sleep disorders, cognitive impairment are common non-motor symptoms. The drug categories associated with Parkinson's core symptoms treatment are about 20% to 30% of clinical medicine, the others are associated with the treatment of combined disease, clinical medicine and disease spectrum consistent. Blood circulation topped Chinese agents applied frequency, reaching 44.52%; laxative drugs accounted for 11.66%; detoxification agent representing 9.46%. The first twenty Chinese medicine of the applying frequency reached 56.07% of the total utilization, including 12 kinds of traditional Chinese medicine injections, accounting for 60%. Therefore, in the diagnosis and treatment of Parkinsons syndrome, the treatment of comorbidities is very important, more attentions should be paid to vascular factors of the disease, Chinese medicine should be more concerned to improve the non-motor symptoms, give full play to the pharmaceutical multi-target, the overall regulation of advantages, integrative medicine, and improve

  8. Dementia

    MedlinePlus

    ... Dementia may also cause changes in mood and personality. Early on, lapses in memory and clear thinking ... to tears to anger in a few minutes. Personality changes. People who have dementia may have drastic ...

  9. 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 ... dementia or repair brain damage, they may improve symptoms or slow down ...

  10. 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. PMID:24084382

  11. [Revisit to Kii ALS--the innovated concept of ALS-Parkinsonism-dementia complex, clinicopathological features, epidemiology and etiology].

    PubMed

    Kuzuhara, Shigeki

    2007-10-01

    The high incidence of amyotrophic lateral sclerosis (ALS) in the residents of Hohara and Kozagawa in the Kii peninsula was reported to have disappeared by early 1980 with its etiology unsolved. However, we found continuous high incidence in Hohara that was neuropathologically characterized by ALS pathology associated with many neurofibrillar tangles (NFTs) similar to Guam ALS. We confirmed existence of neuropathologically-verified parkinsonism-dementia complex (PDC) identical to Guamanian PDC clinically and neuropathologically. The core clinical features consisted of motor neuron signs, parkinsonism and dementia, and patients presented with clinical manifestations of ALS, PDC or PDC followed by ALS. PDC predominated over ALS in incidence. Approximately 70% of patients had family history of ALS/PDC. Neuropathological findings of 12 cases revealed that they were very similar to each others, consisting of many NFTs, no or scanty amyloid plaques, and ALS pathology affecting the upper and lower motor neurons. These findings suggest that ALS and PDC may be different clinical manifestations of a single entity "ALS-parkinsonism-dementia complex". TDP-43 positive inclusions were seen in the neurons of the dentate gyrus and spinal cord in all 6 cases examined. A comparison of age-adjusted prevalence rates in 1967 and 1998 revealed moderate decline of ALS and marked increase of PDC in the latter. The age-adjusted 5-year average incidence rates during 1950 and 2000 showed gradual decline of ALS for 50 years and dramatic increase of PDC after 1990. These findings suggest that the clinical manifestations may have changed in Kii ALS/PDC as in ALS/PDC on Guam, partly because of rapid aging of the population. Gene analyses have so far failed to demonstrate mutations of SOD1, parkin, alpha-synuclein, tau, progranulin, TDP-43 and other genes related to dementia, parkinsonism and motor neuron disease. There have been no differences in drinking water and food between the residents in

  12. 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

  13. Integrative Frequency Power of EEG Correlates with Progression of Mild Cognitive Impairment to Dementia in Parkinson's Disease.

    PubMed

    Gu, Youquan; Chen, Jun; Lu, Yaqin; Pan, Suyue

    2016-04-01

    Clinically, predicting the progression of mild cognitive impairment (MCI) and diagnosing dementia in Parkinson's disease (PD) are difficult. This study aims to explore an integrative electroencephalography (EEG) frequency power that could be used to predict the progression of MCI in PD patients. Twenty-six PD patients, in this study, were divided into the mild cognitive impairment group (PDMCI, 17 patients) and dementia group (PDD, 9 patients) according to cognitive performance. Beta peak frequency, alpha relative power, and alpha/theta power were recorded and analyzed for the prediction. Mini Mental State Examination (MMSE) scores at initiation, in the first year, and in the second year were examined. The sensitivity, specificity, positive predictive value, Matthew correlation coefficient, and positive likelihood ratio were calculated in both the integrative EEG biomarkers and single best biomarker. Of the 17 patients with MCI for 2 years, 6 progressed to dementia. Integrative EEG biomarkers, mainly associated with beta peak frequency, can predict conversion from MCI to dementia. These biomarkers had sensitivity of 82% and specificity of 78%, compared with sensitivity of 61% and specificity of 58% of the beta peak frequency. In conclusion, the integrative EEG frequency powers were more sensitive and specific to MCI progression in PD patients. PMID:25519446

  14. The brainstem pathologies of Parkinson's disease and dementia with Lewy bodies.

    PubMed

    Seidel, Kay; Mahlke, Josefine; Siswanto, Sonny; Krüger, Reijko; Heinsen, Helmut; Auburger, Georg; Bouzrou, Mohamed; Grinberg, Lea T; Wicht, Helmut; Korf, Horst-Werner; den Dunnen, Wilfred; Rüb, Udo

    2015-03-01

    Parkinson's disease (PD) and dementia with Lewy bodies (DLB) are among the human synucleinopathies, which show alpha-synuclein immunoreactive neuronal and/or glial aggregations and progressive neuronal loss in selected brain regions (eg, substantia nigra, ventral tegmental area, pedunculopontine nucleus). Despite several studies about brainstem pathologies in PD and DLB, there is currently no detailed information available regarding the presence of alpha-synuclein immunoreactive inclusions (i) in the cranial nerve, precerebellar, vestibular and oculomotor brainstem nuclei and (ii) in brainstem fiber tracts and oligodendroctyes. Therefore, we analyzed the inclusion pathologies in the brainstem nuclei (Lewy bodies, LB; Lewy neurites, LN; coiled bodies, CB) and fiber tracts (LN, CB) of PD and DLB patients. As reported in previous studies, LB and LN were most prevalent in the substantia nigra, ventral tegmental area, pedunculopontine and raphe nuclei, periaqueductal gray, locus coeruleus, parabrachial nuclei, reticular formation, prepositus hypoglossal, dorsal motor vagal and solitary nuclei. Additionally we were able to demonstrate LB and LN in all cranial nerve nuclei, premotor oculomotor, precerebellar and vestibular brainstem nuclei, as well as LN in all brainstem fiber tracts. CB were present in nearly all brainstem nuclei and brainstem fiber tracts containing LB and/or LN. These findings can contribute to a large variety of less well-explained PD and DLB symptoms (eg, gait and postural instability, impaired balance and postural reflexes, falls, ingestive and oculomotor dysfunctions) and point to the occurrence of disturbances of intra-axonal transport processes and transneuronal spread of the underlying pathological processes of PD and DLB along anatomical pathways. PMID:24995389

  15. Wolff-Parkinson-White syndrome and sudden cardiac death.

    PubMed

    Prystowsky, E N; Fananapazir, L; Packer, D L; Thompson, K A; German, L D

    1987-01-01

    Every year, individuals with no history of heart disease succumb to sudden cardiac death (SCD). Pathologic examination of the hearts usually reveals various forms of heart disease as hypertrophic cardiomyopathy or coronary artery disease. In other cases, however, there is no obvious structural heart disease, and it is possible that some of these individuals died because of a cardiac arrhythmia involving an accessory pathway. If this were the case, the most likely scenario would be onset of atrioventricular reciprocating tachycardia (AVRT), degeneration of the AVRT into atrial fibrillation with a rapid ventricular response over the accessory pathway, and subsequent death caused by the development of ventricular fibrillation. Although these events have been documented, albeit rarely, during intracardiac electrophysiologic studies, in reality very little is known about the natural history of asymptomatic and untreated patients with Wolff-Parkinson-White (WPW) syndrome. In fact, SCD in a previously asymptomatic patient with WPW syndrome is probably relatively rare. Whether asymptomatic WPW patients should undergo electrophysiologic or pharmacologic testing to determine their 'potential' to develop serious cardiac arrhythmias is controversial. The present paucity of data concerning the natural history of WPW syndrome in asymptomatic patients militates against successful identification of those patients who are at risk for sudden death. Long-term prospective studies are necessary to clarify which asymptomatic patients with WPW syndrome require treatment. PMID:3621280

  16. 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…

  17. 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…

  18. Treatable Dementias

    PubMed Central

    Mahler, Michael E.; Cummings, Jeffrey L.; Benson, D. Frank

    1987-01-01

    Dementia is an acquired impairment of intellect produced by brain dysfunction. In the past, dementia was regarded as inevitably chronic, progressive and irreversible. More recently dementia has been viewed as a clinical syndrome that may be produced by both irreversible and reversible conditions. Recognition of the presence of a dementia syndrome should be followed by an evaluation for potentially treatable causes of the intellectual deterioration. Dementia treatment includes therapy for reversible or curable dementias and nonspecific interventions that may improve the condition of patients with progressive dementia syndromes. PMID:3617715

  19. Down syndrome and dementia: a randomized, controlled trial of antioxidant supplementation.

    PubMed

    Lott, Ira T; Doran, Eric; Nguyen, Vinh Q; Tournay, Anne; Head, Elizabeth; Gillen, Daniel L

    2011-08-01

    Individuals with Down syndrome over age 40 years are at risk for developing dementia of the Alzheimer type and have evidence for chronic oxidative stress. There is a paucity of treatment trials for dementia in Down syndrome in comparison to Alzheimer disease in the general (non-Down syndrome) population. This 2-year randomized, double-blind, placebo-controlled trial assessed whether daily oral antioxidant supplementation (900 IU of alpha-tocopherol, 200 mg of ascorbic acid and 600 mg of alpha-lipoic acid) was effective, safe and tolerable for 53 individuals with Down syndrome and dementia. The outcome measures comprised a battery of neuropsychological assessments administered at baseline and every 6 months. Compared to the placebo group, those individuals receiving the antioxidant supplement showed neither an improvement in cognitive functioning nor a stabilization of cognitive decline. Mean plasma levels of alpha-tocopherol increased ~2-fold in the treatment group and were consistently higher than the placebo group over the treatment period. Pill counts indicated good compliance with the regimen. No serious adverse events attributed to the treatment were noted. We conclude that antioxidant supplementation is safe, though ineffective as a treatment for dementia in individuals with Down syndrome and Alzheimer type dementia. Our findings are similar to studies of antioxidant supplementation in Alzheimer disease in the general population. The feasibility of carrying out a clinical trial for dementia in Down syndrome is demonstrated. PMID:21739598

  20. 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

  1. Dementia

    MedlinePlus

    ... agitated or see things that are not there. Memory loss is a common symptom of dementia. However, memory loss by itself does not mean you have ... with two or more brain functions, such as memory and language. Although dementia is common in very ...

  2. 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

  3. 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…

  4. 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

  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. 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…

  7. 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…

  8. [Sudden death in the Wolff-Parkinson-White syndrome].

    PubMed

    García-Cosío Mir, F

    1989-04-01

    Clinical electrophysiologic studies in patients with Wolff-Parkinson-White syndrome (WPW) suffering from ventricular fibrillation have shown a high prevalence of short anterograde refractory period of the accessory pathway (less than or equal to 250 ms), short preexcited RR intervals during atrial fibrillation (less than or equal to 250 ms), and multiple accessory pathways. Unfortunately the specificity of these findings is low, as they are present in almost 50% of patients with WPW without a history of ventricular fibrillation, and in 17% of patients with asymptomatic WPW. Pharmacologic and exercise testing detect a population of WPW with a low probability of having a short anterograde refractory period of the accessory pathway, but don't rule-out the ability of these patients to develop very short RR intervals during atrial fibrillation. Natural history studies show that sudden death in WPW occurs with an incidence less than or equal to 1:1,000 per year. The low predictive value of electrophysiologic and noninvasive studies for sudden death, makes then a poor means for screening patients at risk. Some clinical factors, such as the frequency of tachycardias and/or the detection of episodes of atrial flutter or fibrillation are markers of higher sudden death risk, and indications for aggressive electrophysiologic evaluation. PMID:2675223

  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. [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) PMID:7702432

  11. Neuroleptic-induced Parkinson's syndrome: clinical features and results of treatment with levodopa.

    PubMed Central

    Hardie, R J; Lees, A J

    1988-01-01

    Twenty six consecutive patients with neuroleptic-induced Parkinson's syndrome (NIPS) are described. Their median age was 61 years, 60% were female, and most had received chronic neuroleptic medication for psychiatric indications. The clinical features were indistinguishable from idiopathic Parkinson's disease, except for the presence of co-existing orofacial chorea, limb dyskinesia or akathisia which provided an aetiological clue in 11 cases. Complete resolution of NIPS occurred in only two patients, one of whom later developed Parkinson's disease. Sixteen patients were treated with 300-1000 mg levodopa/benserazide for up to 4 years with few adverse effects but therapeutic response was disappointing. PMID:2900293

  12. 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…

  13. Dopamine Dysregulation Syndrome and Deep Brain Stimulation of the Subthalamic Nucleus in Parkinson's Disease

    PubMed Central

    De la Casa-Fages, Beatriz; Grandas, Francisco

    2011-01-01

    Dopamine dysregulation syndrome is a complication of the dopaminergic treatment in Parkinson's disease that may be very disabling due to the negative impact that compulsive medication use may have on patients' social, psychological, and physical functioning. The relationship between subthalamic nucleus deep brain stimulation and dopamine dysregulation syndrome in patients with Parkinson's disease remains unclear. Deep brain stimulation may improve, worsen, or have no effect on preoperative dopamine dysregulation syndrome. Moreover, dopamine dysregulation syndrome may appear for the first time after deep brain stimulation of the subthalamic nucleus. The outcome of postoperative dopamine dysregulation syndrome is poor despite stimulation and medication adjustments. Here we review the phenomenology and neurobiology of this disorder, discuss possible mechanisms that may underlie the diverse outcomes of dopamine dysregulation syndrome after subthalamic nucleus deep brain stimulation, and propose management strategies. PMID:22135744

  14. [A concise history of the Wolff-Parkinson-White syndrome].

    PubMed

    Fazekas, Tamás

    2006-01-01

    In a paper entitled Bundle-branch block with short P-R interval in healthy young people prone to paroxysmal tachycardia (Am Heart J 1930; 5: 685-704), Dr. Louis Wolff (1898-1972), Sir John Parkinson (1885-1976) and Paul Dudley White (1886-1973) described an intricate syndrome. Prior case reports had already pointed out the essentials of this entity, which has borne the eponym (WPW syndrome) since the publication by Levine and Beeson (Am Heart J 1941; 22: 401-409). It was long thought that the first patient with a short PR interval, delta-wave-induced widening of the QRS complex and paroxysmal supraventricular tachycardia (PSVT) was described by Cohn and Fraser in the prestigious cardiological journal edited by Sir Thomas Lewis (1881-1945) in London, UK (Heart 1913/1914; 5: 93-107). Shortly afterwards, Dr János Angyán (1886-1969), a school-founder chairman of medicine (1923-1959) at the University of Pécs, Hungary, also published a clear-cut case of intermittent WPW syndrome in the German periodical Zentralblatt für Herz- und Gefässkrankheiten (1914; 6: 345-349]. In fact, Angyán should be considered the first Hungarian "cardiologist" who dealt steadily with heart diseases and rhythm disturbances. Quite reently, thanks to the activities of Dr Georg von Knorre, Rostock (PACE 2005; 28: 228-230) we have learnt that the earliest documented case of ECGs with ventricular preexcitation and PSVT ("Herzjagen") was published by August Hoffmann (1862-1929) in the 2 Nov 1909 issue of Münchener Medizinische Wochenschrift (56: 2259-2262; Figures 10 and 11). Hoffmann worked as an internist/neurologist and was director of the university hospital in Düsseldorf, Germany. The first successful surgical division of an atrioventricular accessory pathway (bundle of Kent), by Sealy and his coworkers at the Duke University Medical Center in 1967, led to the modern era of curative transcatheter ablation for WPW patients by radiofrequency alternative current or, nowadays, by

  15. 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

  16. Frequency of dementia syndromes with a potentially treatable cause in geriatric in-patients: analysis of a 1-year interval.

    PubMed

    Djukic, Marija; Wedekind, Dirk; Franz, Almuth; Gremke, Melanie; Nau, Roland

    2015-08-01

    In addition to neurodegenerative and vascular causes of dementia, in the differential diagnosis potentially reversible conditions of dementia also must be assessed. Routine laboratory parameters and neuroimaging, which are recommended for the differential diagnosis of suspected dementia by the German S3 Guideline "Dementia", were retrospectively studied in 166 geriatric patients with suspected dementia. Delirium was diagnosed in six patients (3.6%). These six patients were excluded from the study. Of the 160 remaining patients, there were 99 (59.6%) with an already known dementia. In this subgroup of patients, we found a potentially treatable cause of dementia in 18.2%. In the remaining 61 patients (36.8%), the newly diagnosed dementia syndrome was established according to ICD-10 criteria. Potentially reversible causes of the dementia syndrome were found in 19 of these patients (31.1%). The most common cause was depressive pseudodementia in eight patients followed by vitamin B12 deficiency in six patients. A significant amount of our patients showed laboratory or imaging changes suggestive of potentially reversible causes of the dementia syndrome upon admission. The results of our study indicate the importance of careful differential diagnosis of dementia based on the recommendations of guidelines. Although therapy of these potential causes is not always accompanied by a full recovery, the identification and therapy of treatable causes of cognitive deficits are possible even for general practitioners, who often are the primary contact persons of affected individuals. PMID:25716929

  17. Atypical parkinsonism of Japan: amyotrophic lateral sclerosis-parkinsonism-dementia complex of the Kii peninsula of Japan (Muro disease): an update.

    PubMed

    Kuzuhara, Shigeki; Kokubo, Yasumasa

    2005-08-01

    An update of the endemic parkinsonism-dementia complex (PDC) frequently associated with amyotrophic lateral sclerosis (ALS) in the high prevalence ALS focus of the Kii peninsula of Japan is presented. The initial symptom was parkinsonian gait or hypobulia/amnesia, which was followed by akinesia, rigidity, occasional tremor, bradyphrenia, abulia and amnesia, and finally by akinetic mutism. In several years, most of the patients developed ALS symptoms such as muscle atrophy, bulbar palsy, and upper motor neuron signs. Magnetic resonance imaging and computed tomography of the brain showed marked atrophy of the temporal and frontal lobes and the cerebral blood flow reduction on single-photon emission computed tomography. Marked loss of nerve cells associated with abundant neurofibrillar tangles (NFTs) in the entire central nervous system, most predominantly in the brainstem and temporal lobe was characteristic. Concomitant ALS pathology involving the upper and lower motor neurons was common, and senile plaques were absent in most cases. NFTs consisted of twisted tubules on electron microscopy. Western blot of tau protein showed three bands consisting of six tau isoforms, similar to those of Alzheimer's disease. A family history of ALS/PDC was recorded in more than 70% of patients, but no abnormal mutation or polymorphism was found in the genes of SOD1, tau, and apolipoprotein E. Familial nature and continuing morbidity of Kii ALS/PDC suggest that genetic factors may be more likely in its pathogenesis. PMID:16092099

  18. Dementia

    PubMed Central

    2012-01-01

    Introduction Dementia is characterised by chronic, global, non-reversible deterioration in memory, executive function, and personality. Speech and motor function may also be impaired. Methods and outcomes We conducted a systematic review and aimed to answer the following clinical questions: What are the effects of treatments on cognitive symptoms of dementia (Alzheimer's, Lewy body, or vascular)? What are the effects of treatments on behavioural and psychological symptoms of dementia (Alzheimer's, Lewy body, or vascular)? We searched: Medline, Embase, The Cochrane Library, and other important databases up to July 2011 (Clinical Evidence reviews are updated periodically; please check our website for the most up-to-date version of this review). We included harms alerts from relevant organisations such as the US Food and Drug Administration (FDA) and the UK Medicines and Healthcare products Regulatory Agency (MHRA). Results We found 49 systematic reviews, RCTs, or observational studies that met our inclusion criteria. We performed a GRADE evaluation of the quality of evidence for interventions. Conclusions In this systematic review, we present information relating to the effectiveness and safety of the following interventions: acetylcholinesterase inhibitors (donepezil, galantamine, rivastigmine), antidepressants (clomipramine, fluoxetine, imipramine, sertraline), antipsychotics (haloperidol, olanzapine, quetiapine, risperidone), aromatherapy, benzodiazepines (diazepam, lorazepam), cognitive behavioural therapy (CBT), cognitive stimulation, exercise, ginkgo biloba, memantine, mood stabilisers (carbamazepine, sodium valproate/valproic acid), music therapy, non-steroidal anti-inflammatory drugs (NSAIDs), omega 3 (fish oil), reminiscence therapy, and statins. PMID:23870856

  19. Dementia

    PubMed Central

    2010-01-01

    Introduction Dementia is characterised by chronic, global, non-reversible deterioration in memory, executive function, and personality. Speech and motor function may also be impaired. Methods and outcomes We conducted a systematic review and aimed to answer the following clinical question: What are the effects of treatments on cognitive symptoms of dementia (Alzheimer's, Lewy body, or vascular)? What are the effects of treatments on behavioural and psychological symptoms of dementia (Alzheimer's, Lewy body, or vascular)? We searched: Medline, Embase, The Cochrane Library, and other important databases up to April 2008 (Clinical Evidence reviews are updated periodically; please check our website for the most up-to-date version of this review). We included harms alerts from relevant organisations such as the US Food and Drug Administration (FDA) and the UK Medicines and Healthcare products Regulatory Agency (MHRA). Results We found 33 systematic reviews, RCTs, or observational studies that met our inclusion criteria. We performed a GRADE evaluation of the quality of evidence for interventions. Conclusions In this systematic review, we present information relating to the effectiveness and safety of the following interventions: acetylcholinesterase inhibitors (donepezil, galantamine, rivastigmine), antidepressants (clomipramine, fluoxetine, imipramine, sertraline), antipsychotics (haloperidol, olanzapine, quetiapine, risperidone), aromatherapy, benzodiazepines (diazepam, lorazepam), cognitive behavioural therapy (CBT), cognitive stimulation, exercise, ginkgo biloba, memantine, mood stabilisers (carbamazepine, sodium valproate/valproic acid), music therapy, non-steroidal anti-inflammatory drugs (NSAIDs), omega 3 (fish oil), reminiscence therapy, and statins. PMID:21726471

  20. "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…

  1. 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…

  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. 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…

  4. 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.

  5. 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.

  6. How to maintain the oral health of a child with Wolff-Parkinson-White syndrome: a case report

    PubMed Central

    2014-01-01

    Introduction Wolff-Parkinson-White syndrome is one of the most important disorders of the heart conduction system. It is caused by the presence of an abnormal accessory electrical conduction pathway between the atria and the ventricles. Case presentation In the present report, we describe the correct oral health management of a 12-year-old Caucasian girl with Wolff-Parkinson-White syndrome. Conclusions We successfully undertook the dental care of a girl with Wolff-Parkinson-White syndrome, which we describe here. PMID:25269932

  7. Quantitative Electroencephalography as a Diagnostic Tool for Alzheimer's Dementia in Adults with Down Syndrome

    PubMed Central

    Salem, Lise Cronberg; Sabers, Anne; Kjaer, Troels W.; Musaeus, Christian; Nielsen, Martin N.; Nielsen, Anne-Grete; Waldemar, Gunhild

    2015-01-01

    Background Assessment of dementia in individuals with intellectual disability is complex due to great inter-individual variability in cognitive function prior to dementia and a lack of standardized instruments. Studies have indicated that quantitative electroencephalography (qEEG) results may be used as a diagnostic marker for dementia. The aim of this study was to examine the value of qEEG in the diagnostic evaluation of dementia in patients with Down syndrome (DS). Method The study included 21 patients with DS and mild-to-moderate dementia due to Alzheimer's disease (DS-AD) and 16 age-matched adults with DS without cognitive deterioration assessed by the informant-based Dementia Screening Questionnaire in Intellectual Disability (DSQIID). Conventional EEG was performed and analysed quantitatively using fast Fourier transformation. Outcomes were centroid frequency, peak frequency, absolute power, and relative power. Results In several regions of the brain, a significant decrease in the theta-1 band (4-7 Hz) was identified for the centroid frequency. A significant negative correlation was demonstrated between the mean of the centroid frequency of the theta-1 band and the total DSQIID score. Conclusion We found that qEEG can detect a significant decrease in centroid frequency in a sample of patients with DS-AD as compared to a sample of adults with DS and no cognitive deterioration. PMID:26628899

  8. Consistency of handwriting movements in dementia of the Alzheimer's type: a comparison with Huntington's and Parkinson's diseases.

    PubMed

    Slavin, M J; Phillips, J G; Bradshaw, J L; Hall, K A; Presnell, I

    1999-01-01

    Patients with dementia of the Alzheimer's type (DAT) and their matched controls wrote, on a computer graphics tablet, 4 consecutive, cursive letter 'l's, with varying levels of visual feedback: noninking pen and blank paper so that only the hand movements could be seen, noninking pen and lined paper to constrain their writing, goggles to occlude the lower visual field and eliminate all relevant visual feedback, and inking pen with full vision. The kinematic measures of stroke length, duration, and peak velocity were expressed in terms of consistency via a signal-to-noise ratio (M value of each parameter divided by its SD). Irrespective of medication or severity, DAT patients had writing strokes of significantly less consistent lengths than controls', and were disproportionately impaired by reduced visual feedback. Again irrespective of medication or severity, patients' strokes were of significantly less consistent duration, and significantly less consistent peak velocity than controls', independent of feedback conditions. Patients, unlike controls, frequently perseverated, producing more than 4 'l's, or multiple sets of responses, which was not differentially affected by level of visual feedback. The more variable performance of patients supports a degradation of the base motor program, and resembles that of Huntington's rather than Parkinson's disease patients. It may indeed reflect frontal rather than basal ganglia dysfunction. PMID:9989020

  9. 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.

  10. Dysfunction of the locus coeruleus-norepinephrine system and related circuitry in Parkinson's disease-related dementia.

    PubMed

    Del Tredici, Kelly; Braak, Heiko

    2013-07-01

    Although resting tremor, cogwheel rigidity, hypokinesia/bradykinesia and postural instability usually dominate the clinical picture of sporadic Parkinson's disease (PD), both clinical and epidemiological data reveal that a wide variety of additional symptoms impair patients' quality of life considerably, parallel to the chronic progressive neurodegenerative movement disorder. Autopsy based retrospective studies have shown that α-synuclein immunoreactive Lewy pathology (LP) develops in the locus coeruleus (LC) of patients with neuropathologically confirmed sporadic PD, as well as in individuals with incidental (prodromal or premotor) Lewy body disease but not in age and gender matched controls. Using five case studies, this review discusses the possible role of LP (axonopathy, cellular dysfunction and nerve cell loss) in the LC, catecholaminergic tract and related circuitry in the development of PD-related dementia. The contribution of noradrenergic deficit to cognitive dysfunction in PD has been underappreciated. Noradrenergic therapeutic interventions might not only alleviate depressive symptoms and anxiety but also delay the onset of cognitive decline. PMID:23064099

  11. Blue rubber bleb nevus syndrome in a patient with ataxia and dementia.

    PubMed

    Vig, Elizabeth K; Brodkin, Kayla I; Raugi, Gregory J; Gladstone, Hayes

    2002-01-01

    Blue rubber bleb nevus syndrome (BRBNS), an uncommon disorder characterized by cavernous hemangiomas, most often of the skin and gastrointestinal tract, is usually diagnosed during childhood and young adulthood. We made this diagnosis in an octogenarian referred to a geriatric medicine clinic because of concerns about his ability to live independently. Ataxia, dementia, focal neurologic signs, and bluish/purplish vascular nodules on his lips, buccal mucosa, tongue, chest, and neck were noted on physical examination. Magnetic resonance imaging (MRI) revealed an old left parietal infarction, multiple cavernous hemangiomas most densely concentrated in the subcortical structures and cerebellum, and areas of hemosiderin deposition. Skin biopsy findings were consistent with hemangioma. The physical examination, MRI, and skin biopsy made a diagnosis of BRBNS likely. The patient's ataxia, dementia, and other neurologic signs can be explained by previous hemorrhage from the vascular malformations in his brain. Blue rubber bleb nevus syndrome is an uncommon cause of a relatively common geriatric syndrome presentation. PMID:11936246

  12. Multiple cardiac rhabdomyomas, wolff-Parkinson-white syndrome, and tuberous sclerosis: an infrequent combination.

    PubMed

    Castilla Cabanes, Elena; Lacambra Blasco, Isaac

    2014-01-01

    Cardiac rhabdomyomas are benign cardiac tumours and are often associated with tuberous sclerosis. They are often asymptomatic with spontaneus regresion but can cause heart failure, arrhythmias, and obstruction. There have also been a few isolated reports of Wolff-Parkinson-White syndrome occurring in association with tuberous sclerosis and the great majority has been detected in patients with concomitant rhabdomyomas. We report a 12-day-old infant girl with tuberous sclerosis who presented with intraparietal and intracavitary rhabdomyomas with a Wolff-Parkinson-White syndrome (WPW). She represents one of the few published cases of WPW syndrome and tuberous sclerosis and particularly interesting because of intramural rhabdomyomas regression with persistent intracavitary rhabdomyomas after two years of followup. PMID:25328743

  13. Frontotemporal dementia with parkinsonism linked to chromosome 17 with the MAPT R406W mutation presenting with a broad distribution of abundant senile plaques.

    PubMed

    Ishida, Chiho; Kobayashi, Katsuji; Kitamura, Tatsuru; Ujike, Hiroshi; Iwasa, Kazuo; Yamada, Masahito

    2015-02-01

    We report the autopsy results of a patient with familial dementia who was diagnosed as having frontotemporal dementia with parkinsonism linked to chromosome 17 (FTDP-17) with an R406W mutation in the microtubule-associated protein tau (MAPT) gene. This patient showed Alzheimer's disease (AD)-like clinical manifestations from the age of 59, with reduced β-amyloid1-42 (Aβ42 ) and elevated total and phosphorylated tau levels in the cerebrospinal fluid. He did not present with any apparent parkinsonism throughout the disease course. His autopsy at age 73 showed atrophy and neurodegeneration in many brain regions, particularly in the antero-medial temporal cortex and hippocampus, followed by the frontal lobes, with abundant neurofibrillary tangles. In addition, a diffuse distribution of Aβ-positive senile plaques, including many neuritic plaques, was observed and classified as stage C according to the Consortium to Establish a Registry for Alzheimer's Disease (CERAD) criteria. These results suggest that analyzing of the MAPT gene is essential for diagnosing familial dementia, even if amyloid markers such as Aβ42 in the cerebrospinal fluid and amyloid imaging are positive, or if neuropathological findings indicate a diagnosis of AD. PMID:25377499

  14. Catheter ablation of the accessory pathways of the Wolff-Parkinson-White syndrome and its variants.

    PubMed

    Plumb, V J

    1995-01-01

    The basis of arrhythmias in the Wolff-Parkinson-White (WPW) syndrome and its variants is the presence of accessory atrioventricular connections. Those variants include the concealed form of the WPW syndrome, the permanent form of junctional reciprocating tachycardia, and Mahaim preexcitation. In all forms of symptomatic WPW syndrome, catheter ablation of the accessory atrioventricular connections using radiofrequency current has become the treatment of choice. This review traces the development of this therapy, outlines the basics of the technique, summarizes the results reported in the largest series, indicate remaining areas of controversy, and discusses the indications and limitations of radiofrequency ablation therapy. PMID:7871178

  15. Twiddler syndrome in a patient with tremor dominant Parkinson's disease. A case report and literature review.

    PubMed

    Sobstyl, Michał; Ząbek, Mirosław; Górecki, Wojciech; Brzuszkiewicz-Kuźmicka, Grażyna

    2015-01-01

    Twiddler syndrome is described as a spontaneous rotation or intentional external manipulation of implanted cardiac or occasionally deep brain stimulation (DBS) devices. We report this hardware related complication in a patient with tremor dominant Parkinson's disease (PD), who underwent unilateral subthalamic nucleus (STN) DBS and subsequently developed twiddler syndrome. The clinical course of twiddler syndrome in this patient is described. Some surgical nuances which may prevent its occurrence are suggested. Our case report indicates that twiddler syndrome occurs in DBS patients. Impedance check of DBS hardware, plain chest X-ray, or palpation for a knobbly extension lead through the skin above the IPG allows the correct diagnosis and subsequently a prompt surgical revision. Our subsequent literature review revealed only 10 patients with twiddler syndrome in DBS patient population worldwide. This number may suggest that this syndrome may be unrecognized or underreported, given the number of patients with movement disorders implanted with DBS hardware worldwide. PMID:26652885

  16. iTRAQ and multiple reaction monitoring as proteomic tools for biomarker search in cerebrospinal fluid of patients with Parkinson's disease dementia.

    PubMed

    Lehnert, Stefan; Jesse, Sarah; Rist, Wolfgang; Steinacker, Petra; Soininen, Hilkka; Herukka, Sanna-Kaisa; Tumani, Hayrettin; Lenter, Martin; Oeckl, Patrick; Ferger, Boris; Hengerer, Bastian; Otto, Markus

    2012-04-01

    About 30% of patients with Parkinson's disease (PD) develop Parkinson's disease dementia (PDD) in the course of the disease. Until now, diagnosis is based on clinical and neuropsychological examinations, since so far there is no laboratory marker. In this study we aimed to find a neurochemical marker which would allow a risk assessment for the development of a dementia in PD patients. For this purpose, we adopted a gel-free proteomic approach (iTRAQ-method) to identify biomarker-candidates in the cerebrospinal fluid (CSF) of patients with PD, PDD and non-demented controls (NDC). Validation of these candidates was then carried out by multiple-reaction-monitoring (MRM) optimised for CSF. Using the iTRAQ-approach, we were able to identify 16 differentially regulated proteins. Fourteen out of these 16 proteins could then be followed-up simultaneously in our optimised MRM-measurement protocol. However only Tyrosine-kinase-non-receptor-type 13 and Netrin-G1 differed significantly between PDD and NDC cohorts. In addition, a significant difference was found for Golgin-160 and Apolipoprotein B-100 between PD and NDC. Apart from possible pathophysiological considerations, we propose that Tyrosine-kinase non-receptor-type 13 and Netrin G1 are biomarker candidates for the development of a Parkinson's disease dementia. Furthermore we suggest that iTRAQ and MRM are valuable tools for the discovery of biomarker in cerebrospinal fluid. However further validation studies need to be done with larger patient cohorts and other proteins need to be checked as well. PMID:22327139

  17. A human granin-like neuroendocrine peptide precursor (proSAAS) immunoreactivity in tau inclusions of Alzheimer's disease and parkinsonism-dementia complex on Guam.

    PubMed

    Wada, Manabu; Ren, Chang-Hong; Koyama, Shingo; Arawaka, Shigeki; Kawakatsu, Shinobu; Kimura, Hideki; Nagasawa, Hikaru; Kawanami, Toru; Kurita, Keiji; Daimon, Makoto; Hirano, Asao; Kato, Takeo

    2004-02-01

    The deposition of tau inclusions is one of the neuropathological hallmarks in neurodegenerative disorders with dementia. We have reported that the N-terminal fragment of a human granin-like neuroendocrine peptide precursor (N-proSAAS) is accumulated in Pick bodies. However, it is unknown whether N-proSAAS is widely accumulated in tau inclusions in other tauopathies. Here, we performed an immunohistochemical examination using antibodies against both the N- and C-terminal sequence of proSAAS in the brains of patients with Alzheimer's disease and parkinsonism-dementia complex on Guam. The antibody against N-proSAAS immunostained neurofibrillary tangles and neuritic plaques in both diseases, whereas the antibody against the C-terminal sequence of proSAAS did not. The results of the present study suggest that N-proSAAS or proSAAS-like molecules were trapped within the tau fibrils and accumulated in tau inclusions. PMID:14746899

  18. Atypical association of semantic dementia, corticobasal syndrome, and 4R tauopathy.

    PubMed

    Clerc, Marie-Therese; Deprez, Manuel; Leuba, Genevieve; Lhermitte, Benoît; Lopez, Ursula; von Gunten, Armin

    2015-02-01

    A 57-year-old male with no family history was diagnosed with semantic dementia. He also showed some unusual cognitive features such as episodic memory and executive dysfunctions, spatial disorientation, and dyscalculia. Rapidly progressive cognitive and physical decline occurred. About 1.5 years later, he developed clinical features of a corticobasal syndrome. He died at the age of 60. Brain autopsy revealed numerous 4R-tau-positive lesions in the frontal, parietal and temporal lobes, basal ganglia, and brainstem. Neuronal loss was severe in the temporal cortex. Such association of semantic dementia with tauopathy and corticobasal syndrome is highly unusual. These findings are discussed in the light of current knowledge about frontotemporal lobar degeneration. PMID:24156410

  19. 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

  20. New perspective on parkinsonism in frontotemporal lobar degeneration.

    PubMed

    Park, Hee Kyung; Chung, Sun J

    2013-05-01

    Frontotemporal dementia (FTD) is the second most common type of presenile dementia. Three clinical prototypes have been defined; behavioral variant FTD, semantic dementia, and progressive nonfluent aphasia. Progressive supranuclear palsy, corticobasal degeneration, and motor neuron disease may possess clinical and pathological characteristics that overlap with FTD, and it is possible that they may all belong to the same clinicopathological spectrum. Frontotemporal lobar degeneration (FTLD) is a clinicopathological syndrome that encompasses a heterogenous group of neurodegenerative disorders. Owing to the advancement in the field of molecular genetics, diagnostic imaging, and pathology, FTLD has been the focus of great interest. Nevertheless, parkinsonism in FTLD has received relatively less attention. Parkinsonism is found in approximately 20-30% of patients in FTLD. Furthermore, parkinsonism can be seen in all FTLD subtypes, and some patients with familial and sporadic FTLD can present with prominent parkinsonism. Therefore, there is a need to understand parkinsonism in FTLD in order to obtain a better understanding of the disease. With regard to the clinical characteristics, the akinetic rigid type of parkinsonism has predominantly been described. Parkinsonism is frequently observed in familial FTD, more specifically, in FTD with parkinsonism linked to chromosome 17q (FTDP-17). The genes associated with parkinsonism are microtubule associated protein tau (MAPT), progranulin (GRN or PGRN), and chromosome 9 open reading frame 72 (C9ORF72) repeat expansion. The neural substrate of parkinsonism remains to be unveiled. Dopamine transporter (DAT) imaging revealed decreased uptake of DAT, and imaging findings indicated atrophic changes of the basal ganglia. Parkinsonism can be an important feature in FTLD and, therefore, increased attention is needed on the subject. PMID:24868417

  1. New Perspective on Parkinsonism in Frontotemporal Lobar Degeneration

    PubMed Central

    Park, Hee Kyung; Chung, Sun J.

    2013-01-01

    Frontotemporal dementia (FTD) is the second most common type of presenile dementia. Three clinical prototypes have been defined; behavioral variant FTD, semantic dementia, and progressive nonfluent aphasia. Progressive supranuclear palsy, corticobasal degeneration, and motor neuron disease may possess clinical and pathological characteristics that overlap with FTD, and it is possible that they may all belong to the same clinicopathological spectrum. Frontotemporal lobar degeneration (FTLD) is a clinicopathological syndrome that encompasses a heterogenous group of neurodegenerative disorders. Owing to the advancement in the field of molecular genetics, diagnostic imaging, and pathology, FTLD has been the focus of great interest. Nevertheless, parkinsonism in FTLD has received relatively less attention. Parkinsonism is found in approximately 20–30% of patients in FTLD. Furthermore, parkinsonism can be seen in all FTLD subtypes, and some patients with familial and sporadic FTLD can present with prominent parkinsonism. Therefore, there is a need to understand parkinsonism in FTLD in order to obtain a better understanding of the disease. With regard to the clinical characteristics, the akinetic rigid type of parkinsonism has predominantly been described. Parkinsonism is frequently observed in familial FTD, more specifically, in FTD with parkinsonism linked to chromosome 17q (FTDP-17). The genes associated with parkinsonism are microtubule associated protein tau (MAPT), progranulin (GRN or PGRN), and chromosome 9 open reading frame 72 (C9ORF72) repeat expansion. The neural substrate of parkinsonism remains to be unveiled. Dopamine transporter (DAT) imaging revealed decreased uptake of DAT, and imaging findings indicated atrophic changes of the basal ganglia. Parkinsonism can be an important feature in FTLD and, therefore, increased attention is needed on the subject. PMID:24868417

  2. Retinal Nerve Fiber Layer Thinning in Alzheimer's Disease: A Case-Control Study in Comparison to Normal Aging, Parkinson's Disease, and Non-Alzheimer's Dementia.

    PubMed

    Pillai, Jagan A; Bermel, Robert; Bonner-Jackson, Aaron; Rae-Grant, Alexander; Fernandez, Hubert; Bena, James; Jones, Stephen E; Ehlers, Justis P; Leverenz, James B

    2016-08-01

    Retinal nerve fiber layer (RNFL) thickness, ganglion cell layer (GCL) thickness, and macular volume (MV) utilizing spectral domain optical coherence tomography (SD-OCT) were compared among patients with Alzheimer's disease (AD) dementia, non-Alzheimer's disease (non-AD) dementia, amnestic mild cognitive impairment (aMCI), Parkinson's disease (PD), and age- and sex-matched controls in a cross-sectional cohort study. A total of 116 participants were diagnosed and evaluated (21 AD, 20 aMCI, 20 non-AD, 20 PD, and 34 controls) after comprehensive neurological, neuropsychology, and magnetic resonance imaging (MRI) volumetric evaluations. Retinal nerve fiber layer thickness, GCL thickness, and MV were measured. Analysis of variance models were used to compare groups on MRI volumetric measures, cognitive test results, and SD-OCT measures. Associations between SD-OCT measures and other measures were performed using mixed-effect models. Spectral domain optical coherence tomography analysis of retinal markers, including RNFL thickness, GCL thickness, and MV, did not differ between amnestic MCI, AD dementia, PD, non-AD, dementia, and age- and sex-matched controls in a well-characterized patient cohort. PMID:26888864

  3. N-methyl D-aspartate (NMDA) receptor antagonists and memantine treatment for Alzheimer's disease, vascular dementia and Parkinson's disease.

    PubMed

    Olivares, David; Deshpande, Varun K; Shi, Ying; Lahiri, Debomoy K; Greig, Nigel H; Rogers, Jack T; Huang, Xudong

    2012-07-01

    Memantine, a partial antagonist of N-methyl-D-aspartate receptor (NMDAR), approved for moderate to severe Alzheimer's disease (AD) treatment within the U.S. and Europe under brand name Namenda (Forest), Axura and Akatinol (Merz), and Ebixa and Abixa (Lundbeck), may have potential in alleviating additional neurological conditions, such as vascular dementia (VD) and Parkinson's disease (PD). In various animal models, memantine has been reported to be a neuroprotective agent that positively impacts both neurodegenerative and vascular processes. While excessive levels of glutamate result in neurotoxicity, in part through the over-activation of NMDARs, memantine-as a partial NMDAR antagonist, blocks the NMDA glutamate receptors to normalize the glutamatergic system and ameliorate cognitive and memory deficits. The key to memantine's therapeutic action lies in its uncompetitive binding to the NMDAR through which low affinity and rapid off-rate kinetics of memantine at the level of the NMDAR-channel preserves the physiological function of the receptor, underpinning memantine's tolerability and low adverse event profile. As the biochemical pathways evoked by NMDAR antagonism also play a role in PD and since no other drug is sufficiently effective to substitute for the first-line treatment of L-dopa despite its side effects, memantine may be useful in PD treatment with possibly fewer side effects. In spite of the relative modest nature of its adverse effects, memantine has been shown to provide only a moderate decrease in clinical deterioration in AD and VD, and hence efforts are being undertaken in the design of new and more potent memantine-based drugs to hopefully provide greater efficacy. PMID:21875407

  4. Brain functional connectivity patterns for emotional state classification in Parkinson's disease patients without dementia.

    PubMed

    Yuvaraj, R; Murugappan, M; Acharya, U Rajendra; Adeli, Hojjat; Ibrahim, Norlinah Mohamed; Mesquita, Edgar

    2016-02-01

    Successful emotional communication is crucial for social interactions and social relationships. Parkinson's Disease (PD) patients have shown deficits in emotional recognition abilities although the research findings are inconclusive. This paper presents an investigation of six emotions (happiness, sadness, fear, anger, surprise, and disgust) of twenty non-demented (Mini-Mental State Examination score >24) PD patients and twenty Healthy Controls (HCs) using Electroencephalogram (EEG)-based Brain Functional Connectivity (BFC) patterns. The functional connectivity index feature in EEG signals is computed using three different methods: Correlation (COR), Coherence (COH), and Phase Synchronization Index (PSI). Further, a new functional connectivity index feature is proposed using bispectral analysis. The experimental results indicate that the BFC change is significantly different among emotional states of PD patients compared with HC. Also, the emotional connectivity pattern classified using Support Vector Machine (SVM) classifier yielded the highest accuracy for the new bispectral functional connectivity index. The PD patients showed emotional impairments as demonstrated by a poor classification performance. This finding suggests that decrease in the functional connectivity indices during emotional stimulation in PD, indicating functional disconnections between cortical areas. PMID:26515932

  5. [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. PMID:1632713

  6. 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. PMID:12858653

  7. A case of surgically corrected Wolff-Parkinson-White syndrome. Clinical and histological data.

    PubMed Central

    Rossi, L; Thiene, G; Knippel, M

    1978-01-01

    A case of type B Wolff-Parkinson-White syndrome, with intractable atrial fibrillation, underwent surgical division of a right-sided accessory atrioventricular bundle of Kent. Pre-excitation and complicating tachyarrhythmias were henceforth abolished for 6 weeks, when the patient died of infective endocarditis. Histological examination showed a divided Kent's accessory atrioventricular pathway and apparently functionless James and Mahaim fibres. Images PMID:656230

  8. 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. PMID:25431454

  9. Cortical Lewy body dementia: clinical features and classification.

    PubMed Central

    Gibb, W R; Luthert, P J; Janota, I; Lantos, P L

    1989-01-01

    Seven patients, aged 65-72 years, are described with dementia and cortical Lewy bodies. In one patient a Parkinsonian syndrome was followed by dementia and motor neuron disease. In the remaining six patients dementia was accompanied by dysphasia, dyspraxia and agnosia. One developed a Parkinsonian syndrome before the dementia, in three cases a Parkinsonian syndrome occurred later, and in two cases not at all. All patients showed Lewy bodies and cell loss in the substantia nigra, locus coeruleus and dorsal vagal nucleus, as in Parkinson's disease. The severity of cell loss in the nucleus basalis varied from mild to severe. Lewy bodies were also present in the parahippocampus and cerebral cortex, but Alzheimer-type pathology was mild or absent, and insufficient for a diagnosis of Alzheimer's disease. Patients with moderate or severe dementia, some with temporal or parietal features, may have cortical Lewy body disease, Alzheimer's disease, or a combination of the two. Cortical Lewy body disease may be associated with dementia in Parkinson's disease more often than realised, but is not necessarily associated with extensive Alzheimer pathology. Images PMID:2467966

  10. [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. PMID:27276720

  11. Impact of acetylcholinesterase inhibitors on the occurrence of acute coronary syndrome in patients with dementia

    PubMed Central

    Wu, Ping-Hsun; Lin, Yi-Ting; Hsu, Po-Chao; Yang, Yi-Hsin; Lin, Tsung-Hsien; Huang, Chia-Tsuan

    2015-01-01

    The study aimed to investigate the association of acetylcholinesterase inhibitors (AChEIs) use with the risk of acute coronary syndrome (ACS). We conducted a population-based retrospective cohort study of dementia patients during 1 January 1999 to 31 December 2008 using the National Health Insurance Database in Taiwan. New AChEI users during the study period were matched with AChEI nonusers in age-matched and gender-matched cohorts. The risk of ACS associated with use of AChEIs was analyzed using modified Kaplan-Meier analysis and Cox proportional hazard models after adjustment for competing death risk. Use of AChEIs was associated with a lower incidence of ACS (212.8/10,000 person-years) compared to the matched reference cohort (268.7/10,000 person-years). The adjusted hazard ratio for ACS in patients with dementia treated with AChEIs was 0.836 (95% confidence interval, 0.750–0.933; P < 0.001). Further sensitivity analysis of different study populations demonstrated consistent results. A statistical dose–response relationship for AChEI use and ACS risk was significant for the patients with dementia. In patients with dementia, AChEI treatment was associated with decreased risk of ACS. PMID:26577589

  12. Impact of acetylcholinesterase inhibitors on the occurrence of acute coronary syndrome in patients with dementia.

    PubMed

    Wu, Ping-Hsun; Lin, Yi-Ting; Hsu, Po-Chao; Yang, Yi-Hsin; Lin, Tsung-Hsien; Huang, Chia-Tsuan

    2015-01-01

    The study aimed to investigate the association of acetylcholinesterase inhibitors (AChEIs) use with the risk of acute coronary syndrome (ACS). We conducted a population-based retrospective cohort study of dementia patients during 1 January 1999 to 31 December 2008 using the National Health Insurance Database in Taiwan. New AChEI users during the study period were matched with AChEI nonusers in age-matched and gender-matched cohorts. The risk of ACS associated with use of AChEIs was analyzed using modified Kaplan-Meier analysis and Cox proportional hazard models after adjustment for competing death risk. Use of AChEIs was associated with a lower incidence of ACS (212.8/10,000 person-years) compared to the matched reference cohort (268.7/10,000 person-years). The adjusted hazard ratio for ACS in patients with dementia treated with AChEIs was 0.836 (95% confidence interval, 0.750-0.933; P < 0.001). Further sensitivity analysis of different study populations demonstrated consistent results. A statistical dose-response relationship for AChEI use and ACS risk was significant for the patients with dementia. In patients with dementia, AChEI treatment was associated with decreased risk of ACS. PMID:26577589

  13. [A case of intermittent Wolff-Parkinson-White syndrome caused by high spinal anesthesia].

    PubMed

    Shiroyama, K; Nakagawa, I; Izumi, H; Kurokawa, H; Kuroda, M

    1994-04-01

    We experienced a case of intermittent Wolff-Parkinson-White (WPW) syndrome following spinal anesthesia. This patient had neither any past history of cardiac symptoms nor any abnormal finding in the preoperative electrocardiogram. Soon after spinal anesthesia, the level of anesthesia spread to C6. Both abbreviated PR intervals and delta waves characteristic of WPW syndrome appeared on the electrocardiogram monitor. These abnormal wave-forms continued throughout the operation, but disappeared after three days. This case was diagnosed as intermittent WPW syndrome based upon these observations. High spinal anesthesia effectively blocks the cardiac sympathetic nerve and suppresses the normal atrioventricular conduction. Further, conduction by the accessory pathway is facilitated by the relative excitement of parasympathetic nerve. In the present case, the conduction by the accessory pathway, which had originally been very poor, was transiently promoted by the above-mentioned mechanism, and abnormal wave-forms characteristic of WPW syndrome appeared temporarily in the electrocardiogram. PMID:8189627

  14. Parkinson syndrome. A significant risk factor in the patient with acute surgical disorder.

    PubMed

    Simon, D; Shapira, O M; Mor, E; Pfefferman, R

    1992-01-01

    Ten Parkinsonic patients presenting with acute surgical disease were studied to determine the effects of both conditions on each other and the patient's outcome. Severe motion and communication disturbances led, invariably, to a delay in seeking medical assistance, with most of the patients' presenting symptoms and signs being non-specific and misleading. As a result half the patients presented already in a state of septic shock, and a correct preoperative diagnosis could be achieved in three patients only. The functional status significantly deteriorated in six patients, in four of whom a prolonged rehabilitation course was necessary. Although there was no immediate perioperative mortality, morbidity was significant as 50% and 100% major "surgery-related" and "Parkinson-related" complications accordingly. It is concluded that the coexistence of acute surgical disease with Parkinson syndrome has a profound adverse effect on the patient's outcome. High index of suspicion, early mobilisation, intense physiotherapy and early resumption of the anti-Parkinson drugs are the key points in the management of these patients. PMID:1478816

  15. Handwriting changes due to aging and Parkinson's syndrome.

    PubMed

    Walton, J

    1997-08-22

    Wills signed by elderly people are often contested on the grounds the the signature is different from their earlier specimen signatures. Neurological disease, which can affect handwriting, is very common and progressive amongst elderly people. Handwriting change due to old age and neurological disease is poorly understood. To better understand this subject, we carried out a large methodical study based on almost 200 handwriting specimens of Parkinson patients and age-matched controls. Interestingly, our findings indicate that some of the handwriting changes which occur in these populations tend to resemble forgery indicia although upon close inspection they are distinguishable from them. Thus, document examiners are urged to exercise caution in assessing purported forgeries on wills and other documents signed of written during older age or a writer suffering from neurological disease. PMID:9291592

  16. Genetics Underlying Atypical Parkinsonism and Related Neurodegenerative Disorders

    PubMed Central

    Scholz, Sonja W.; Bras, Jose

    2015-01-01

    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. PMID:26501269

  17. Genetics Underlying Atypical Parkinsonism and Related Neurodegenerative Disorders.

    PubMed

    Scholz, Sonja W; Bras, Jose

    2015-01-01

    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. PMID:26501269

  18. 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. PMID:26831029

  19. Extracorporeal life support for cardiac arrest in a 13-year-old girl caused by Wolff-Parkinson-White syndrome.

    PubMed

    Song, Kyoung Hwan; Lee, Byung Kook; Jeung, Kyung Woon; Lee, Dong Hun

    2015-10-01

    Generally, Wolff-Parkinson-White (WPW) syndrome presents good prognosis. However, several case reports demonstrated malignant arrhythmia or sudden cardiac death as WPW syndrome's first presentation. Cardiopulmonary resuscitation using extracorporeal life support is a therapeutic option in refractory cardiac arrest. We present a WPW syndrome patient who had sudden cardiac arrest as the first presentation of the disease and treated it using extracorporeal life support with good neurologic outcome. PMID:26314214

  20. The use of sugammadex in a pregnant patient with Wolff-Parkinson-White syndrome.

    PubMed

    Sengul, Turker; Saracoglu, Ayten; Sener, Sibel; Bezen, Olgac

    2016-09-01

    Wolff-Parkinson-White (WPW) syndrome is a rare pre-excitation syndrome which develops when atrioventricular conduction occurs through a pathologic accessory pathway known as the bundle of Kent instead of atrioventricular node, hence resulting in tachycardia. Patients with WPW syndrome may experience various symptoms arising from mild-to-moderate chest disease, palpitations, hypotension, and severe cardiopulmonary dysfunction. These patients are most often symptomatic because of cardiac arrhythmias. In this case report, we present an uneventful anesthetic management of a pregnant patient with WPW syndrome undergoing cesarean delivery. A 23-year-old American Society of Anesthesiologists class 2 pregnant patient was diagnosed with WPW syndrome. Her preoperative 12-lead electrocardiogram showed a sinus rhythm at 82 beats per minute, a delta wave, and a short PR interval. After an uneventful surgery, sugammadex 2mg/kg was administered as a reversal agent instead of neostigmine. Then she was discharged to her obstetrics service. Serious hemodynamic disorders may occur in patients with WPW syndrome due to development of fatal arrhythmias. Neostigmine used as a reversal agent in general anesthesia can trigger such fatal arrhythmias by leading changes in cardiac conduction. We believe that sugammadex, which is safely used in many areas in the scope of clinical practice, can be also used for patients diagnosed with WPW syndrome. PMID:27555124

  1. Sugammadex Use in a Patient with Wolff-Parkinson-White (WPW) Syndrome

    PubMed Central

    Şahin, Sevtap Hekimoğlu; Öztekin, İlhan; Kuzucuoğlu, Aytuna; Aslanoğlu, Ayça

    2015-01-01

    Background: Wolff-Parkinson-White (WPW) syndrome is a disease associated with episodes of supraventricular tachycardia and ventricular pre-excitation or atrial fibrillation. WPW is characterized by an aberrant electrical conduction pathway between atria and ventricles. Case Report: The major anesthetic problem connected with WPW syndrome is the risk of tachyarrhythmias due to accessory pathway. Therefore, it has been proposed that the aim of anesthetic management should be the avoidance of tachyarrhythmia and sympathetic stimulation. Sugammadex was administered as a neuromuscular reversal agent in this case. To our knowledge, this is the first case report of sugammadex use in a patient with WPW. This report presents a case of general anesthesia management in a patient with WPW syndrome. Conclusion: We think that it is appropriate to use sugammadex to reverse rocuronium for the prevention of sudden hemodynamic changes in patients with WPW who underwent general anesthesia. PMID:26185726

  2. Systemic sclerosis sine scleroderma associated with Wolff-Parkinson-White syndrome.

    PubMed

    Park, Y-W; Woo, H; Yoon, H-J; Park, H-W; Cho, J-G; Shin, S-S; Lee, S-S

    2007-01-01

    The term "systemic sclerosis sine scleroderma" (ssSSc) has been used to designate a rare progressive systemic sclerosis of visceral organs without skin manifestations. A variety of visceral organs, including the gastrointestinal tract, lung, heart, and kidney, can be involved. We describe a case of 59-year-old female patient with both Wolff-Parkinson-White (WPW) syndrome and ssSSc. She was diagnosed as having ssSSc with Raynaud's phenomenon, anti-nuclear antibody (ANA) and anti-topoisomerase antibody positivity, interstitial pulmonary infiltrates, suspected pulmonary hypertension, subclinical oesophageal dysmotility but no skin thickening. She had a history of paroxysmal tachycardia together with Raynaud's phenomenon and exercise-induced dyspnoea. Electrophysiological study confirmed WPW syndrome with left posterior bypass tract. This case highlights cardiac arrhythmia caused by WPW syndrome as a clinical manifestation of the heart in ssSSc. PMID:17454939

  3. [Wolf-Parkinson-White syndrome in neonatal age].

    PubMed

    Rózsavölgyi, A; Garamvölgyi, G; Szarvas, Z; Büky, B

    1990-12-01

    Description of history data, course of the disease and therapy is given of WPW syndrome diagnosed in neonatal age. In connection with the case the authors deal in details with the etiology, pathomechanism, course and complications of the disease. Problems of differential diagnostics and therapy are discussed. A relatively infrequent case is presented; the importance of pre- and postnatal observation and diagnosis being in close connection with the way of therapy and prognosis is emphasized. PMID:2263353

  4. Mutation of mitochondrial DNA G13513A presenting with Leigh syndrome, Wolff-Parkinson-White syndrome and cardiomyopathy.

    PubMed

    Wang, Shi-Bing; Weng, Wen-Chin; Lee, Ni-Chung; Hwu, Wuh-Liang; Fan, Pi-Chuan; Lee, Wang-Tso

    2008-08-01

    Mutation of mitochondrial DNA (mtDNA) G13513A, encoding the ND5 subunit of respiratory chain complex I, can cause mitochondrial encephalopathy with lactic acidosis and stroke-like episodes (MELAS) and Leigh syndrome. Wolff-Parkinson-White (WPW) syndrome and optic atrophy were reported in a high proportion of patients with this mutation. We report an 18-month-old girl, with an 11-month history of psychomotor regression who was diagnosed with WPW syndrome and hypertrophic cardiomyopathy, in association with Leigh syndrome. Supplementation with coenzyme Q10, thiamine and carnitine prevented further regression in gross motor function but the patient's heart function deteriorated and dilated cardiomyopathy developed 11 months later. She was found to have a mutation of mtDNA G13513A. We suggest that mtDNA G13513A mutation is an important factor in patients with Leigh syndrome associated with WPW syndrome and/or optic atrophy, and serial heart function monitoring by echocardiography is recommended in this group of patients. PMID:19054921

  5. Radiofrequency catheter ablation in patients with Wolff-Parkinson-White syndrome.

    PubMed Central

    Thakur, R K; Klein, G J; Yee, R

    1994-01-01

    OBJECTIVE: To report on the experience with radiofrequency catheter ablation of accessory atrioventricular pathways in patients with Wolff-Parkinson-White syndrome in terms of the duration of fluoroscopy exposure to the patient and the operator and the effect of accessory-pathway location and operator experience on the success rate. DESIGN: Retrospective review. SETTING: Tertiary care university hospital. PATIENTS: Two hundred consecutive patients with Wolff-Parkinson-White syndrome who underwent radiofrequency catheter ablation between September 1990 and June 1992. INTERVENTIONS: Electrophysiologic study and radiofrequency catheter ablation. MAIN OUTCOME MEASURES: Success rate, duration of fluoroscopy, complications and long-term follow-up. RESULTS: Of the 224 accessory pathways in the 200 patients 135 were left free wall, 47 posteroseptal, 32 right free wall and 10 anteroseptal. The overall success rate increased from 53% in the first 3 months of the study period to 96% in the last 3 months. The success rate depended on the location of the accessory pathway. The duration of fluoroscopic exposure decreased from 50 (standard deviation [SD] 21) minutes in the first 3 months to 40 (SD 15) minutes in the last 3 months (p < 0.05). Complications occurred in 3.5% of the patients; they included hemopericardium, cerebral embolism, perforation of the right atrial wall, air embolism in a coronary artery and hematoma at the arterial perforation site. None of the complications resulted in death. CONCLUSIONS: With experience, radiofrequency catheter ablation of accessory pathways can have an overall success rate of more than 95% and a complication rate of less than 4%. Such rates make this procedure suitable for first-line therapy for patients with Wolff-Parkinson-White syndrome. Images Fig. 1 PMID:8087753

  6. Natural history of Wolff-Parkinson-White syndrome diagnosed in childhood.

    PubMed

    Cain, Nicole; Irving, Claire; Webber, Steven; Beerman, Lee; Arora, Gaurav

    2013-10-01

    Wolff-Parkinson-White (WPW) syndrome carries a risk for symptomatic arrhythmias and sudden death. The aim of this study was to examine the natural history of patients with Wolff-Parkinson-White syndrome diagnosed in childhood followed longitudinally at a single institution. The study population consisted of 446 patients. The median age of diagnosis was 7 years, and 61% were male. Associated heart disease was present in 40 patients (9%). Modes of presentation included supraventricular tachycardia (38%), palpitations (22%), chest pain (5%), syncope (4%), atrial fibrillation (0.4%), sudden death (0.2%), and incidental findings (26%); data were unavailable in 4%. During the study period, a total of 243 patients (54%) had supraventricular tachycardia, and 7 patients (1.6%) had atrial fibrillation. Of patients who presented at ≤3 months of age, 35% had resolution of manifest preexcitation compared with 5.8% who presented at >3 months of age (p <0.0001). There were 6 sudden deaths (1.3%), with an incidence of 2.8 per 1,000 patient-years. Two of these patients had structurally normal hearts (incidence 1.1 per 1,000 patient-years). Four of these patients had associated heart disease (incidence 27 per 1,000 patient-years) (p <0.01). In conclusion, in a large population of patients with Wolff-Parkinson-White syndrome diagnosed in childhood, 64% had symptoms at presentation, and an additional 20% developed symptoms during follow-up. There were 6 sudden deaths (1.3%), with an overall incidence of 1.1 per 1,000 patient-years in patients with structurally normal hearts and 27 per 1,000 patient-years in patients with associated heart disease. PMID:23827401

  7. 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

  8. Spinal Anaesthesia is Safe in a Patient with Wolff-Parkinson-White Syndrome Undergoing Evacuation of Molar Pregnancy.

    PubMed

    Deviseti, Pravalika; Pujari, Vinayak S

    2016-02-01

    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. Safinamide for the treatment of Parkinson's disease, epilepsy and restless legs syndrome.

    PubMed

    Chazot, Paul L

    2007-07-01

    Merck Serono SA (formerly Serono), under license from Newron Pharmaceuticals SpA (following its acquisition of the rights from Pharmacia and Upjohn AB [now Pfizer Inc]), is developing the oral alpha-aminoamide derivative of milacemide, safinamide, a monoamine oxidase-B and glutamate release inhibitor, for the potential treatment of Parkinson's disease, epilepsy and restless legs syndrome. In March 2007, plans to develop the agent for the potential treatment of other cognitive disorders, such as Alzheimer's disease, were being finalized and testing was expected to begin before the end of that year. PMID:17659477

  10. Noninvasive diagnostic mapping of supraventricular arrhythmias (Wolf-Parkinson-White syndrome and atrial arrhythmias).

    PubMed

    Cakulev, Ivan; Sahadevan, Jayakumar; Waldo, Albert L

    2015-03-01

    The 12-lead electrocardiogram has limited value in precisely identifying the origin of focal or critical component of reentrant arrhythmias during supraventricular arrhythmias, as well as precisely locating accessory atrioventricular conduction pathways. Because of these limitations, efforts have been made to reconstruct epicardial activation sequences from body surface measurements obtained noninvasively. The last decade has registered significant progress in obtaining clinically useful data from the attempts to noninvasively map the epicardial electrical activity. This article summarizes the recent advances made in this area, specifically addressing the clinical outcomes of such efforts relating to atrial arrhythmias and Wolf-Parkinson-White syndrome. PMID:25784024

  11. Ebstein's anomaly presenting as Wolff-Parkinson white syndrome in a postpartum patient.

    PubMed

    Mathew, S T; Matthew, S T; Federico, G F; Singh, B K

    2003-01-01

    Ebstein's anomaly is a common congenital abnormality in the Wolff-Parkinson white syndrome (WPW). The term WPW is applied to patients with both preexcitation on ECG and paroxysmal tachycardias. In this case review, we describe a female with a history of intermittent palpitations who presented in the postpartum period with WPW. Subsequent testing revealed an underlying Ebstein's anomaly. In the United States, heart disease is responsible for 10% of maternal deaths. Although pregnancy is well known to exacerbate symptoms in patients with WPW, postpartum exacerbation has not been clearly described. This unusual case suggests that monitoring beyond the peurperium would be advisable in patients at risk to develop malignant tachyarrhythmias. PMID:12852798

  12. [Eosinophilic fasciitis associated with Parkinson's syndrome - a case report].

    PubMed

    Budisin, Vesna; Vrabec-Matković, Dragica; Milavac-Puretić, Visnja

    2013-01-01

    We present a 67 year old patient with erythema and swelling of the right arm. Suspected erysipelas and lymphedema are diagnosed at infectious department in the second month of the disease. He was treated with parenteral antibiotics (clindamycin + quinolon). After that, he was hospitalized at rheumatology department as right hand lymphedema, condition after erysipelas, cervicobrachial syndrome and ulnar epicondylitis of right elbow. Lymphatic drainage of right hand was performed, but with no effect. In the seventh month of the disease, the diagnosis of eosinophilic faciitis was established and started treatment with corticosteroids. Besides mentioned, dizziness, tremor, balance disorders, impaired hearing and pain in the cervical and lumbar spine were apeared. The therapy was introduced with levodopa and ropanirol and there is a slight improvement of neurological manifestations of extrapyramidal syndrome. After 18 months of disease the patient has a contracture of his right shoulder, induration and painful movements right forearm, pronounced tremor of the head and hands, balance disorders, neck pain and back pain, difficulty in walking. PMID:24003683

  13. [Frequent arrhythmias in Wolff-Parkinson-White syndrome].

    PubMed

    Cárdenas, M

    1990-01-01

    The real knowledge of the natural history in preexcitation syndrome is not possible, Circus movement reciprocal atrioventricular tachycardia is seen en 60% of the patients. Mostly of them are orthodromic. In this group, A-H increases in duration. H-V become normal and the delta wave disappears in the electrophysiologic study during the tachycardia. In the antidromic group, A-delta decreases and H deflexion cannot be recognized. Pre-excitation with atrial flutter is a rare but very dangerous situation when atrial impulses are conducted by the anomalous bundle, because the very high ventricular rate with 250 mseg R-R intervals. The same happens in patients with atrial fibrillation, observed in 5% of the patients with WPW syndrome; this arrhythmia may provoke multiform ventricular tachycardia, ventricular fibrillation and sudden death. Sudden death occurs in 0.1% of asymptomatic patients with WPW in 1% in those with reciprocal tachycardias and in 5.6% of the patients with atrial fibrillation and R-R intervals of 250 mseg of less. PMID:2091550

  14. Environmental risk factors for Parkinson's disease and parkinsonism: the Geoparkinson study

    PubMed Central

    Dick, F D; De Palma, G; Ahmadi, A; Scott, N W; Prescott, G J; Bennett, J; Semple, S; Dick, S; Counsell, C; Mozzoni, P; Haites, N; Wettinger, S Bezzina; Mutti, A; Otelea, M; Seaton, A; Söderkvist, P; Felice, A

    2007-01-01

    Objective To investigate the associations between Parkinson's disease and other degenerative parkinsonian syndromes and environmental factors in five European countries. Methods A case–control study of 959 prevalent cases of parkinsonism (767 with Parkinson's disease) and 1989 controls in Scotland, Italy, Sweden, Romania and Malta was carried out. Cases were defined using the United Kingdom Parkinson's Disease Society Brain Bank criteria, and those with drug‐induced or vascular parkinsonism or dementia were excluded. Subjects completed an interviewer‐administered questionnaire about lifetime occupational and hobby exposure to solvents, pesticides, iron, copper and manganese. Lifetime and average annual exposures were estimated blind to disease status using a job‐exposure matrix modified by subjective exposure modelling. Results were analysed using multiple logistic regression, adjusting for age, sex, country, tobacco use, ever knocked unconscious and family history of Parkinson's disease. Results Adjusted logistic regression analyses showed significantly increased odds ratios for Parkinson's disease/parkinsonism with an exposure–response relationship for pesticides (low vs no exposure, odds ratio (OR) = 1.13, 95% CI 0.82 to 1.57, high vs no exposure, OR = 1.41, 95% CI 1.06 to 1.88) and ever knocked unconscious (once vs never, OR = 1.35, 95% CI 1.09 to 1.68, more than once vs never, OR = 2.53, 95% CI 1.78 to 3.59). Hypnotic, anxiolytic or antidepressant drug use for more than 1 year and a family history of Parkinson's disease showed significantly increased odds ratios. Tobacco use was protective (OR = 0.50, 95% CI 0.42 to 0.60). Analyses confined to subjects with Parkinson's disease gave similar results. Conclusions The association of pesticide exposure with Parkinson's disease suggests a causative role. Repeated traumatic loss of consciousness is associated with increased risk. PMID:17332139

  15. Anaesthetic management of a case of Wolff-Parkinson-White syndrome.

    PubMed

    Kabade, Savitri D; Sheikh, Safiya; Periyadka, Bhavya

    2011-07-01

    We report a case of fibroid uterus with Wolff-Parkinson-White (WPW) syndrome in a 48-year-old female, posted for elective hysterectomy. Patient gave history of short recurrent episodes of palpitation and electrocardiograph confirmed the diagnosis of WPW syndrome. The anaesthetic management of these patients is challenging as they are known to develop life threatening tachyarrhythmia like paroxysmal supra-ventricular tachycardia (PSVT) and atrial fibrillation (AF). Epidural anaesthesia is preferred compared to general anaesthesia to avoid polypharmacy, noxious stimuli of laryngoscopy and intubation. To deal with perioperative complications like PSVT and AF, anti-arrhythmic drugs like adenosine, beta blockers and defibrillator should be kept ready. Perioperative monitoring is essential as patients can develop complications. PMID:22013256

  16. [The history of Wolf-Parkinson-White syndrome and evolution of surgical methods for its management].

    PubMed

    Bokeriia, L A; Revishvili, A Sh; Melikulov, A Kh; Le, T G; Khusainov, R Kh; Glushko, L A

    2009-01-01

    Wolf-Parkinson-White syndrome (WPW syndrome) affects roughly 1% of the population. It usually occurs in subjects with normal heart function but may combine with congenital cardiac failure and cardiomyopathy. Paroxysmal tachycardia is recorded in 40-80% of he WPW patients, largely in the form of reciprocal tachycardia related to circulation of excitation in the atrioventricular junction and Kent's bundle. Development and improvement of surgical methods for the management of supraventricular tachycardia became possible with the advent of transcatheter registration of electrical activity in different heart regions and programmed heart stimulation techniques. Catheter-assisted methods for the treatment of cardiovascular disorders including arrhythmia have been extensively used in recent decades. Transvenous fulguration is one of them replaced at present by radiofrequency ablation (RFA). The discovery of WPW syndrome made possible a new approach to the the problem of sudden death in young age. Treatment of this syndrome by RFA of additional atrioventricular junction in the last 20 years permitted not only to manage the syndrome itself but also to ensure practically complete recovery of the patients. PMID:20143549

  17. 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. PMID:25367918

  18. 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…

  19. Causes of Age-Related Decline in Adaptive Behavior of Adults with Down Syndrome: Differential Diagnoses of Dementia.

    ERIC Educational Resources Information Center

    Prasher, V. P.; Chung, Man Cheung

    1996-01-01

    A study was conducted of 201 adults with Down's syndrome to investigate the differential causes of decline in adaptive behavior. Results indicated that aging, dementia, and severity of mental retardation were significant factors, while absence of a medical illness predicted a higher level of adaptive behavior. (CR)

  20. [Cognitive impairment in Parkinson's disease].

    PubMed

    Tachibana, Hisao

    2013-01-01

    Cognitive impairment is a common finding in Parkinson's disease (PD), even in the early stages. The concept of mild cognitive impairment (MCI) in PD was recently formalized with diagnosis being reached after impairments in neuropsychological tasks become significant in at least one domain. The brain profile of cognitive deficits involves executive functions (e. g., planning, set shifting, set maintenance, problem solving), attention and memory function. Memory deficits are characterized by impairments in delayed recall, temporal ordering and conditional associate learning. PD patients demonstrate relatively preserved recognition. Visuospatial dysfunctions have also been reported, while language is largely preserved. The existence of two distinct mild cognitive syndromes has also been suggested. One of these affects mainly the frontostriatal executive deficits that are modulated by dopaminergic medications and by a genetically determined level of prefrontal cortex dopamine release. The other affects the more-posterior cortical abilities, such as visuospatial and memory functions, and is suggested to be associated with an increased risk for conversion to dementia. Cross-sectional studies have commonly reported dementia in 20-30% of PD patients, although the 8-year cumulative incidence of dementia may be as high as 78%. Factors associated with dementia in PD are age at onset, age at the time of examination, akinetic-rigid form PD, depression, hallucination, rapid eye movement sleep behavioral disorder and severe olfactory deficits. Clinical features generally involve the same type of deficits as those found in MCI patients, which are more severe and more extensive. The phenomenology of the dementia syndrome is similar to that seen in dementia with Lewy bodies, and clinicopathological correlation studies have revealed varying results with regard to neurochemical deficits and the pathological substrate underlying cognitive impairment and dementia. Early cognitive

  1. A rare manifestation of atrial fibrillation in the presence of Wolff-Parkinson-White syndrome: tachycardia-induced cardiomyopathy.

    PubMed

    Değirmencioğu, Aleks; Karakuş, Gültekin; Baysal, Erkan; Zencirci, Ertuğrul; Çakmak, Nazmiye

    2014-03-01

    We report a 68-year-old man who presented with heart failure and atrial fibrillation (AF) with rapid ventricular response and wide QRS complexes. Tachycardia-induced cardiomyopathy (TIC) due to persistent AF developing on the basis of Wolff-Parkinson-White (WPW) syndrome was considered. Signs and symptoms of heart failure improved with restoration of sinus rhythm. This case suggested that persistent AF in a patient with WPW syndrome is one of the rare causes of TIC. PMID:24643151

  2. Dementia with lewy bodies.

    PubMed

    Posner, H; Chin, S; Marder, K

    2001-10-17

    In this case study, we describe the symptoms, neuropsychological testing, and brain pathology of a man with dementia with Lewy bodies. Dementia with Lewy bodies might be the second most common form of degenerative dementia in the elderly. Progressive cognitive decline, well-formed visual hallucinations, and parkinsonism are core features of this disease. This case was marked by preserved verbal expression despite impairment in memory, visuospatial skills, and attention span. Development of visual symptoms and parkinsonism occurred very early in the course of the disease. PMID:14602963

  3. 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. PMID:24528871

  4. 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…

  5. Rapidly progressed acquired immunodeficiency syndrome dementia complex as an initial manifestation.

    PubMed

    Takeuchi, Makoto; Nobukuni, Keigo; Takata, Hiroshi; Kawata, Noriko; Hayashibara, Noriko; Ishizu, Hideki; Takahashi, Kiyoshi

    2005-07-01

    We report a patient with acquired immunodeficiency syndrome dementia complex (ADC) that presented human immunodeficiency virus infection as an initial manifestation. A 34-year-old man developed disturbance of consciousness and severe abulia over 3 months. The CD4 lymphocyte count was 7.9/microl, while human immunodeficiency virus RNA in blood amounted to 4.2 x 10(4) copies/ml. T2-weighted magnetic resonance imaging showed diffusely high signal intensity in the deep white matter of both cerebral hemispheres. On the 20th hospital day, the patient died of sepsis caused by methicillin-resistant Staphylococcus aureus. Autopsy findings in the brain included increased glial cells and multinucleated giant cells in cerebral white matter and subcortical gray matter. These features were compatible with ADC. PMID:16093602

  6. [Syndrome of partial cholinergic deafferentation of the cortical mantle--a concept for describing the brain-behavior relationship in dementia diseases].

    PubMed

    Arendt, T

    1991-03-01

    The identification of morphological and biochemical changes in neurodegenerative disorders with both common and different patterns of neuropsychological dysfunction may help to define the neurobiological substrate of amnesic and dementing disorders, and, furthermore, will give some insight into the neuronal organisation of memory processes. The concept of "subcortical and cortical dementia" and the "cholinergic hypothesis of memory dysfunction" reflect two different theoretical approaches which relate psychopathological disturbances in Alzheimer's disease, Parkinson's disease, Korsakoff's psychosis and related conditions either to structurally or to chemically defined systems of the brain. In order to overcome limitations arising from this dichotomy of structural and chemical approaches to the brain-behaviour-relationship, the concept of a "syndrome of partial cholinergic deafferentation of the cortical mantle" is suggested in the present paper. This concept is supported by evidence derived from the biochemical, morphological and behavioural sequelae of acute and chronic experimental interference with the cholinergic afferentation of the cortical mantle by the application of neurotoxins, by pharmacological intervention and by neurotransplantation in rat. Regarding the cholinergic projection neurons of the basal forebrain and upper brainstem as components of the reticular activating system, the involvement of the cholinergic afferentation of the cortical mantle in the mediation of memory processes and their dysfunction under the conditions of neurodegenerative disorders can be explained on the basis of the "Hippocampal Memory Indexing Theory" of Teyler and DiScenna. PMID:2050315

  7. Association of a Wolff-Parkinson-White syndrome and a fistula from the coronary to the pulmonary artery.

    PubMed

    Fonteyne, W; Van Nooten, G; Jordaens, L

    1993-01-01

    We report the case history of a 52-year-old man with the Wolff-Parkinson-White syndrome and a fistula from the left anterior descending artery to the pulmonary artery. He had a left lateral bypass tract. To our knowledge, this is the first reported case of this association. Arterial malformations, along with vascular malformations of the coronary sinus, can be present in patients with a WPW syndrome. Coronary angiography with attention to the possible presence of arterial and venous malformations is indicated when atypical symptoms or signs are present in the WPW syndrome. PMID:8447186

  8. Cognitive impairment in Parkinson's disease.

    PubMed

    Ransmayr, Gerhard

    2015-12-01

    Parkinson's disease is the second most frequent neurodegenerative disorder. There is significantly elevated risk of cognitive decline and associated neuropsychiatric symptoms. Dementia may develop insidiously several years after manifestation of Parkinson motor symptoms (dementia associated with Parkinson's disease; Parkinson's disease dementia) or in close temporal relationship (within one year) after onset of motor symptoms (Dementia with Lewy bodies). There are clinical, pathophysiological and therapeutic similarities between these two conditions. Men are more frequently affected than women. Risk factor or indicators are advanced age at disease onset, disease duration, rigidity, akinesia and posture and gait impairment and falls as opposed to tremor dominance, and associated neuropsychiatric symptoms (depression, apathy, hallucinosis, delirium). Dementia is treatable with cholinesterase inhibitors (rivastigmine, donepezil), memantine, and adjustment of the pharmacological regimen of parkinsonian motor symptoms. Concomitant autonomic nervous system symptoms and neuropsychiatric complications warrant early clinical awareness and are accessible to pharmacological therapy. PMID:26609664

  9. Exercise testing in children with Wolff-Parkinson-White syndrome: what is its value?

    PubMed

    Dalili, M; Vahidshahi, K; Aarabi-Moghaddam, M Y; Rao, J Y; Brugada, P

    2014-10-01

    This study was conducted to evaluate the accuracy of exercise testing for predicting accessory pathway characteristics in children with Wolff-Parkinson-White (WPW) syndrome. The study enrolled 37 children with WPW syndrome and candidates for invasive electrophysiologic study (EPS). Exercise testing was performed for all the study participants before the invasive study. Data from the invasive EPS were compared with findings from the exercise testing. The sudden disappearance of the delta (Δ) wave was seen in 10 cases (27 %). No significant correlation was found between the Δ wave disappearance and the antegrade effective refractory period of the accessory pathway (AERP-AP) or the shortest pre-excited RR interval (SPERRI). The sensitivity, specificity, and positive and negative predictive values of Δ wave disappearance, based on AERP-AP as gold standard, were respectively 29.4, 80, 71.4, and 40 %. The corresponding values with SPERRI as the gold standard were respectively 23.8, 71.4, 71.4 and 23.8 %. Exercise testing has a medium to low rate of accuracy in detecting low-risk WPW syndrome patients in the pediatric age group. PMID:24728424

  10. Exercise testing in Wolff-Parkinson-White syndrome: case report with ECG and literature review.

    PubMed

    Jezior, Matthew R; Kent, Steven M; Atwood, J Edwin

    2005-04-01

    ECG changes during exercise stress testing, such as false-positive ST-segment depression and disappearance of the delta wave, are reported in patients with the Wolff-Parkinson-White (WPW) pattern. We present a case of exercise testing in a 53-year-old man with WPW syndrome with ischemic-appearing ECG changes and normal nuclear stress perfusion study findings who was thought to be at clinically low risk for having significant coronary disease. A literature review is discussed. Although ST-segment depression typical for ischemia occurs in half of the patients in whom WPW syndrome is reported, exercise testing is still an important tool in their evaluation. Data other than ECG response can be interpreted in the context of clinical history and physical examination findings to stratify the risk of coronary disease. Complete and sudden disappearance of the delta wave has been seen during exercise in 20% of patients with WPW syndrome and can identify those who are at low risk for sudden arrhythmic death. PMID:15821231

  11. Exome analysis of a family with Wolff-Parkinson-White syndrome identifies a novel disease locus.

    PubMed

    Bowles, Neil E; Jou, Chuanchau J; Arrington, Cammon B; Kennedy, Brett J; Earl, Aubree; Matsunami, Norisada; Meyers, Lindsay L; Etheridge, Susan P; Saarel, Elizabeth V; Bleyl, Steven B; Yost, H Joseph; Yandell, Mark; Leppert, Mark F; Tristani-Firouzi, Martin; Gruber, Peter J

    2015-12-01

    Wolff-Parkinson-White (WPW) syndrome is a common cause of supraventricular tachycardia that carries a risk of sudden cardiac death. To date, mutations in only one gene, PRKAG2, which encodes the 5'-AMP-activated protein kinase subunit γ-2, have been identified as causative for WPW. DNA samples from five members of a family with WPW were analyzed by exome sequencing. We applied recently designed prioritization strategies (VAAST/pedigree VAAST) coupled with an ontology-based algorithm (Phevor) that reduced the number of potentially damaging variants to 10: a variant in KCNE2 previously associated with Long QT syndrome was also identified. Of these 11 variants, only MYH6 p.E1885K segregated with the WPW phenotype in all affected individuals and was absent in 10 unaffected family members. This variant was predicted to be damaging by in silico methods and is not present in the 1,000 genome and NHLBI exome sequencing project databases. Screening of a replication cohort of 47 unrelated WPW patients did not identify other likely causative variants in PRKAG2 or MYH6. MYH6 variants have been identified in patients with atrial septal defects, cardiomyopathies, and sick sinus syndrome. Our data highlight the pleiotropic nature of phenotypes associated with defects in this gene. PMID:26284702

  12. Intellectual impairment in Parkinson's disease: clinical, pathologic, and biochemical correlates.

    PubMed

    Cummings, J L

    1988-01-01

    The prevalence of overt dementia in 27 studies representing 4,336 Parkinson's disease (PD) patients was 39.9%. The studies reporting the highest incidence of intellectual impairment (69.9%) used psychologic assessment techniques, whereas studies identifying the lowest prevalence of dementia (30.2%) depended on nonstandardized clinical examinations. Neuropsychologic investigations reveal that PD patients manifest impairment in memory, visuospatial skills, and set aptitude. Language function is largely spared. Intellectual deterioration in PD correlates with age, akinesia, duration, and treatment status. Neuropathologic and neurochemical observations demonstrate that PD is a heterogeneous disorder: the classic subcortical pathology with dopamine deficiency may be complicated by atrophy of nucleus basalis and superimposed cortical cholinergic deficits, and a few patients have the histopathologic hallmarks of Alzheimer's disease. Mild intellectual loss occurs with the classic pathology, and the more severe dementia syndromes have cholinergic alterations or Alzheimer's disease. Thus, PD includes several syndromes of intellectual impairment with variable pathologic and neurochemical correlates. PMID:2908099

  13. [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. PMID:3146959

  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. Syncope in the Wolff-Parkinson-White syndrome: incidence and electrophysiologic correlates.

    PubMed

    Yee, R; Klein, G J

    1984-05-01

    Syncope in the patient with Wolff-Parkinson-White (WPW) syndrome raises the specter of rapid tachyarrhythmias and the possibility of sudden cardiac death. We reviewed the records of 55 consecutive WPW patients referred for electrophysiologic evaluation of known or suspected arrhythmias to determine the incidence and significance of syncope. Twelve patients (22.6%) reported the occurrence of at least one episode of syncope. In eleven (20%) of these, syncope was preceded by rapid palpitations. Forty-three patients (77.4%) had no syncopal episodes. These two groups did not differ significantly with regard to age, sex, presence of associated cardiac or neurologic disease, drug history or accessory pathway location. There was no significant difference in cycle length of reciprocating tachycardia (syncope = 295.6 +/- 59.8 vs non-syncope = 334.5 +/- 59.6 ms, p less than .5), shortest R-R intervals between preexcited beats (260 +/- 78.6 vs 246.7 +/- 55.4 ms, p less than .5) and average R-R interval (364.4 +/- 37.9 vs 367.4 +/- 77.5 ms, p less than .5) measured during atrial fibrillation. The anterograde effective refractory period of the accessory pathway (292.1 +/- 31.9 vs 299 +/- 58.1 ms, p less than .5) and the shortest cycle length with 1:1 conduction over the accessory pathway (306.7 +/- 75 vs 289.1 +/- 77.5 ms, p less than .5) similarly did not differ. We conclude that syncope occurs in approximately 20% of patients with the Wolff-Parkinson-White syndrome referred for assessment of tachycardia. Patients with syncope do not have distinct clinical features or a more malignant electrophysiologic profile, suggesting that other extracardiac factors may play an important role in the genesis of syncope in this group. PMID:6204291

  16. The neuropsychiatry of Parkinson's disease and related disorders.

    PubMed

    Lauterbach, Edward C

    2004-12-01

    Parkinson's disease is associated with classical Parkinsonian features that respond to dopaminergic therapy. Neuropsychiatric sequelae include dementia, major depression, dysthymia, anxiety disorders, sleep disorders, and sexual disorders. Panic attacks are particularly common. With treatment, visual hallucinations, paranoid delusions, mania, or delirium may evolve. Psychosis is a key factor in nursing home placement, and depression is the most significant predictor of quality of life. Clozapine may be the safest treatment for psychotic features, but more research is needed to establish the efficacy of antidepressant treatments. Dementia with Lewy bodies, the second most common dementia in the elderly, may present in association with systematized delusions, depression, or RBD. Early evidence suggests the utility of rivastigmine, donepezil, low-dose olanzapine, and quetiapine in treating DLB. Parkinson-plus syndromes generally lack a good response to dopaminergic treatment and evidence additional features, including dysautonomia, cerebellar and pontine features, eye signs, and other movement disorders. MSA is associated with dysautonomia and RBD. SND (MSA-P) is associated with frontal cognitive impairments, but dementia, psychosis, and mood disorders have not been strikingly apparent unless additional pathological findings are present. In SDS (MSA-A), impotence is almost ubiquitous; urinary incontinence is frequent; depression is occasional, and sleep apnea should be treated to avoid sudden death during sleep. OPCA neuropsychiatric correlates await further definition. Progressive supranuclear palsy neuropsychiatric features include apathy, subcortical dementia, pathological emotionality, mild depression and anxiety, and lack of appreciable response to donepezil. CBD usually is recognized by early frontal dementia with ideomotor apraxia, often in the right upper extremity, attended later by poorly responsive unilateral Parkinsonism, with additional signs including

  17. Grammatical abilities in Parkinson's disease: evidence from written sentences.

    PubMed

    Small, J A; Lyons, K; Kemper, S

    1997-12-01

    This study examined the grammatical content of written sentences elicited from 96 Parkinson's patients, 30 Parkinson's with dementia patients and 167 control subjects. Parkinson's patients without dementia or with mild dementia presented no impairments in sentence length, syntactic complexity or amount of information content. Moderately demented Parkinson's patients showed reduced sentence length and information content but normal syntactic complexity. This pattern of results provides evidence that lexical-semantic content is more susceptible to decline than syntactic structure with the progression of dementia in Parkinson's disease. PMID:9460727

  18. Longitudinal live imaging of retinal α-synuclein::GFP deposits in a transgenic mouse model of Parkinson's Disease/Dementia with Lewy Bodies.

    PubMed

    Price, Diana L; Rockenstein, Edward; Mante, Michael; Adame, Anthony; Overk, Cassia; Spencer, Brian; Duong-Polk, Karen X; Bonhaus, Douglas; Lindsey, James; Masliah, Eliezer

    2016-01-01

    Abnormal α-synuclein (α-syn) accumulation in the CNS may underlie neuronal cell and synaptic dysfunction leading to motor and cognitive deficits in synucleinopathies including Parkinson's disease (PD) and Dementia with Lewy Bodies (DLB). Multiple groups demonstrated α-syn accumulation in CNS accessory structures, including the eyes and olfactory terminals, as well as in peripheral organs of Parkinsonian patients. Retinal imaging studies of mice overexpressing fused α-syn::GFP were conducted to evaluate the presence and progression of retinal pathology in a PD/DLB transgenic mouse model. Bright-field image retinal maps and fluorescent images were acquired at 1-month intervals for 3 months. Retinal imaging revealed the accumulation of GFP-tagged α-syn in retinal ganglion cell layer and in the edges of arterial blood vessels in the transgenic mice. Double labeling studies confirmed that the α-syn::GFP-positive cells were retinal ganglion cells containing α-syn. Accumulation of α-syn persisted in the same cells and increased with age. Accumulation of α-syn::GFP was reduced by immunization with single chain antibodies against α-syn. In conclusion, longitudinal live imaging of the retina in the PDGF-α-syn::GFP mice might represent a useful, non-invasive tool to monitor the fate of α-syn accumulation in the CNS and to evaluate the therapeutic effects of compounds targeting α-syn. PMID:27389831

  19. 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

  20. 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

  1. 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

  2. The behavioural variant frontotemporal dementia (bvFTD) syndrome in psychiatry.

    PubMed

    Lanata, Serggio C; Miller, Bruce L

    2016-05-01

    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

  3. [Dyskinesia-hyperpyrexia syndrome in a patient with Parkinson's disease: a case report].

    PubMed

    Taguchi, Soutarou; Niwa, Jun-ichi; Ibi, Tohru; Doyu, Manabu

    2015-01-01

    Non-physiological, excessive dopaminergic stimulation can cause dyskinesia-hyperpyrexia syndrome (DHS), which was initially reported by Gil-Navarro and Grandas in 2010. A 70-years-old woman with a 13-years history of Parkinson's disease (PD) was hospitalized due to difficulty walking, despite being treated with levodopa/carbidopa (600 mg/day), immediate-release pramipexole (3 mg/day), and selegiline (5 mg/day). Immediate-release pramipexole was changed to extended-release pramipexole without changing the dose or levodopa equivalent dose (LED). The patient's adherence to drugs was good. The parkinsonism gradually improved and the patient was discharged. One month later, the patient developed severe generalized athetotic dyskinesia with visual hallucinations and hyperpyrexia that lasted for a week, and she was readmitted to hospital. On admission, the patient was conscious but slightly disoriented. Body temperature was 40.3°C with hyperhidrosis. Leukocyte count in the peripheral blood was 1.78×10(4)/ml and serum creatine kinase was >3×10(4) U/l. Chest survey, whole-body computed tomography, and cranial magnetic resonance imaging showed no abnormalities. The patient was diagnosed with DHS and treated by tapering the oral administration of dopaminergic drugs, including extended-release pramipexole. Her clinical condition recovered without dyskinesia, and serum creatine kinase level swiftly normalized. DHS and resemblant conditions are reported to occur in long-term PD patients with motor complications. In advanced stage PD, loss of dopaminergic neurons impairs the dopamine holding capacity of the striatum and exogenous dopaminergic drugs can result in uncontrollable and excessive fluctuations in dopamine concentration. Our case recommends caution when switching to long-acting dopaminergic drugs, even if the dose is unchanged, could lead to excessive dopaminergic stimulation. This case highlights the importance of considering both the LED and the duration of

  4. [Effect of high dose pergolide mesilate on restless legs syndrome associated with Parkinson disease].

    PubMed

    Imamura, Akiko; Tsuboi, Yoshio; Tanaka, Miki; Obata, Toyoshi; Yamada, Tatsuo

    2007-04-01

    Restless legs syndrome (RLS) is one of the common nocturnal disturbance seen in Parkinson's disease (PD) patients. The prevalence of RLS with PD is greater than that of general populations; however, etiology of RLS in patients with PD is still controversial. We report a 63-year-old man with PD, who was admitted to our hospital with uncontrollable unpleasant feeling in both legs leading to sleep disturbance. At age 59, he experienced numbness and nocturnal myoclonus in his right foot. One year later, he developed resting tremor and bradykinesia in his right hand, and was diagnosed as PD. Levodopa was initiated with favorable response for his resting tremor and bradykinesia, however, his dysesthesia of the legs spread to both side and associated with an urge to move which occurs at rest and was ameliorated by walking. On admission, his parkinsonism was well controlled by 400 mg/ day of levodopa/benserazide. Polysomnography (PSG) revealed periodic limb movements in sleep (PLMS). Secondary RLS such as drug-induced, iron deficiency and uraemia, was excluded in this patient. Because levodopa did not improve his RLS, additional symptomatic RLS treatment was initiated. Oral dosage with 150 microg pergolide did not have any effect on his RLS symptoms. An increase up to 750 microg pergolide led to a marked reduction of symptoms. Repeated PSG showed significant reduction of PLMS and improved sleep efficacy. Usually, low dose of dopamine agonist is enough to treat RLS occurred in general populations. However, moderate to high dose of dopamine agonists were needed for our patient with RLS, indicating that pharmacological responses might be different between RLS in general and that associated with PD. It is important to consider that PD-related RLS can be treated with high dose dopamine agonist to obtain favorable management of nocturnal disturbances. PMID:17511286

  5. Irritable Bowel Syndrome Is Associated with an Increased Risk of Dementia: A Nationwide Population-Based Study

    PubMed Central

    Chen, Chien-Hua; Lin, Cheng-Li; Kao, Chia-Hung

    2016-01-01

    Purpose Abnormal interaction in the brain–gut axis has emerged as one of the relevant pathophysiological mechanisms for the development of irritable bowel syndrome (IBS). Moreover, the brain–gut axis has recently been demonstrated to be crucial for the maintenance of cognitive performance. Therefore, we assessed the risk of dementia following diagnosis of IBS. Methods Using the Taiwan National Health Insurance Research Database (NHIRD) to obtain medical claims data from 2000 to 2011, we employed a random sampling method to enroll32 298 adult patients with IBS and frequency-matched them according to sex, age, and baseline year with 129 192 patients without IBS. Results The patients with IBS exhibited an increased risk of dementia [adjusted hazard ratio (aHR) = 1.26, 95% confidence interval (CI) = 1.17–1.35]after adjustment for age, sex, diabetes, hypertension, stroke, coronary artery disease (CAD), head injury, depression, and epilepsy, and the overall incidence of dementia for the cohorts with and without IBS was 4.86 and 3.41 per 1000 person-years, respectively. IBS was associated with an increased risk of dementia in patients older than 50 years in both male and female, and in those with comorbidity or without comorbidity. After adjustment for age, sex, and comorbidity, patients with IBS were also more likely to develop either non- Alzheimer’s disease (AD) dementia (aHR = 1.24, 95% CI = 1.15–1.33) or AD (aHR = 1.76, 95% CI = 1.28–2.43). Conclusions IBS is associated with an increased risk of dementia, and this effect is obvious only in patients who are ≥50 years old. PMID:26731277

  6. [Atrial fibrillation in Wolff-Parkinson-White syndrome. Development and therapy].

    PubMed

    Duckeck, W; Kuck, K H

    1993-02-01

    In patients with Wolff-Parkinson-White syndrome the accessory pathway may participate in various tachyarrhythmias thereby influencing symptoms and prognosis. Atrial fibrillation occurs in 10 to 32% of patients and may have life-threatening consequences by precipitating ventricular fibrillation in patients with rapid conduction due to an accessory pathway with short anterograde refractory period (< 250 ms). Pathogenesis of atrial fibrillation in the WPW syndrome and therapeutic options are reviewed in this presentation. Spontaneous degeneration of atrioventricular reentrant tachycardia has been reported to represent the most frequent mode of initiation of atrial fibrillation during electrophysiologic study (up to 64% of episodes). Hemodynamic changes during tachycardia may lead to increased sympathetic tone, hypoxemia or increased tension of the atrial wall, thus, triggering atrial fibrillation. Induction of reentrant tachycardia during electrophysiologic study also has shown to be strongly correlated to its clinical prevalence and is inducible in up to 77% of patients with atrial fibrillation. The pathogenesis and high incidence of atrial fibrillation in patients with WPW syndrome is related to presence and functional properties of the accessory pathway. After surgical excision or catheter ablation more than 90% of patients are free of this arrhythmia. Anterograde conduction properties of the pathway appear to be more important than retrograde properties. High incidence of atrial fibrillation is related to short anterograde refractory periods, and of note, this arrhythmia is rare (3%) in patients with concealed pathways. With intracardiac recordings, Jackman et al. could demonstrate atrial fibrillation due to micro-reentry originating in accessory pathway networks.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:8454253

  7. [A patient with Parkinson's disease complicated by hypothyroidism who developed malignant syndrome after discontinuation of etizolam].

    PubMed

    Kawajiri, Masakazu; Ohyagi, Yasumasa; Furuya, Hirokazu; Araki, Takehisa; Inoue, Naohide; Esaki, Shigemitsu; Yamada, Takeshi; Kira, Junichi

    2002-02-01

    A 59-year-old man, who was diagnosed as having Parkinson's disease and depression seven years ago and was on oral antiparkinsonian agents, antianxiety agents, and antidepressants, developed a high fever, disturbed consciousness, and marked muscle rigidity after discontinuation of etizolam and amitriptyline. He was admitted to a nearby hospital. Hypothyroidism had been noted two months before admission. Marked muscle rigidity and increased serum CK were observed. Since discontinuation of benzodiazepine has been known to rarely trigger a neuroleptic malignant syndrome (NMS), he was diagnosed as having NMS. After receiving dantrolene and bromocriptine, these symptoms temporarily improved but he again developed consciousness disturbance, and convulsive seizures associated with an elevated serum CK. He was transferred to our hospital. On admission, the CK level was normal at 168 IU/l, while free T4 was 0.6 ng/dl (normal range, 0.9-2.3) and TSH was 108.7 mU/ml (normal range, 0.2-4.2) in serum, indicating the presence of primary hypothyroidism. As an increase in thyroid hormone dosage improved the thyroid function to normal level, his disturbed consciousness and muscle rigidity gradually improved. Convulsive seizure and recurrence of NMS in a short interval are unusual in neuroleptic malignant syndrome. In this patient, hypothyroidism may have contributed to the development of malignant syndrome through metabolic changes of the central dopaminergic system, and discontinuation of etizolam, a kind of benzodiazepine, may have triggered NMS, since there has not been reported that discontinuation of antidepressants including amitriptyline triggers NMS. PMID:12424963

  8. 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

  9. 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

  10. Frontotemporal Dementia-Like Syndrome Following Recall of Childhood Sexual Abuse.

    PubMed

    Cohen, Lisa J; Brody, David

    2015-06-01

    Numerous psychopathological syndromes have been attributed to posttraumatic stress, both at the time of the trauma and many years later. To date, however, there is little literature on pseudodementia as a delayed traumatic stress response. The authors present a case history of a 50-year-old woman who developed severe cognitive impairment following retrieval of previously forgotten memories of childhood sexual abuse. Her cognitive condition deteriorated rapidly and dramatically. Neuropsychological assessment and clinical presentation led to a diagnosis of frontotemporal dementia (vs. corticobasal degeneration). Detailed neurologic and medical evaluations could not identify any underlying physical cause. Her condition progressively worsened over 9 months, at which point memantine, an N-methyl-D-aspartate receptor antagonist, was begun. The patient regained full functioning over the next year. Although an organic cause could not be ruled out, it was likely that recovery of traumatic memories was contributory to the patient's condition, as ongoing psychotherapy had begun 1 year into the course. If additional cases with similar presentations are reported, such cases would corroborate the notion that persistent, severe, and reversible cognitive impairment constitutes a previously unrecognized and atypical posttraumatic response. PMID:25991053

  11. 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

  12. Cognitive Dysfunction and Dementia in Primary Sjögren's Syndrome

    PubMed Central

    Blanc, Frederic; Longato, Nadine; Jung, Barbara; Kleitz, Catherine; Di Bitonto, Laure; Cretin, Benjamin; Collongues, Nicolas; Sordet, Christelle; Fleury, Marie; Poindron, Vincent; Gottenberg, Jacques-Eric; Anne, Olivier; Lipsker, Dan; Martin, Thierry; Sibilia, Jean; de Seze, Jérôme

    2013-01-01

    Background. Primary Sjögren's syndrome (PSS) is a frequent systemic autoimmune disease. In this study, we aimed to explore the cognitive impairment and the correlations with brain MRI. Methods. Twenty-five patients (mean age 55 ± 11.8 years, 21 females) with PSS were prospectively selected and tested with a French translation of the Brief Repeatable Battery for Neuropsychological Examination. The results were compared with the scores for 25 matched patients with multiple sclerosis (MS) and 25 controls. Brain lesions were assessed by brain MRI using the Wahlund classification. Results. Fifteen of the 25 PSS patients (60%) presented with cognitive disorders versus 19/25 MS patients (76%). Five patients had dementia in the PSS group. Speed of information processing, attention, immediate and long-term memory, and executive functions were frequently impaired. The mean duration of cognitive complaints was 5.6 ± 6.1 years, and the mean duration of PSS was 15.8 ± 14.0 years. A trend towards a correlation was found between the severity of cognitive impairment and the degree of white matter lesions (WML) (P = 0.03, rho = 0.43). Conclusion. Cognitive impairment—mild or dementia—exists in patients with PSS. Further MRI studies are needed to better understand the precise neural basis of cognitive impairment in PSS patients. PMID:24224097

  13. Lewy body dementias.

    PubMed

    Walker, Zuzana; Possin, Katherine L; Boeve, Bradley F; Aarsland, Dag

    2015-10-24

    The broad importance of dementia is undisputed, with Alzheimer's disease justifiably getting the most attention. However, dementia with Lewy bodies and Parkinson's disease dementia, now called Lewy body dementias, are the second most common type of degenerative dementia in patients older than 65 years. Despite this, Lewy body dementias receive little attention and patients are often misdiagnosed, leading to less than ideal management. Over the past 10 years, considerable effort has gone into improving diagnostic accuracy by refining diagnostic criteria and using imaging and other biomarkers. Dementia with Lewy bodies and Parkinson's disease dementia share the same pathophysiology, and effective treatments will depend not only on successful treatment of symptoms but also on targeting the pathological mechanisms of disease, ideally before symptoms and clinical signs develop. We summarise the most pertinent progress from the past 10 years, outlining some of the challenges for the future, which will require refinement of diagnosis and clarification of the pathogenesis, leading to disease-modifying treatments. PMID:26595642

  14. Thyroid storm in a patient with Wolff-Parkinson-White syndrome.

    PubMed

    Naqvi, Syed Yaseen; Luebbert, Jeffrey J; Rosen, Stephen G

    2015-01-01

    A 44-year-old woman with no medical history presented to the emergency department with a 2 h history of sudden onset chest pressure, palpitations, diaphoresis and shortness of breath. She reported a 90-pound unintentional weight loss, increased appetite, irritability, night sweats and palpitations for 2 months. Physical examination revealed a heart rate (HR) of 269 bpm and a blood pressure of 116/94 mm Hg. Her ECG revealed a wide-complex tachycardia with right bundle branch morphology and an HR of 265 bpm. Intravenous adenosine was administered with resolution of the arrhythmia and symptoms. Her subsequent ECG revealed sinus tachycardia with δ waves, which was consistent with Wolff-Parkinson-White (WPW) syndrome. Laboratory findings confirmed thyroid storm and treatment began with intravenous hydrocortisone, methimazole, metoprolol, amiodarone and iodine drops. Graves' disease was confirmed based on the presence of serum thyroid-stimulating hormone receptor antibody. The patient underwent successful WPW accessory tract ablation 6 weeks after initial presentation. PMID:26670895

  15. [Prognostic significance of syncope in patients with Wolff-Parkinson-White syndrome].

    PubMed

    Auricchio, A; Klein, H; Trappe, H J; Troester, J

    1990-12-01

    The prognostic value of syncope in symptomatic patients with Wolff-Parkinson-White syndrome (WPW) is unknown. Therefore, in order to evaluate the sensibility, specificity, positive and negative predictive value of syncope and compare those values with the one obtained for the shortest RR interval (less than or equal to 250 msec) as well as for the anterograde refractory period of the accessory pathway (less than 270 msec), we reviewed the clinical and electrophysiological data of 158 symptomatic patients with WPW. Fourty-eight patients (30%) reported at least one episode of syncope, and 24 out of 158 patients experienced an aborted sudden death, probably due to rapid conduction via the accessory pathway during atrial fibrillation. Syncope has poor sensibility but high specificity in recognizing an aborted sudden death. However, the syncope demonstrated it had a lower prognostic value when compared with other electrophysiological parameters in correctly identifying patients with a history of ventricular tachycardia and/or fibrillation. In conclusion, the data of this study propose the symptom "syncope" as a frequent event in the history of symptomatic patients with WPW referred to electrophysiological study. Generally its presence does not correctly identify patients who experienced an aborted sudden death. Furthermore, its prognostic value is significantly lower than a shorter RR interval (less than or equal to 250 msec) during atrial fibrillation and an anterograde effective refractory period less than 270 msec. PMID:2083811

  16. Catheter ablation of Wolff-Parkinson-White syndrome associated with congenital absence of inferior vena cava.

    PubMed

    Inama, G; Vergara, G; Gramegna, L; Rillo, M; Fuochi, C; Furlanello, F

    1998-09-01

    In the present report we describe a patient (a 36-year-old woman with 15 year history of supraventricular tachyarrhythmias) with congenital absence of inferior vena cava (IVC) revealed during radiofrequency (RF) catheter ablation procedure for right postero-septal Wolff-Parkinson-White syndrome (WPW). For the absence of IVC, the ablation procedure was more difficult, because we had to perform the ablation with the catheters (the ablator catheter and the coronary sinus catheter) introduced both through the superior vena cava. The application of RF energy (35 Watt for 60 seconds) at successful site abolished accessory pathway conduction. The following day was performed the venous angiography, showing the absence of the IVC and a venous return via paravertebral venous plexus to the azygous vein and superior vena cava into the right atrium. Computer tomography confirmed the absence of the IVC with azygous continuation. The drainage via the azygous system modified the radiological image on chest roentgenogram of the right mediastinal silhouette. During cardiogenesis fusion of the IVC and organisation of the heart occur between the 33rd to 40th embryonic days. It is therefore possible that some unknown teratogenic mechanism at this critical period might have caused, in the patient, both the developmental arrest of IVC and failure of regression of atrio-ventricular anatomical and electrical continuity in the right postero-septal region. PMID:9870026

  17. 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. PMID:22237833

  18. Synergistic effects of ceftriaxone and erythropoietin on neuronal and behavioral deficits in an MPTP-induced animal model of Parkinson's disease dementia.

    PubMed

    Huang, Chiu-Ku; Chang, Yen-Ting; Amstislavskaya, Tamara G; Tikhonova, Maria A; Lin, Chih-Li; Hung, Ching-Sui; Lai, Te-Jen; Ho, Ying-Jui

    2015-11-01

    Both ceftriaxone (CEF) and erythropoietin (EPO) show neuroprotection and cognitive improvement in neurodegenerative disease. The present study was aimed at clarifying whether combined treatment with CEF and EPO (CEF+EPO) had superior neuroprotective and behavioral effects than treatment with CEF or EPO alone in a 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP)-induced Parkinson's disease (PD) rat model. The rats were injected with CEF (5 mg/kg/day), EPO (100 IU/kg/day), or CEF+EPO after MPTP lesioning and underwent the bar-test, T-maze test, and object recognition test, then the brains were taken for histological evaluation. MPTP lesioning resulted in deficits in working memory and in object recognition, but the cognitive deficits were markedly reduced or eliminated in rats treated with CEF or CEF+EPO, with the combination having a greater effect. Lesioning also caused neurodegeneration in the nigrostriatal dopaminergic system and the hippocampal CA1 area and these changes were reduced or eliminated by treatment with CEF, EPO, or CEF+EPO, with the combination having a greater effect than single treatment in the densities of DAergic terminals in the striatum and neurons in the hippocampal CA1 area. Thus, compared to treatment with CEF or EPO alone, combined treatment with CEF+EPO had a greater inhibitory effect on the lesion-induced behavioral and neuronal deficits. To our knowledge, this is the first study showing a synergistic effect of CEF and EPO on neuroprotection and improvement in cognition in a PD rat model. Combined CEF and EPO treatment may have clinical potential for the treatment of the dementia associated with PD. PMID:26296668

  19. 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

  20. Some is not enough: quantifier comprehension in corticobasal syndrome and behavioral variant frontotemporal dementia.

    PubMed

    Morgan, Brianna; Gross, Rachel G; Clark, Robin; Dreyfuss, Michael; Boller, Ashley; Camp, Emily; Liang, Tsao-Wei; Avants, Brian; McMillan, Corey T; Grossman, Murray

    2011-11-01

    Quantifiers are very common in everyday speech, but we know little about their cognitive basis or neural representation. The present study examined comprehension of three classes of quantifiers that depend on different cognitive components in patients with focal neurodegenerative diseases. Patients evaluated the truth-value of a sentence containing a quantifier relative to a picture illustrating a small number of familiar objects, and performance was related to MRI grey matter atrophy using voxel-based morphometry. We found that patients with corticobasal syndrome (CBS) and posterior cortical atrophy (PCA) are significantly impaired in their comprehension of cardinal quantifiers (e.g. "At least three birds are on the branch"), due in part to their deficit in quantity knowledge. MRI analyses related this deficit to temporal-parietal atrophy found in CBS/PCA. We also found that patients with behavioral variant frontotemporal dementia (bvFTD) are significantly impaired in their comprehension of logical quantifiers (e.g. "Some of the birds are on the branch"), associated with a simple form of perceptual logic, and this correlated with their deficit on executive measures. This deficit was related to disease in rostral prefrontal cortex in bvFTD. These patients were also impaired in their comprehension of majority quantifiers (e.g. "At least half of the birds are on the branch"), and this too was correlated with their deficit on executive measures. This was related to disease in the basal ganglia interrupting a frontal-striatal loop critical for executive functioning. These findings suggest that a large-scale frontal-parietal neural network plays a crucial role in quantifier comprehension, and that comprehension of specific classes of quantifiers may be selectively impaired in patients with focal neurodegenerative conditions in these areas. PMID:21930136

  1. 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

  2. Nature and extent of person recognition impairments associated with Capgras syndrome in Lewy body dementia.

    PubMed

    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

  3. SOME IS NOT ENOUGH: QUANTIFIER COMPREHENSION IN CORTICOBASAL SYNDROME AND BEHAVIORAL VARIANT FRONTOTEMPORAL DEMENTIA

    PubMed Central

    Morgan, Brianna; Gross, Rachel; Clark, Robin; Dreyfuss, Michael; Boller, Ashley; Camp, Emily; Liang, Tsao-Wei; Avants, Brian; McMillan, Corey; Grossman, Murray

    2011-01-01

    Quantifiers are very common in everyday speech, but we know little about their cognitive basis or neural representation. The present study examined comprehension of three classes of quantifiers that depend on different cognitive components in patients with focal neurodegenerative diseases. Patients evaluated the truth-value of a sentence containing a quantifier relative to a picture illustrating a small number of familiar objects, and performance was related to MRI grey matter atrophy using voxel-based morphometry. We found that patients with corticobasal syndrome (CBS) and posterior cortical atrophy (PCA) are significantly impaired in their comprehension of Cardinal Quantifiers (e.g. “At least three birds are on the branch”), due in part to their deficit in quantity knowledge. MRI analyses related this deficit to temporal-parietal atrophy found in CBS/PCA. We also found that patients with behavioral variant frontotemporal dementia (bvFTD) are significantly impaired in their comprehension of Logical Quantifiers (e.g. “Some the birds are on the branch”), associated with a simple form of perceptual logic, and this correlated with their deficit on executive measures. This deficit was related to disease in rostral prefrontal cortex in bvFTD. These patients were also impaired in their comprehension of Majority Quantifiers (e.g. “At least half of the birds are on the branch”), and this too was correlated with their deficit on executive measures. This was related to disease in the basal ganglia interrupting a frontal-striatal loop critical for executive functioning. These findings suggest that a large-scale frontal-parietal neural network plays a crucial role in quantifier comprehension, and that comprehension of specific classes of quantifiers may be selectively impaired in patients with focal neurodegenerative conditions in these areas. PMID:21930136

  4. Sleep disorders in Parkinson's disease.

    PubMed

    Thorpy, Michael J

    2004-01-01

    Depression, dementia, and physiologic changes contribute to the high prevalence of sleep disturbances in patients with Parkinson's disease (PD). Antiparkinsonian drugs also play a role in insomnia by increasing daytime sleepiness and affecting motor symptoms and depression. Common types of sleep disturbances in PD patients include nocturnal sleep disruption and excessive daytime sleepiness, restless legs syndrome, rapid eye movement sleep behavior disorder, sleep apnea, sleep walking and sleep talking, nightmares, sleep terrors, and panic attacks. A thorough assessment should include complete medical and psychiatric histories, sleep history, and a 1- to 2-week sleep diary or Epworth Sleepiness Scale evaluation. Polysomnography or actigraphy may also be indicated. Treatment should address underlying factors such as depression or anxiety. Hypnotic therapy for sleep disturbances in PD patients should be approached with care because of the risks of falling, agitation, drowsiness, and hypotension. Behavioral interventions may also be useful. PMID:15259535

  5. Classifying late-onset dementia with MRI: Is arteriosclerotic brain degeneration the most common cause of Alzheimer’s syndrome?

    PubMed Central

    Henry-Feugeas, Marie Cécile; Onen, Fannie; Claeys, Elisabeth Schouman

    2008-01-01

    Our aim was to use early magnetic resonance imaging (MRI) to investigate the causes of cognitive decline in elderly people with mild cognitive impairment (MCI). Baseline structural and flow quantification MR sequences, and clinical and neuropsychological follow-up for at least two years, were performed on 62 elderly subjects with MCI. Of these subjects, 17 progressed to dementia, and 15 of these progressed to dementia of the Alzheimer type (DAT). Conversion to clinically diagnosed DAT was related to six distinct MR profiles, including one profile suggesting severe AD (20% of these converters) and five profiles suggesting severe cerebrovascular dysfunction. Two profiles suggested arteriosclerotic brain degeneration, one profile suggested severe venous windkessel dysfunction, and two suggested marked cerebral hypoperfusion associated with very low craniospinal compliance or marked brain atrophy. As compared with vascular MR type converters, AD MR type converters showed high executive and mobility predementia performances. Severe whole anteromesial temporal atrophy and predominantly left brain atrophy on visual MR analysis was only observed in AD MR type converters. In conclusion, these observations enhance the pathogenic complexity of the Alzheimer syndrome, and suggest that the role of arteriosclerotic brain degeneration in late life dementia is underestimated. PMID:18488889

  6. 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

  7. Ventricular fibrillation development following atrial fibrillation after the ingestion of sildenaphil in a patient with Wolff-Parkinson-White syndrome.

    PubMed

    Inci, Sinan; Izgu, Ibrahim; Aktas, Halil; Dogan, Pinar; Dogan, Ali

    2015-08-01

    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. 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

  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. PMID:27462241

  10. A Rare Case of Parkinson's Disease with Severe Neck Pain Owing to Crowned Dens Syndrome

    PubMed Central

    Takahashi, Teruyuki; Tamura, Masato; Osabe, Keiichi; Tamiya, Takashi; Miki, Kenji; Yamaguchi, Mai; Akira, Kanno; Kamei, Satoshi; Takasu, Toshiaki

    2014-01-01

    Background Pain is regarded as one of the most common nonmotor symptoms in Parkinson's disease (PD). In particular, musculoskeletal pain has been reported as the most common type of PD-associated pain. Crowned dens syndrome (CDS), related to microcrystalline deposition in the periodontoid process, is the main cause of acute or chronic cervical pain. Case Presentation This report describes the case of an 87-year-old woman who had severe bradykinesia, muscle rigidity, gait disturbance and neck pain. Laboratory examination revealed marked elevations of white blood cells (10,100/µl) and C-reactive protein (CRP; 8.63 mg/dl). She was primarily diagnosed with severe and untreated PD, corresponding to Hoehn and Yahr scale score IV, with musculoskeletal pain and urinary tract infection. The patient was treated with antiparkinsonism drugs, antibiotic agents and nonsteroidal anti-inflammatory drugs, but they had only limited effects. Cervical plain computed tomography (CT) scanning detected remarkable crown-like calcification surrounding the odontoid process. Based on CT findings, the patient was diagnosed as having CDS with PD, and was immediately treated with corticosteroid. The severe neck rigidity with pain and the serum CRP level (0.83 mg/dl) of the patient were drastically improved within a week by the additional corticosteroid therapy. Conclusion Severe neck rigidity and bradykinesia in this patient might have strengthened the chondrocalcinosis around the odontoid process. Cervical plain CT scan is necessary and useful for the definitive diagnosis of CDS. CDS should be considered as a differential diagnosis of a possible etiology for musculoskeletal pain related to rigidity and bradykinesia in PD. PMID:24926265

  11. Accuracy of screening instruments for detection of neuropsychiatric syndromes in Parkinson's disease.

    PubMed

    Martinez-Martin, Pablo; Leentjens, Albert F G; de Pedro-Cuesta, Jesus; Chaudhuri, Kallol Ray; Schrag, Anette E; Weintraub, Daniel

    2016-03-01

    Parkinson's disease includes neuropsychiatric manifestations, such as depression, anxiety, apathy, psychosis, and impulse control disorders, which often are unreported by patients and caregivers or undetected by doctors. Given their substantial impact on patients and caregivers as well as the existence of effective therapies for some of these disorders, screening for neuropsychiatric symptoms is important. Instruments for screening have a particular methodology for validation, and their performance is expressed in terms of accuracy compared with formal diagnostic criteria. The present study reviews the attributes of the screening instruments applied for detection of the aforementioned major neuropsychiatric symptoms in Parkinson's disease. A quasi-systematic review (including predefined selection criteria, but not evaluating the quality of the reviewed studies) was carried out on the basis of previous systematic reviews (commissioned by the American Academy of Neurology and the Movement Disorder Society) and made current by conducting a literature search (2005-2014). For depression, 11 scales and questionnaires were shown to be valid for Parkinson's disease screening. The recently developed Parkinson Anxiety Scale and the Geriatric Anxiety Inventory demonstrate satisfactory properties as screening instruments for anxiety, and the Lille Apathy Rating Scale for detection of apathy. No scale adequately screens for psychosis, so a specific psychosis instrument should be developed. The Questionnaire for Impulsive-Compulsive Disorders in Parkinson's Disease (Questionnaire and Rating Scale) are valid for comprehensive screening of impulse control disorders, and the Parkinson's Disease-Sexual Addiction Screening Test for hypersexuality specifically. © 2015 International Parkinson and Movement Disorder Society. PMID:26695691

  12. Secondary parkinsonism

    MedlinePlus

    Parkinsonism - secondary; Atypical Parkinson disease ... to be less responsive to medical therapy than Parkinson disease. ... Unlike Parkinson disease, some types of secondary parkinsonism may stabilize or even improve if the underlying cause is treated. ...

  13. Secondary parkinsonism

    MedlinePlus

    Parkinsonism - secondary; Atypical Parkinson disease ... to be less responsive to medical therapy than Parkinson disease. ... Unlike Parkinson disease, some types of secondary parkinsonism may stabilize or even improve if the underlying cause is treated. Brain ...

  14. Progressive "vascular" corticobasal syndrome due to bilateral ischemic hemispheric lesions.

    PubMed

    Kim, Young-Do; Kim, Joong-Seok; Lee, Eek-Sung; Yang, Dong-Won; Lee, Kwang-Soo; Kim, Yeong-In

    2009-01-01

    We report a patient that presented with the corticobasal syndrome (CBS), including progressive dementia, asymmetric parkinsonism associated with constructional and ideomotor apraxia, action myoclonus and focal hand dystonia. Magnetic resonance imaging of the brain revealed extensive ischemic lesions in both fronto-temporo-parieto-occipital lobes and steno-occlusion of the middle cerebral arteries, bilaterally. This case illustrates that extensive cortical vascular-ischemic lesions may present with symptoms mimicking the corticobasal syndrome. PMID:19755778

  15. 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

  16. Familial Hypertrophic cardiomyopathy with Wolff-Parkinson-White syndrome maps to a locus on chromosome 7q3.

    PubMed Central

    MacRae, C A; Ghaisas, N; Kass, S; Donnelly, S; Basson, C T; Watkins, H C; Anan, R; Thierfelder, L H; McGarry, K; Rowland, E

    1995-01-01

    We have mapped a disease locus for Wolff-Parkinson-White syndrome (WPW) and familial hypertrophic cardiomyopathy (FHC) segregating in a large kindred to chromosome 7 band q3. Although WPW syndrome and FHC have been observed in members of the same family in prior studies, the relationship between these two diseases has remained enigmatic. A large family with 25 surviving individuals who are affected by one or both of these conditions was studied. The disease locus is closely linked to loci D7S688, D7S505, and D7S483 (maximum two point LOD score at D7S505 was 7.80 at theta = 0). While four different FHC loci have been described this is the first locus that can be mutated to cause both WPW and/or FHC. PMID:7657794

  17. 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. PMID:23992123

  18. 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.

  19. 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…

  20. 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…

  1. [Cardioversion for paroxysmal supraventricular tachycardia during lung surgery in a patient with concealed Wolff-Parkinson-White syndrome].

    PubMed

    Sato, Yoshiharu; Nagata, Hirofumi; Inoda, Ayako; Miura, Hiroko; Watanabe, Yoko; Suzuki, Kenji

    2014-10-01

    We report a case of paroxysmal supraventricular tachycardia (PSVT) that occurred during video-assisted thoracoscopic (VATS) lobectomy in a patient with concealed Wolff-Parkinson-White (WPW) syndrome. A 59-year-old man with lung cancer was scheduled for VATS lobectomy under general anesthesia. After inserting a thoracic epidural catheter, general anesthesia was induced with intravenous administration of propofol. Anesthesia was maintained with inhalation of desfurane in an air/oxygen mixture and intravenous infusion of remifentanil. Recurrent PSVT occurred three times, and the last episode of PSVT continued for 50 minutes regardless of administration of antiarrhythmic drugs. Synchronized electric shock via adhesive electrode pads on the patient's chest successfully converted PSVT back to normal sinus rhythm. The remaining course and postoperative period were uneventful. An electrophysiological study performed after hospital discharge detected concealed WPW syndrome, which had contributed to the development of atrioventricular reciprocating tachycardia. Concealed WPW syndrome is a rare, but critical complication that could possibly cause lethal atrial tachyarrhythmias during the perioperative period. In the present case, cardioversion using adhesive electrode pads briefly terminated PSVT in a patient with concealed WPW syndrome. PMID:25693338

  2. 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…

  3. 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. PMID:26054567

  4. Dissociating Statistically–Determined Alzheimer/Vascular Dementia Neuropsychological Syndromes Using White and Gray Neuroradiological Parameters

    PubMed Central

    Price, Catherine C.; Tanner, Jared J.; Schmalfuss, Ilona M.; Brumback, Babette; Heilman, Kenneth M.; Libon, David J.

    2016-01-01

    Objectives To use statistical modeling of neuropsychological data to determine subgroups of dementia patients clinically diagnosed with Alzheimer’s disease (AD) or vascular dementia (VaD) and then, using brain imaging, investigate between group differences in gray and white matter regions of interest. Methods An analysis of neuropsychological functioning was obtained from dementia patients clinically diagnosed with AD/VaD characterized with significant leukoaraiosis (LA) and/or lacunes where a k-means cluster analysis requested a 3-group solution. MRI measures of hippocampal, caudate, ventricular, subcortical lacunar infarction, whole brain volume and LA were analyzed. Three regions of LA volumes were quantified and these included the periventricular (5mm around the ventricles), infracortical (5mm beneath the gray matter), and deep (between periventricular and infracortical) regions. Results Cluster analysis sorted AD/VaD patients into single domain amnestic (n=41), single-domain dysexecutive (n=26), and multi-domain (n=26) phenotypes. The multi-domain patients exhibited worst performance on language tests; however, multi-domain patients were equally impaired on memory tests when compared to amnestic patients. Statistically-determined groups were relatively dissociated using neuroradiological parameters such that amnestic and multi-domain groups presented with smaller hippocampal volume while the dysexecutive group presented with greater deep, periventricular, and whole brain LA. Neither caudate nor lacunar infarction volume differed between cluster-determined groups. Caudate nucleus volume negatively correlated with total LA in the dysexecutive and multi-domain groups. Conclusions Results suggest that embedded within patients diagnosed clinically with AD/VaD spectrum dementia there are at least three distinct subtypes which can be operationally-defined. Further research is needed to assess the neuroradiological substrates underlying statistically-determined AD

  5. 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. PMID:26505869

  6. Studies in a newborn infant with supraventricular tachycardia and Wolff-Parkinson-White syndrome.

    PubMed Central

    Kaye, H H; Reid, D S; Tynan, M

    1975-01-01

    A neonatal patient with Wolff-Parkinson-White type A and supraventricular tachycardia is described. Demonstration of the mechanism of the tachycardia and its termination were achieved using intracardiac electrography and cardiac pacing. Quantitative left ventricular angiography and echocardiography showed impaired left ventricular function following the arrhythmia. Serial echocardiograms demonstrated the subsequent return to normal left ventricular performance. Images PMID:1138737

  7. [Vascular parkinsonism].

    PubMed

    Yamanouchi, H

    1997-01-01

    Critchley speculated that multiple vascular lesions of the basal ganglia must have an etiological connection to the symptoms of so-called vascular parkinsonism (VP), but without neuropathological confirmation. Some had doubts about its existence because of the lack of the pathologically confirmed case with adequate clinical correlation. At present, VP is characterized clinically by the short-stepped or frozen gait, lead-pipe rigidity, the symmetry of findings, absence of resting tremor, and negative response to levodopa in elderly patients with cerebrovascular lesions on CT/MRI. Pseudobulbar palsies, pyramidal tract findings, and/or multi-infarct dementia coexist in some of the cases. Most of clinically suspected VP patients have cerebral white matter lesions as well as basal ganglia lesions. PMID:9014431

  8. A young patient with atypical type-B Wolff-Parkinson-White syndrome accompanied by left ventricular dysfunction.

    PubMed

    Takeuchi, Takahiro; Tomita, Takeshi; Kasai, Hiroki; Kashiwagi, Daisuke; Yoshie, Koji; Yaguchi, Tomonori; Oguchi, Yasutaka; Kozuka, Ayako; Gautam, Milan; Motoki, Hirohiko; Okada, Ayako; Shiba, Yuji; Aizawa, Kazunori; Izawa, Atsushi; Miyashita, Yusuke; Koyama, Jun; Hongo, Minoru; Ikeda, Uichi

    2015-02-01

    A 15-year-old asymptomatic male patient presented with an electrocardiographic abnormality and left ventricular (LV) dysfunction (left ventricle ejection fraction of 40%) in a physical examination performed 2 years previously. LV dysfunction did not improve despite optimal medical therapy for dilated cardiomyopathy. Twelve-lead electrocardiography revealed a normal PR interval (138 ms) with a small delta-like wave in V2, but not a typical diagnostic wave that could be diagnosed as Wolff-Parkinson-White (WPW) syndrome by an electrocardiogram auto-analysis. Transthoracic echocardiography showed a remarkable asynchronous septal motion. An electrophysiological study was performed to exclude WPW syndrome. An accessory pathway (AP) was revealed on the lateral wall of the right ventricle, and radiofrequency catheter ablation was successfully performed to disconnect the AP. Thereafter, the dyssynchrony disappeared, and LV function improved. The intrinsic atrioventricular nodal conduction was very slow (A-H, 237 ms). The results of electrocardiogram auto-analysis could not be used to confirm the diagnosis of WPW syndrome because of the atypical delta wave. Conduction via the right lateral AP caused electrical dyssynchrony in the LV. This case suggests that atypical delta waves should be evaluated without depending on electrocardiographic auto-analyses in patients with LV dysfunction accompanied by dyssynchrony. PMID:26336525

  9. 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.

  10. Serotonin syndrome after the use of tramadol and ziprasidone in a patient with a deep brain stimulator for Parkinson disease.

    PubMed

    El-Okdi, Nasser Samir; Lumbrezer, Daniel; Karanovic, Djuro; Ghose, Abhimanyu; Assaly, Ragheb

    2014-01-01

    Serotonin syndrome (SS) is a life-threatening adverse reaction that can result from the therapeutic use of serotonergic drugs or accidental drug interactions. Tramadol is a drug that is widely prescribed because of its low abuse potential, but physicians need to be aware of its significant potential to cause SS because it inhibits serotonin reuptake. Ziprasidone is an atypical antipsychotic that can also cause dangerous interactions to cause SS because it is not only a potent 5-HT1A agonist but also has been reported to inhibit serotonin reuptake with an affinity similar to tricyclic antidepressants, in addition to inhibiting reuptake of norepinephrine. We are describing the clinical characteristics of a gentleman with bipolar disorder and Parkinson disease who presented with SS, despite having a deep brain stimulator in the subthalamic nucleus, which decreases central serotonin levels, and a discussion of the factors that contributed to his presentation. PMID:24158007

  11. A progressive syndrome of autism, dementia, ataxia, and loss of purposeful hand use in girls: Rett's syndrome: report of 35 cases.

    PubMed

    Hagberg, B; Aicardi, J; Dias, K; Ramos, O

    1983-10-01

    Thirty-five patients, exclusively girls, from three countries had a uniform and striking progressive encephalopathy. After normal general and psychomotor development up to the age of 7 to 18 months, developmental stagnation occurred, followed by rapid deterioration of higher brain functions. Within one-and-a-half years this deterioration led to severe dementia, autism, loss of purposeful use of the hands, jerky truncal ataxia, and acquired microcephaly. The destructive stage was followed by apparent stability lasting through decades. Additional insidious neurological abnormalities supervened, mainly spastic parapareses, vasomotor disturbances of the lower limbs, and epilepsy. Prior extensive laboratory investigations have not revealed the cause. The condition is similar to a virtually overlooked syndrome described by Rett in the German literature. The exclusive involvement of females, correlated with findings in family data analyses, suggests a dominant mutation on one X chromosome that results in affected girls and nonviable male hemizygous conceptuses. PMID:6638958

  12. 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

  13. Antipsychotic Drugs Tied to Risk of Early Death in Parkinson's Patients

    MedlinePlus

    ... Drugs Tied to Risk of Early Death in Parkinson's Patients But it's unclear whether the medications or ... 22, 2016 (HealthDay News) -- New research suggests that Parkinson's patients who are given antipsychotics to treat dementia ...

  14. DAT imaging in drug-induced and psychogenic parkinsonism.

    PubMed

    Tolosa, Eduardo; Coelho, Miguel; Gallardo, Marisol

    2003-10-01

    Parkinson's syndrome (PS) is frequently encountered in disorders associated with prominent degeneration of the nigrostriatal pathway as in Parkinson's disease, multisystem atrophy, and progressive supranuclear palsy (presynaptic PS). Drug-induced parkinsonism, a common, underdiagnosed health problem and psychogenic parkinsonism are causes of Parkinson's syndrome which, evidence suggests, occurs without degeneration of nigrostriatal structures. We review clinical features and results of DAT imaging in drug-induced parkinsonism and psychogenic parkinsonism. These two conditions normally give normal striatal DAT imaging results; an abnormal result in either case could exclude both conditions, corroborating a diagnosis of organic parkinsonism in uncertain cases. PMID:14531043

  15. [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. PMID:2378538

  16. Study of restriction fragment length polymorphism in the cystatin C gene of elderly patients with dementia and aged Down's syndrome patients.

    PubMed

    Palsdottir, A; Thorsteinsson, L; Jonsdottir, S; Arnason, A; Snaedal, J; Magnusson, T; Snorrason, E; Ingibergsdottir, R; Abrahamson, M; Olafsson, I

    1989-01-01

    Using a full length cystatin C cDNA probe and the Alu I restriction enzyme a total of 33 patients with senile dementia, Alzheimer type and 31 Down's syndrome patients have been investigated for the presence of the 630 bp Alu I restriction fragment length polymorphism in the cystatin C gene detected in Icelandic patients with hereditary cystatin C amyloid angiopathy. Results showed that all the patients had normal cystatin C fragment length of 600 bp. PMID:2574869

  17. 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.

  18. Taste in dementing diseases and parkinsonism.

    PubMed

    Lang, C J G; Leuschner, T; Ulrich, K; Stössel, C; Heckmann, J G; Hummel, T

    2006-10-25

    Like with many sensory abilities a reduction of taste and smell occurs during aging. Since there are hints to an additional reduction in dementing diseases, we assessed 52 patients, 26 women and 26 men, who were presented to a memory clinic, using the Sniffin' Sticks, Whole Mouth and Taste Strip Tests. While smoking, alcohol consumption, intake of drugs and sex exerted only minor impact, age and the severity of cognitive impairment were of major importance. There was a moderate but significant correlation between the severity of dementia, taste and smell, even if the age effect was partialled out. Notably, patients with Parkinson syndrome showed worse taste and smell abilities than those without. Here the differences were indeed marked enough to play a possible role in making the diagnosis. This exploratory study confirms a mild reduction of gustatory function in dementing diseases over and beyond that of normal aging which--in addition to a reduction of smell--seems to be especially marked in Parkinson syndromes. PMID:16769086

  19. [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. PMID:18364297

  20. Primary caregivers' awareness and perception of early-onset dementia conditions in adolescents and young and middle-aged adults with Down syndrome.

    PubMed

    Lin, Jin-Ding; Chen, Wen-Xiu; Hsu, Shang-Wei; Lin, Lan-Ping; Lin, Fu-Gong; Tang, Chi-Chieh; Wu, Jia-Ling; Chu, Cordia; Chou, Yu-Ching

    2014-09-01

    The present study aims to investigate the onset of dementia conditions using the Dementia Screening Questionnaire for Individuals with Intellectual Disabilities (DSQIID) scale and to identify the possible factors associated with DSQIID scores in people with Down syndrome (DS). The study population was recruited from the voluntary registry members of the Republic of China Foundation for Persons with Down syndrome; primary caregivers provided DSQIID information on 196 adolescents and adults with DS (aged 15-48 years) who were entered into the database and analyzed using SPSS 20.0 software. The results described the distribution of early-onset dementia conditions in 53 adolescents and adults with DS, and 2.6% of the subjects with DS had possible dementia (DSQIID score ≧ 20). Univariate analyses found that older age (p=0.001) and comorbid conditions (p=0.003) were significantly associated with DSQIID scores. Older subjects were more likely to have higher DSQIID scores than were younger age groups after ANOVA and Scheffe's tests. Lastly, a multiple linear regression analysis revealed that age (p<0.01), severe disability level (p<0.05) and comorbid condition (p<0.01) significantly explained 13% of the variation in DSQIID scores after adjusting for the factors of gender, education level and multiple disabilities in adolescents and adults with DS. The study highlights that future research should focus on the occurrence of dementia in people with DS and on identifying its influencing factors based on sound measurements, to initiate appropriate healthy aging policies for this group of people. PMID:24858786

  1. Relation of syncope in young patients with Wolff-Parkinson-White syndrome to rapid ventricular response during atrial fibrillation.

    PubMed

    Paul, T; Guccione, P; Garson, A

    1990-02-01

    Syncope in patients due to Wolff-Parkinson-White (WPW) syndrome may be related either to a rapid rate of supraventricular tachycardia or to rapid ventricular response over the accessory pathway during atrial fibrillation (AF). From 1982 to 1987, 74 patients less than or equal to 25 years old (mean age 12.6 years) with WPW syndrome on electrocardiogram underwent electrophysiologic study. Of the 74 patients, 14 (19%) had a history of syncope. During electrophysiologic study 9 of 14 patients with syncope had sustained (greater than 5 minutes or requiring termination due to hypotension) AF. Of the remaining 5 patients, 3 had inducible nonsustained AF and 2 had no AF. None of the 60 patients without syncope developed sustained AF; 34 had nonsustained and 26 had no AF. Occurrence of sustained AF had a sensitivity of 64% and specificity of 100% for history of syncope. All patients with syncope and AF (12) had a short RR interval between 2 consecutive preexcited QRS complexes during AF at less than or equal to 220 ms, in contrast to 9 of 34 patients without syncope (p less than 0.001, sensitivity 100%, specificity 74%). No patient with a short RR interval between 2 consecutive preexcited QRS complexes during AF of greater than 220 ms had a history of syncope. Thus, in these young patients with WPW syndrome, occurrence of AF with a rapid ventricular response during electrophysiologic study correlated well with a history of syncope and may be the cause of syncope in most patients. Electrophysiologic study may be helpful in identification of young patients with WPW at risk for syncope. PMID:2301260

  2. [Effect of radiofrequency ablation of accessory atrio-ventricular junctions on electromechanical coupling of the myocardium in children with manifesting Wolf-Parkinson-White syndrome].

    PubMed

    Martsinkevich, G I; Sokolov, A A; Murzina, O Iu; Kovalev, I A; Popov, S V

    2007-01-01

    Aim of the study was to assess immediate and remote hemodynamic and electromechanical effects of radiofrequency ablation of accessory atrio-ventricular pathways in children with manifesting Wolf-Parkinson-White (WPW) syndrome. Radiofrequency ablation of accessory atrio-ventricular pathways resulted in diminishment of intraventricular dyssynchrony, what appeared as lowering of the degree of heterogeneity of electromechanical intervals and was associated with significant augmentation of left ventricular stroke volume. Presence of pronounced intraventricular asynchrony might be an additional indication to radiofrequency ablation of accessory atrio-ventricular pathways in patients with WPW syndrome without history of documented attacks of tachycardia. PMID:18260859

  3. 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. PMID:25554999

  4. 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. PMID:26614001

  5. [Apathy and Dementia].

    PubMed

    Okada, Kazunori; Yamaguchi, Shuhei

    2016-07-01

    Apathy, which has been attracting attention since Marin's report in 1990, is ubiquitous among neuropsychiatric diseases. It has a major impact on the quality of life in both patients and their caregivers and impairs rehabilitation and outcome. Furthermore, apathy is important as a prodromal syndrome in the development of dementia in mild cognitive impairment (MCI). We reviewed the neurobiological basis, prevalence and assessment of potential benefits of non-pharmacologic and pharmacologic interventions for apathy in MCI and dementia. PMID:27395461

  6. Protagonists with Parkinson's disease.

    PubMed

    Haan, Joost

    2013-01-01

    Parkinson's disease is a complex disorder with many fascinating features. Its onset is creeping, the progression is slow but inevitable. There are motor symptoms, such as a tremor, rigidity, bradykinesia, mask-like facial expression, and postural abnormalities, but also hallucinations, cognitive deterioration, and depression. In many novels, fictive patients with Parkinson's disease play a role. It seems that authors have used many aspects of the disease to emphasize their messages. Their narratives include themes such as rigidity, petrifaction, confusion, dementia, and hallucinations. In this chapter, as examples, several protagonists with Parkinson's disease will be described from works of John Updike, Jonathan Franzen, Sue Miller, J.M. Coetzee, and John Harding, among others. PMID:23485900

  7. The value of novel MRI techniques in Parkinson-plus syndromes: diffusion tensor imaging and anatomical connectivity studies.

    PubMed

    Tir, M; Delmaire, C; Besson, P; Defebvre, L

    2014-04-01

    Conventional MRI is a well-described, highly useful tool for the differential diagnosis of degenerative parkinsonian syndromes. Nevertheless, the observed abnormalities may only appear in late-stage disease. Diffusion tensor imaging (DTI) can identify microstructural changes in brain tissue integrity and connectivity. The technique has proven value in the differential diagnosis of multiple system atrophy (MSA), progressive supranuclear palsy (PSP) and Parkinson's disease (PD). Here, we performed a systematic review of the literature on the main corticosubcortical DTI abnormalities identified to date in the context of the diagnosis of MSA and PSP with diffusion-weighted imaging, diffusion tensor imaging and anatomical connectivity studies. In good agreement with the histological data, increased diffusivity in the putamen (in MSA and PSP), in the middle cerebellar peduncles (in MSA) and in the upper cerebellar peduncles (in PSP) has been reported. Motor pathway involvement is characterized by low fraction anisotropy (FA) in the primary motor cortex in MSA-P and PSP, a high apparent diffusion coefficient (ADC) and low FA in the supplementary motor area in PSP. We then outline the value of these techniques in differential diagnosis (especially with respect to PD). Anatomical connectivity studies have revealed a lower number of fibers in the corticospinal tract in MSA and PSP (relative to PD and controls) and fewer tracked cortical projection fibers in patients with PSP or late-stage MSA (relative to patients with early MSA or PD and controls). Lastly, we report the main literature data concerning the value of DTI parameters in monitoring disease progression. The observed correlations between DTI parameters on one hand and clinical scores and/or disease duration on the other constitute strong evidence of the value of DTI in monitoring disease progression. In MSA, the ataxia score was correlated with ADC values in the pons and the upper cerebellar peduncles, whereas both

  8. Parkinson disease

    MedlinePlus

    American Parkinson Disease Association. Parkinson's Disease Handbook: A Guide for Patients and Their Families. Revised 2009. Available at: www.apdaparkinson.org/uploads/files/MP51919AmParkinsonHBK-vaU.pdf . Accessed September 15, ...

  9. Cortical correlates of affective syndrome in dementia due to Alzheimer's disease.

    PubMed

    Hayata, Thaís T; Bergo, Felipe P G; Rezende, Thiago J; Damasceno, Alfredo; Damasceno, Benito P; Cendes, Fernando; Stella, Florindo; Balthazar, Marcio L F

    2015-07-01

    Neuropsychiatric symptoms in Alzheimer's disease (AD) are prevalent, however their relationship with patterns of cortical atrophy is not fully known. Objectives To compare cortical atrophy's patterns between AD patients and healthy controls; to verify correlations between neuropsychiatric syndromes and cortical atrophy. Method 33 AD patients were examined by Neuropsychiatric Inventory (NPI). Patients and 29 controls underwent a 3T MRI scanning. We considered four NPI syndromes: affective, apathy, hyperactivity and psychosis. Correlations between structural imaging and neuropsychiatric scores were performed by Freesurfer. Results were significant with a p-value < 0.05, corrected for multiple comparisons. Results Patients exhibited atrophy in entorhinal cortices, left inferior and middle temporal gyri, and precuneus bilaterally. There was correlation between affective syndrome and cortical thickness in right frontal structures, insula and temporal pole. Conclusion Cortical thickness measures revealed atrophy in mild AD. Depression and anxiety symptoms were associated with atrophy of right frontal, temporal and insular cortices. PMID:26200048

  10. Behavioral dysfunction in Parkinson's disease.

    PubMed

    Friedman, J H

    1998-01-01

    Behavioral manifestations of Parkinson's disease (PD) are often more debilitating than the motor manifestations. These occur both as primary manifestations of the disease and as drug-induced complications. While dementia and abulia are common problems that are not currently treatable, depression and psychosis often respond extremely well to medication. Phenomenology, pathology, and general approaches to treatment will be discussed. PMID:10785833

  11. The initial recognition and diagnosis of dementia.

    PubMed

    Knopman, D S

    1998-04-27

    Dementia is characterized by a decline in cognition, behavioral disturbances, and interference with daily functioning and independence. Diagnosis is sometimes delayed as patients or family members often misattribute obvious manifestations of cognitive decline to normal aging rather than to the onset of a degenerative disease. Many physicians do not perform mental status examinations or do not use them effectively to detect early symptoms. Clinical markers are available to decrease the difficulty in distinguishing dementia from depression and confusional states such as delirium. Alzheimer's disease (AD) is the most common form of dementia; others include rapidly progressive dementias, dementias associated with strokes and Parkinson's disease, and frontotemporal dementias. Often, AD coexists with other forms of dementia. Sensitivity to early warning signs, interviews with family members, and mental status examinations are essential to early detection of AD, and will prove useful to primary-care physicians who care for older patients. PMID:9617846

  12. 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.

  13. 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…

  14. 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…

  15. Mutation in GM2A Leads to a Progressive Chorea-dementia Syndrome

    PubMed Central

    Salih, Mustafa A.; Seidahmed, Mohammed Z.; El Khashab, Heba Y.; Hamad, Muddathir H. A.; Bosley, Thomas M.; Burn, Sabrina; Myers, Angela; Landsverk, Megan L.; Crotwell, Patricia L.; Bilguvar, Kaya; Mane, Shrikant; Kruer, Michael C.

    2015-01-01

    Background The etiology of many cases of childhood-onset chorea remains undetermined, although advances in genomics are revealing both new disease-associated genes and variant phenotypes associated with known genes. Methods We report a Saudi family with a neurodegenerative course dominated by progressive chorea and dementia in whom we performed homozygosity mapping and whole exome sequencing. Results We identified a homozygous missense mutation in GM2A within a prominent block of homozygosity. This mutation is predicted to impair protein function. Discussion Although discovered more than two decades ago, to date, only five patients with this rare form of GM2 gangliosidosis have been reported. The phenotype of previously described GM2A patients has been typified by onset in infancy, profound hypotonia and impaired volitional movement, intractable seizures, hyperacusis, and a macular cherry red spot. Our findings expand the phenotypic spectrum of GM2A mutation-positive gangliosidosis to include generalized chorea without macular findings or hyperacusis and highlight how mutations in neurodegenerative disease genes may present in unexpected ways. PMID:26203402

  16. Should radiofrequency current ablation be performed in asymptomatic patients with the Wolff-Parkinson-White syndrome?

    PubMed

    Steinbeck, G

    1993-03-01

    The exiting new method of ablation of accessory pathways using radiofrequency current applied by catheters will dramatically change our therapeutic decisions in these patients in the near future. This brief survey reviews the existing literature about the risk of the disease as well as of the procedure of catheter ablation. From these data, the risk of sudden death appears to be extremely low in asymptomatic Wolff-Parkinson-White (WPW) individuals. Side effects of catheter ablation may result from the invasive procedure as well as from radiation exposure (the latter to the patient as well as to operating physicians). While the complication rate in experienced centers is extremely low, a multicenter registry of the success and complication rate is urgently needed in view of the many centers starting with catheter ablation. Based on a subjective benefit-to-risk analysis, asymptomatic WPW individuals should be offered catheter ablation only under special circumstances (high risk profession, athletes, family history of sudden death). On the other hand, catheter ablation need not be and should not be considered generally in asymptomatic individuals with WPW pattern. Finally, this author cannot imagine that the energy, time, and money spent for mass screening and eventual catheter ablation of asymptomatic WPW individuals with its attending risks can be outweighed by the potential benefits for these asymptomatic individuals. PMID:7681970

  17. 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

  18. Parkinson's disease (PD) in the elderly: an example of geriatric syndrome (GS)?

    PubMed

    Lauretani, Fulvio; Maggio, Marcello; Silvestrini, Claudio; Nardelli, Anna; Saccavini, Marsilio; Ceda, Gian Paolo

    2012-01-01

    PD is an age-related neurodegenerative disorder that affects as many as 1-2% of persons aged 60 years and older. In the latest decade, the approach to PD was dramatically changed. In fact, although for many years PD has been considered only "a disease that affects walking", with a key role of the neurotransmitter dopamine, recently the neurological approach has been substantially modified. The approach for this disease is not only a neurological issue. Given the complexity of its clinical aspects, such as depression, anxiety, dementia, sleep disorder, pneumonia dysfagia-related and malnutrition, a multidisciplinary evaluation and not just a neurological evaluation is needed. We suggest a n multidisciplinary approach for this old actor, underlying a subtle link between neurophatological stages of the disease (Braak's classification) and clinical aspects (Braak's stages 1 and 2 associated with the premotor phase; Braak's stages 3-4 associated with the motor symptoms and Braak's stages 5-6 associated with cognitive impairment). In addition, we emphasize the usefulness of geriatric evaluation for the identification of frail "in situ", frail, and disable status for improving care and treatment in this multifaceted disease. PMID:21459464

  19. C19orf12 mutation leads to Karak pallido-pyramidal syndrome

    PubMed Central

    Kruer, Michael C.; Salih, Mustafa A.; Mooney, Catherine; Alzahrani, Jawahir; Elmalik, Salah A.; Kabiraj, Mohammad M.; Khan, Arif O.; Paudel, Reema; Houlden, Henry; Azzedine, Hamid; Alkuraya, Fowzan

    2015-01-01

    Pallido-pyramidal syndromes combine dystonia with or without parkinsonism and spasticity as part of a mixed neurodegenerative disorder. Several causative genes have been shown to lead to pallido-pyramidal syndromes, including FBXO7, ATP13A2, PLA2G6, PRKN and SPG11. In particular, mutations in PLA2G6 have been identified in patients with Karak syndrome, a neurodegenerative disorder that features ataxia, dystonia-parkinsonism, dementia and spasticity with neuroradiologic evidence of cerebellar atrophy and/or brain iron deposition. Some patients with phenotypic Karak syndrome do not have demonstrable mutations in PLA2G6. Using homozygosity mapping and direct sequencing in a multiplex consanguineous Saudi Arabian family with Karak syndrome, we identified a homozygous p.G53R mutation in C19orf12. Our findings expand the phenotypic spectrum associated with C19orf12 mutations. PMID:24361204

  20. 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

  1. [Syncope in patients with Wolff-Parkinson-White syndrome: clinical data, electrophysiologic substrate and prognostic value].

    PubMed

    Auricchio, A; Klein, H; Trappe, H J; Becht, I; Wenzlaff, P; Iacono, A

    1989-10-01

    Syncope in Wolff-Parkinson-White (WPW) patients might be considered a premonitory event heralding the future development of sudden death. Therefore, we reviewed the clinical and electrophysiologic data of 91 WPW patients referred for invasive evaluation of known arrhythmias, in order to assess the incidence and clinical relevance of syncope. Thirty-four patients (37%, Group I) reported the occurrence of 1 or more syncopal episodes, while 57 patients (63%, Group II) had no syncope. These 2 groups did not differ significantly with regard to age, sex, incidence and characteristics of arrhythmias, clinical history and frequency of arrhythmic events, presence of associated cardiac diseases. Eleven patients in Group I and 9 in Group II were resuscitated from a cardiac arrest. The sensitivity (40%), the specificity (64%) as well as the positive predictive value (32%) and the negative predictive value (71%) of syncope vs a cardiac arrest were not significant. There were no statistical differences in the effective refractory period of the right atrium, atrioventricular node, accessory pathway and right ventricle between the 2 groups. Furthermore, no differences were noted in the cycle length of tachycardia (327 +/- 60 ms in Group I and 335 +/- 46 ms in Group II) and in the minimum RR interval during atrial fibrillation (248 +/- 49 ms and 244 +/- 43 ms, in Group I and II, respectively) as well as in the number of patients who had a minimum RR interval during atrial fibrillation less than or equal to 250 ms (15 patients--65%--in Group I and 21 patients--62%--in Group II).(ABSTRACT TRUNCATED AT 250 WORDS) PMID:2605574

  2. Great shakes: famous people with Parkinson disease.

    PubMed

    Jones, Jeffrey M

    2004-12-01

    James Parkinson is credited with the first complete clinical description of the syndrome attributed to his name, Parkinson disease. It is recognized as the first syndrome defined after neurology became a specialty. Descriptions of Parkinson features are rare in antiquity, and famous people with this disorder have not been found before the 1800s. During the 20th century, more and more famous people appear to be afflicted with Parkinson, and this article reviews some of those who have withstood the "test of Time magazine," and examines some of the reasons why the syndrome is a relatively recent disorder. PMID:15646755

  3. Genetic susceptibility variants in parkinsonism.

    PubMed

    Soto-Ortolaza, Alexandra I; Ross, Owen A

    2016-01-01

    Parkinsonism is an umbrella term for a group of disorders characterized by the clinical signs of tremor, bradykinesia, rigidity, and postural instability. On neuropathologic examination parkinsonism can display alternate protein pathologies (e.g. α-synucleinopathy or tauopathy) but the degeneration of nigral neurons is consistent. The main forms of parkinsonism are, Parkinson's disease (PD), Dementia with Lewy Bodies (DLB), Multiple System Atrophy (MSA), Progressive Supranuclear Palsy (PSP) and Corticobasal Degeneration (CBD). Genetic studies from candidate gene, to unbiased genome-wide approaches including association and next-generation sequencing have nominated a number of disease determinants. Within this review we will highlight the genetic loci that are associated with disease and discuss the implications and importance for a better understanding of the genes involved and thus the underlying pathophysiology of these disorders. PMID:26414118

  4. Coexistence of Wolff-Parkinson-white and Brugada syndrome: mere curiosity?

    PubMed

    Kaiser, Elisabeth; Sacilotto, Luciana; Darrieux, Francisco; Sosa, Eduardo

    2014-09-01

    The association between Brugada syndrome (BS) and ventricular preexcitation is a rare condition, with sporadic cases already reported. We report the case of a 29-year-old man, with palpitation unrelated to physical or emotional stress. The electrocardiogram of the first visit revealed a ventricular preexcitation pattern and an end-conduction delay, with negative T wave in V1 and intraventricular conduction disturbance in V2 (atypical for BS). The typical aspect of BS occurred after introduction of propafenone for the prevention of atrioventricular tachycardia. We discuss the recognition of this rare association, the proarrhythmic effects of some drugs, treatment options, and prognosis. PMID:24517421

  5. Semantic Verbal Fluency Pattern, Dementia Rating Scores and Adaptive Behavior Correlate With Plasma Aβ42 Concentrations in Down Syndrome Young Adults

    PubMed Central

    Hoyo, Laura Del; Xicota, Laura; Sánchez-Benavides, Gonzalo; Cuenca-Royo, Aida; de Sola, Susana; Langohr, Klaus; Fagundo, Ana B.; Farré, Magí; Dierssen, Mara; de la Torre, Rafael

    2015-01-01

    Down syndrome (DS) is an intellectual disability (ID) disorder in which language and specifically, verbal fluency are strongly impaired domains; nearly all adults show neuropathology of Alzheimer’s disease (AD), including amyloid deposition by their fifth decade of life. In the general population, verbal fluency deficits are considered a strong AD predictor being the semantic verbal fluency task (SVFT) a useful tool for enhancing early diagnostic. However, there is a lack of information about the association between the semantic verbal fluency pattern (SVFP) and the biological amyloidosis markers in DS. In the current study, we used the SVFT in young adults with DS to characterize their SVFP, assessing total generated words, clustering, and switching. We then explored its association with early indicators of dementia, adaptive behavior and amyloidosis biomarkers, using the Dementia Questionnaire for Persons with Intellectual Disability (DMR), the Adaptive Behavior Assessment System-Second Edition (ABAS-II), and plasma levels of Aβ peptides (Aβ40 and Aβ42), as a potent biomarker of AD. In DS, worse performance in SVFT and poorer communication skills were associated with higher plasma Aβ42 concentrations, a higher DMR score and impaired communication skills (ABAS–II). The total word production and switching ability in SVFT were good indicators of plasma Aβ42 concentration. In conclusion, we propose the SVFT as a good screening test for early detection of dementia and amyloidosis in young adults with DS. PMID:26635555

  6. Semantic Verbal Fluency Pattern, Dementia Rating Scores and Adaptive Behavior Correlate With Plasma Aβ42 Concentrations in Down Syndrome Young Adults.

    PubMed

    Hoyo, Laura Del; Xicota, Laura; Sánchez-Benavides, Gonzalo; Cuenca-Royo, Aida; de Sola, Susana; Langohr, Klaus; Fagundo, Ana B; Farré, Magí; Dierssen, Mara; de la Torre, Rafael

    2015-01-01

    Down syndrome (DS) is an intellectual disability (ID) disorder in which language and specifically, verbal fluency are strongly impaired domains; nearly all adults show neuropathology of Alzheimer's disease (AD), including amyloid deposition by their fifth decade of life. In the general population, verbal fluency deficits are considered a strong AD predictor being the semantic verbal fluency task (SVFT) a useful tool for enhancing early diagnostic. However, there is a lack of information about the association between the semantic verbal fluency pattern (SVFP) and the biological amyloidosis markers in DS. In the current study, we used the SVFT in young adults with DS to characterize their SVFP, assessing total generated words, clustering, and switching. We then explored its association with early indicators of dementia, adaptive behavior and amyloidosis biomarkers, using the Dementia Questionnaire for Persons with Intellectual Disability (DMR), the Adaptive Behavior Assessment System-Second Edition (ABAS-II), and plasma levels of Aβ peptides (Aβ40 and Aβ42), as a potent biomarker of AD. In DS, worse performance in SVFT and poorer communication skills were associated with higher plasma Aβ42 concentrations, a higher DMR score and impaired communication skills (ABAS-II). The total word production and switching ability in SVFT were good indicators of plasma Aβ42 concentration. In conclusion, we propose the SVFT as a good screening test for early detection of dementia and amyloidosis in young adults with DS. PMID:26635555

  7. [Usefulness of the thoracic circle in localizing the pre-excitation zone in Wolff-Parkinson-White syndrome].

    PubMed

    Iturralde, P; de Micheli, A; Romero, L; Hernández, D; Méndez, A; Gutiérrez, A; Colin, L; González Hermosillo, J A

    1989-01-01

    In this study thoracic circle lead electrocardiogram were recorded during sinus rhythm in 50 patients with Wolff Parkinson White Syndrome. We analyzed: delta wave polarity, QRS axis in the frontal plane, ventricular preexcitation, the pattern of precordial R wave transition and QRS morphology in the unipolar leads, also concordance between electrocardiographic patterns and the site of the accessory pathway determined during electrophysiological study. Electrocardiograms from patients with left lateral sites showed negative delta waves in leads LI or a VL, V7 to V9, positive delta waves in V3R to V9R, a normal QRS axis and early precordial R wave transition (20 of 23 patients). Left posterior sites manifested negative Delta waves in L3, a VF, V7 to V9, V7R to V9R and a prominent R wave in V1 (4 of 5 patients). Left posteroparaseptal sites had the same pattern plus negative delta waves in L2, a superior QRS axis, and RS or Rs morphology in V1 (3 of 3 patients). Right posteroparaseptal sites had negative delta waves in L2, L3, a VF, V3R to V9R, positive delta waves in V7 to V9, a superior QRS axis and an R greater than S in V1 (10 of 11 patients). Right free wall locations manifested negative delta waves in L3, a VR, V3R to V9R, positive delta waves in V7 to V9, a normal QRS axis and R wave transition in V3 to V5 with QS morphology since V3R to V9R (6 of 7 patients).(ABSTRACT TRUNCATED AT 250 WORDS) PMID:2624501

  8. Tomographic phase analysis to detect the site of accessory conduction pathway in Wolff-Parkinson-White syndrome

    SciTech Connect

    Nakajima, K.; Bunko, H.; Tada, A.; Tonami, N.; Taki, J.; Nanbu, I.; Hisada, K.; Misaki, T.; Iwa, T.

    1984-01-01

    Phase analysis has been applied to Wolff-Parkinson-White syndrome (WPW) to detect the site of accessory conduction pathway (ACP); however, there was a limitation to estimate the precise location of ACP by planar phase analysis. In this study, the authors applied phase analysis to gated blood pool tomography. Twelve patients with WPW who underwent epicardial mapping and surgical division of ACP were studied by both of gated emission computed tomography (GECT) and routine gated blood pool study (GBPS). The GBPS was performed with Tc-99m red blood cells in multiple projections; modified left anterior oblique, right anterior oblique and/or left lateral views. In GECT, short axial, horizontal and vertical long axial blood pool images were reconstructed. Phase analysis was performed using fundamental frequency of the Fourier transform in both GECT and GBPS images, and abnormal initial contractions on both the planar and tomographic phase analysis were compared with the location of surgically confirmed ACPs. In planar phase analysis, abnormal initial phase was identified in 7 out of 12 (58%) patients, while in tomographic phase analysis, the localization of ACP was predicted in 11 out of 12 (92%) patients. Tomographic phase analysis is superior to planar phase images in 8 out of 12 patients to estimate the location of ACP. Phase analysis by GECT can avoid overlap of blood pool in cardiac chambers and has advantage to identify the propagation of phase three-dimensionally. Tomographic phase analysis is a good adjunctive method for patients with WPW to estimate the site of ACP.

  9. Exercise testing and thallium-201 myocardial perfusion scintigraphy in the clinical evaluation of patients with Wolff Parkinson White syndrome

    SciTech Connect

    Poyatos, M.E.; Suarez, L.; Lerman, J.; Guibourg, H.; Camps, J.; Perosio, A.

    1986-10-01

    In 58 patients with Wolff Parkinson White syndrome (WPW), we performed exercise stress testing in order to investigate the incidence of normalization of the auriculo-ventricular conduction and the ST-segment changes. For a more accurate evaluation of the latter, exercise and redistribution radionuclide images with Thallium-201 were obtained in 18 cases. Forty-nine had type A and nine had type B of WPW. Forty-eight had permanent, four had alternant and six had no pre-excitation (PE) when they started the test. Mean maximal functional capacity, mean maximal heart rate and mean maximal double product were not different when compared to an age-matched control group. Of the 48 patients who began the test with PE, in 23 (48%) it disappeared while PE persisted in 25 (52%). In 16 cases the disappearance of the PE was sudden and in seven it was progressive. Pre-excitation persisted in 39.5% of patients with type A and in 88.8% with type B (p less than 0.01). ST-segment depression was observed in 76.6% of patients with PE and in 28.6% of cases without PE (p less than 0.01). ST-segment depression occurred in 44.8% of patients with type A and in 100% of cases with type B (p less than 0.05). Transient abnormal Thallium-201 scans were observed in 62.5% of patients without PE and in 20% with PE. No patients showed exertional arrhythmias. This study suggests the possibility of measuring the duration of the refractory period of the accessory pathway in those patients n which the PE disappears suddenly, at a given heart rate.

  10. Why Wait for Dementia?

    ERIC Educational Resources Information Center

    Watchman, Karen

    2003-01-01

    This article offers guidelines for the modification of the living environment of adults with Down syndrome before they develop dementia in order to allow them to remain in familiar surroundings for as long as possible. These include maintaining the person's individuality; aiding his/her communication; changing supports with the course of the…

  11. [Dementia with Lewy bodies].

    PubMed

    Orimo, Satoshi

    2016-03-01

    It is important to differentiate dementia with Lewy bodies (DLB) and other dementia, especially Alzheimer disease (AD), because the medical treatment, management, and the prognosis of these diseases are different. In regard to clinical features, DLB patients have relatively mild memory disturbance, fluctuating cognition, more severe disturbances of attention, executive function, visuospacial function, visual hallucination, depression, autonomic symptoms, REM sleep behavior disorder, and idiopathic parkinsonism compared to AD patients. In regard to imaging tools, DLB patients have milder atrophy of medial temporal lobe by brain MRI, reduced occipital activity by SPECT or PET, reduced MIBG uptake by MIBG cardiac scintigraphy, and low dopamine transporter activity in the basal ganglia by SPECT or PET. PMID:27025091

  12. Cognitive Rehabilitation of Dementia in Adults with Down Syndrome: A Review of Non-Pharmacological Interventions

    PubMed Central

    Fonseca, Luciana Mascarenhas; Navatta, Anna Carolina Rufino; Bottino, Cássio M.C.; Miotto, Eliane Correa

    2015-01-01

    Background There is a close genetic relationship between Alzheimer's disease (AD) and Down syndrome (DS), AD being the most severe mental disorder affecting ageing individuals with DS. The objective of the present study was to evaluate the efficacy of cognitive rehabilitation interventions in DS patients with AD by means of a critical literature review. Summary Because AD is progressive and irreversible, treatment is aimed at delaying and reducing the cognitive and functional decline in order to preserve or improve quality of life. The effects that pharmacological treatments and cognitive interventions have on elderly individuals with AD are well documented. Recent clinical trials have investigated the use of pharmacological treatment in DS patients with AD, generating preliminary results that have been unfavourable. Key Messages There is a clear lack of studies addressing the efficacy of cognitive rehabilitation interventions in DS patients with AD, and there is an urgent need for studies providing evidence to inform decisions regarding the appropriate choice of treatment strategies. PMID:26483832

  13. Other Dementias

    MedlinePlus

    ... National Institute on Aging) Vascular Dementia fact sheet (University of California, San Francisco, Memory and Aging Center) ... Library of Medicine) Lewy Body Dementias fact sheet (University of California, San Francisco, Memory and Aging Center) ...

  14. [Falls in patients with dementia].

    PubMed

    Yamaguchi, Kiyoshi

    2008-11-01

    People with cognitive impairment are at about 2 to 3 times higher risk of falling compared with cognitively intact elderly. Incidence of falls among patients with Alzheimer's disease (AD) is high, nevertheless the clinical feature common in patients with mild to moderate AD is the absence of motor impairment. Recent studies suggest that the divided attention markedly impairs the ability of patients with AD to regulate the gait. Falls are particularly common in Dementia with Lewy bodies (DLB) patients and may aid diagnosis, and the falls are associated with parkinsonism and other unclear factors. Treatment studies evaluating fall reduction strategies in dementia patients are a priority. PMID:18974447

  15. Atypical parkinsonism caused by Pro105Leu mutation of prion protein

    PubMed Central

    Mano, Kagari Koshi; Matsukawa, Takashi; Mitsui, Jun; Ishiura, Hiroyuki; Tokushige, Shin-ichi; Takahashi, Yuji; Sato, Naoko Saito; Nakamoto, Fumiko Kusunoki; Ichikawa, Yaeko; Nagashima, Yu; Terao, Yasuo; Shimizu, Jun; Hamada, Masashi; Uesaka, Yoshikazu; Oyama, Genko; Ogawa, Go; Yoshimura, Jun; Doi, Koichiro; Morishita, Shinichi; Tsuji, Shoji

    2016-01-01

    Objective: To delineate molecular and clinical characteristics of 3 families with PRNP P105L mutation, a variant of Gerstmann-Sträussler-Scheinker syndrome whose main motor symptoms were parkinsonism and/or involuntary movements. Methods: The causative mutation was first determined in the affected patients of family 1 using whole-exome sequencing, and then mutational analysis was extended to families 2 and 3. The clinical features of the patients of these 3 families were summarized. Haplotype analysis was performed using high-density single nucleotide polymorphism array. Results: The whole-exome sequencing revealed that the heterozygous mutation c.314C>T (p.P105L) in PRNP was the only known pathogenic mutation shared by the 3 patients of the family with autosomal dominant parkinsonism. We further identified the same mutation in patients of the other 2 families with autosomal dominant parkinsonism and/or involuntary movements. The clinical features of our patients with PRNP P105L mutation included various motor symptoms such as parkinsonism and involuntary movements in addition to progressive dementia. The clinical features in part overlapped with those of other forms of inherited prion diseases, such as fatal familial insomnia and Huntington disease-like type 1. The patients with PRNP P105L mutation shared a haplotype spanning 7.1 Mb around PRNP, raising the possibility that the mutations in the patients originated from a common founder. Conclusion: Most of the patients presented with parkinsonism in addition to progressive dementia. Although spastic paraparesis has been emphasized as the main clinical feature, the clinical spectrum of patients with PRNP P105L is broader than expected. PMID:27066585

  16. Depression associated with dementia.

    PubMed

    Gutzmann, H; Qazi, A

    2015-06-01

    Depression and cognitive disorders, including dementia and mild cognitive impairment, are common disorders in old age. Depression is frequent in dementia, causing distress, reducing the quality of life, exacerbating cognitive and functional impairment and increasing caregiver stress. Even mild levels of depression can significantly add to the functional impairment of dementia patients and the severity of psychopathological and neurological impairments increases with increasing severity of depression. Depressive symptoms may be both a risk factor for, as well as a prodrome of dementia. Major depressive syndrome of Alzheimer's disease may be among the most common mood disorders of older adults. Treating depression is therefore a key clinical priority to improve the quality of life both of people with dementia as well as their carergivers. Nonpharmacological approaches and watchful waiting should be attempted first in patients who present with mild to moderate depression and dementia. In cases of severe depression or depression not able to be managed through nonpharmacological means, antidepressant therapy should be considered. PMID:25962363

  17. Parkinson's Disease

    MedlinePlus

    ... results in reduction of a critical neurotransmitter called dopamine, a chemical responsible for transmitting messages to parts ... that coordinate muscle movement. Parkinson's patients have less dopamine. Studies have shown that the symptoms of Parkinson's ...

  18. Parkinson's Disease

    MedlinePlus

    ... about 5 to 10 percent of people with Parkinson's have "early-onset" disease which begins before the age of 50. Early-onset forms of Parkinson's are often inherited, though not always, and some ...

  19. Alien Limb Syndrome Responsive to Amantadine in a Patient with Corticobasal Syndrome

    PubMed Central

    Gondim, Francisco de Assis Aquino; Tavares Júnior, José Wagner Leonel; Morais, Arlindo A.; Sales, Paulo Marcelo Gondim; Wagner, Horta Goes

    2015-01-01

    Background Corticobasal syndrome (CBS) is a complex neurodegenerative disorder associated with parkinsonism and alien limb syndrome. Dressing and ideomotor apraxia were reportedly responsive to amantadine. Case Report A 79-year-old female was referred for evaluation of right hemiparesis. Neurological examination showed dementia, normal ocular movements, mild facial hypomimia, and bradykinesia with right hemiparesis. Nine years later, she developed alien limb syndrome and was diagnosed with CBS. After failure to respond to several medications, alien limb syndrome markedly improved with amantadine. Discussion To the best of our knowledge, this is the first report of a consistent response of severe, forced dystonic alien limb syndrome to amantadine in a patient with CBS. PMID:26217545

  20. Dementia and elder abuse.

    PubMed

    Hansberry, Maria R; Chen, Elaine; Gorbien, Martin J

    2005-05-01

    Dementia and elder abuse are relatively common and under-diagnosed geriatric syndromes. A unique relationship is observed when the two entities coexist. Special issues can confound the care of the dementia patient suspected of being abused. Impaired language or motor abilities to communicate abusive situations to a third party, lack of decisional capacity to address the abusive situation, disinhibited behavior that contributes to a cycle of violence, and coincident depression of the abused elder complicate the diagnosis and management of elder abuse. Education of the caregiver and attention to caregiver stress, including depression, may prevent onset and perpetuation of abuse. PMID:15804553

  1. Parkinsonian Syndromes

    PubMed Central

    Williams, David R.; Litvan, Irene

    2013-01-01

    Purpose of Review The different parkinsonian conditions can be challenging to separate clinically. This review highlights the important clinical features that guide the diagnosis of Parkinson disease (PD), progressive supranuclear palsy (PSP), multiple system atrophy (MSA), and corticobasal degeneration (CBD). Strategies for treatment and disease management are also discussed. Recent Findings Over the past decade there has been an increasing recognition of the broad clinical presentations of the neurodegenerative forms of parkinsonism. Nonmotor symptoms in these diseases, including psychiatric, cognitive, autonomic, and gastrointestinal dysfunction, appear to have a major impact on quality of life and disability. PSP and CBD are now considered pathologic diagnoses, with several different and varied clinical phenotypes, that overlap and share features with PDand frontotemporal dementia syndromes. PD is distinguished by its excellent response to dopaminergic medications that is maintained over many years, in contrast to the response seen in patients with MSA and PSP. New diagnostic criteria have been proposed for CBD. No new therapeutic interventions have emerged for PSP, MSA, or CBD. Infusional therapies and deep brain stimulation surgery are established therapies for advanced PD. Summary The “parkinsonian syndromes” encompass a number of nosologic entities that are grouped together on the basis of their shared clinical features but are separated on the basis of their different pathologies. Overall, the consideration of clinical signs, mode of disease onset, and nature of disease progression are all important to make a timely and definitive diagnosis. PMID:24092286

  2. The diagnosis of manganese-induced parkinsonism.

    PubMed

    Cersosimo, Maria G; Koller, William C

    2006-05-01

    Parkinsonism is a clinical syndrome consisting of tremor, bradykinesia, rigidity, gait, balance problems, in addition to various non-motor symptoms. There are many causes of parkinsonism such as neurodegenerative disease, drugs, vascular causes, structural lesions, infections, and toxicants. Parkinson's disease, or idiopathic parkinsonism, is the most common form of parkinsonism observed in the clinic. There is degeneration of the substantia nigra, pars compacta, which results in loss of striatal dopamine. Parkinson's disease is a slowly progressive condition in which there is a dramatic and sustained responsiveness to levodopa therapy. Manganese is an essential trace element that can be associated with neurotoxicity. Hypermanganism can occur in a variety of clinical settings. The clinical symptoms of manganese intoxication include non-specific complaints, neurobehavioral changes, parkinsonism, and dystonia. Although the globus pallidus is the main structure of damage, other basal ganglia areas can also be involved. MRI scans may show globus pallidus changes during (and for a short period after) exposure. Fluorodopa PET scans that assess the integrity of the substantia nigra dopaminergic system are abnormal in Parkinson's disease. However, these scans re-reported to be normal in a few cases studied with manganese-induced parkinsonism. The parkinsonism due to manganese may have some clinical features that occur less commonly in Parkinson's disease, such as kinetic tremor, dystonia, specific gait disturbances, and early mental, balance and speech changes. The clinical signs tend to be bilateral whereas Parkinson's disease begins on one side of the body. Patients with manganese-induced parkinsonism may be younger at the onset of the disease than with Parkinson's disease. Lastly, there appears to be a lack of response to levodopa therapy in manganese-induced parkinsonism. In summary it may be possible to differentiate manganese-induced parkinsonism from Parkinson

  3. Inter-Dependent Mechanisms Behind Cognitive Dysfunction, Vascular Biology and Alzheimer's Dementia in Down Syndrome: Multi-Faceted Roles of APP

    PubMed Central

    Nizetic, Dean; Chen, Christopher L.; Hong, Wanjin; Koo, Edward H.

    2015-01-01

    People with Down syndrome (DS) virtually all develop intellectual disability (ID) of varying degree of severity, and also have a high risk of early Alzheimer's disease (AD). ID prior to the onset of dementia, and its relationship to the onset of dementia in DS is a complex phenomenon influenced by many factors, and scarcely understood. Unraveling the causative factors and modulators of these processes remains a challenge, with potential to be informative for both ID and AD, for the development of early biomarkers and/or therapeutic approaches. We review the potential relative and inter-connected roles of the chromosome 21 gene for amyloid precursor protein (APP), in both pathological conditions. Rare non-DS people with duplication of APP (dupAPP) get familial early onset AD (FEOAD) with virtually 100% penetrance and prominent cerebrovascular pathology, but don't suffer from ID before dementia onset. All of these features appear to be radically different in DS. On the other hand, rare individuals with partial trisomy 21 (T21) (with APP, but not DS-critical region in trisomy) have been described having ID. Likewise, partial T21 DS (without APP trisomy) show a range of ID, but no AD pathology. We review the multi-faceted roles of APP that might affect cognitive functioning. Given the fact that both Aβ secretion and synaptic maturation/plasticity are dependent on neuronal activity, we explore how this conflicting inter-dependency might affect cognitive pathogenesis in a dynamic way in DS, throughout the lifespan of an individual. PMID:26648852

  4. The neurobiology of the spinal cord in experimental parkinsonism and Parkinson's disease.

    PubMed

    Ferrucci, Michela; Biagioni, Francesca; Vivacqua, Giorgio; Busceti, Carla Letizia; Bartalucci, Alessia; Soldani, Paola; D'Este, Loredana; Fumagalli, Lorenzo; Fornai, Francesco

    2013-12-01

    The neurobiology of non-motor symptoms in Parkinson's disease (PD) reveals a number of unexpected areas which once were not recognized a priori as part of the neuropathology underlying PD. These areas may belong either to central nervous system or periphery. Among central areas major efforts in the last decade led to recognize a number of brain nuclei as part of the disease spreading or disease onset in PD patients. Unexpectedly recent evidence deriving from pathological studies in PD patients and corroborated by experimental models of PD provided clear evidence that the spinal cord is often recruited in PD pathology. Such an involvement is intriguing since the major degenerative disease of the spinal cord (amyotrophic lateral sclerosis) features the involvement of dopaminergic neurons of the substantia nigra pars compacta, while some environmental (parkinsonism, ALS, and dementia of Guam) and genetic (Kufor-Rakeb syndrome) diseases are known to be characterized by mixed degeneration of pyramidal and extrapyramidal regions. Thus, the clear-cut between degeneration of dopaminergic neurons in the substantia nigra and the loss of pyramidal motor system appears now more as a continuum of   degeneration which converge in abnormal activity and cell pathology of motor neurons as a final common pathway. Among motor neurons, visceral efferent cells of the spinal cord are involved and provide a robust neurobiological findings which may justify a variety of non-motor autonomic symptoms which characterize PD. Neurodegeneration in the spinal cord extends to the dorsal horn of the grey matter posing an intriguing link between PD and sensory alterations. The present manuscript reviews the involvement of multiple regions of the spinal cord in PD and experimental parkinsonism in the attempt to provide both a neurobiological background to understand non motor symptoms and to provide the anatomical basis for disease spreading. PMID:24873929

  5. Effects of Training on Controllability Attributions of Behavioural Excesses and Deficits Shown by Adults with Down Syndrome and Dementia

    ERIC Educational Resources Information Center

    Kalsy, Sunny; Heath, Rebecca; Adams, Dawn; Oliver, Chris

    2007-01-01

    Background: Whereas there is a knowledge base on staff attributions of challenging behaviour, there has been little research on the effects of training, type of behaviour and biological context on staff attributions of controllability in the context of people with intellectual disabilities and dementia. Methods: A mixed design was used to…

  6. Cognitive impairment in Parkinson's disease.

    PubMed

    Cosgrove, Jeremy; Alty, Jane Elizabeth; Jamieson, Stuart

    2015-04-01

    Cognitive impairment is a significant non-motor symptom of Parkinson's disease (PD). Longitudinal cohort studies have demonstrated that approximately 50% of those with PD develop dementia after 10 years, increasing to over 80% after 20 years. Deficits in cognition can be identified at the time of PD diagnosis in some patients and this mild cognitive impairment (PD-MCI) has been studied extensively over the last decade. Although PD-MCI is a risk factor for developing Parkinson's disease dementia there is evidence to suggest that PD-MCI might consist of distinct subtypes with different pathophysiologies and prognoses. The major pathological correlate of Parkinson's disease dementia is Lewy body deposition in the limbic system and neocortex although Alzheimer's related pathology is also an important contributor. Pathological damage causes alteration to neurotransmitter systems within the brain, producing behavioural change. Management of cognitive impairment in PD requires a multidisciplinary approach and accurate communication with patients and relatives is essential. PMID:25814509

  7. A prospective study of the cumulative incidence and course of restless legs syndrome in de novo patients with Parkinson's disease during chronic dopaminergic therapy.

    PubMed

    Marchesi, Elena; Negrotti, Anna; Angelini, Monica; Goldoni, Matteo; Abrignani, Giorgia; Calzetti, Stefano

    2016-03-01

    The authors report the cumulative incidence of Restless Legs Syndrome (RLS) over a 3 years follow-up period in 92 de novo Parkinson's disease patients under chronic dopaminergic therapy and the clinical course of the sensory-motor disorder over 12 months as from its onset. The overall cumulative incidence of RLS was found by 15.3%, i.e. 14 incident cases, and by 11.9%, i.e. 11 incident cases, after the exclusion of possible "secondary" forms of the disorder. These figures are higher than those reported in general population in Germany (Study of Health in Pomerania), confirming our previous findings of incidence rate of the disorder. At the end of the 3 years follow-up period the prevalence of "current" RLS was significantly higher than that previously found in drug naïve Parkinson's disease patients and in controls, supporting the view that RLS emerging in the course of chronic dopaminergic therapy is the main determinant of the co-morbid association with Parkinson's disease. During the 12 months period of observation the RLS showed a frequency of occurrence of 6.08 episodes per month on average and a remittent clinical course was prevailing in the 11 incident cases, with a significant frequency decrease in the second as compared to the first 6 months, i.e. 3.26 versus 8.9 episodes per month, and none of the patients developed augmentation in the same period. It is hypothesized that the remittent course could be due to long-term adaptation (downregulation) of the hypersensitive post-synaptic dopamine receptors in the spinal cord to a continuous dopaminergic stimulation, possibly coupled with compensatory up-regulation of pre-synaptic dopamine re-uptake mechanism, in the patients in which the hypothalamic A11 area, site of origin of the dopamine-mediated diencephalo-spinal pathway, is involved in the neurodegenerative process. PMID:26566909

  8. The association between behavioural and emotional problems and age in adults with Down syndrome without dementia: Examining a wide spectrum of behavioural and emotional problems.

    PubMed

    Makary, Anna T; Testa, Renee; Einfeld, Stewart L; Tonge, Bruce J; Mohr, Caroline; Gray, Kylie M

    2014-08-01

    The literature on the association between behavioural and emotional problems and ageing in adults with Down syndrome (DS) without dementia is limited and has generally not reported on a wide range of behavioural and emotional problems. This research aimed to extend the field by examining the associations between age and a wide spectrum of behavioural and emotional problems in adults with DS without dementia. A preliminary analysis of the association between potential covariates and behavioural and emotional problems was also undertaken. Parents and caregivers completed a questionnaire on behavioural and emotional problems for 53 adults with DS aged between 16 and 56 years. Twenty-eight adults with DS and their caregivers were part of a longitudinal sample, which provided two time points of data approximately four years apart. Additionally, 25 participants with DS and their caregivers were from a cross sectional sample, which provided one time point of data. Random effects regression analyses were used to examine the patterns in item scores for behavioural and emotional problems associated with age. No significant associations between age and the range or severity of any behavioural and emotional items were found. This suggested a more positive pattern for ageing adults with DS than has been previously described. Given that behavioural and emotional problems were not associated with age, investigation into other factors that may be associated with the behavioural and emotional difficulties for adults with DS is discussed. PMID:24794290

  9. Brain imaging in dementia.

    PubMed

    Bonifacio, Guendalina; Zamboni, Giovanna

    2016-06-01

    The introduction of MRI and positron emission tomography (PET) brain imaging has contributed significantly to the understanding of different dementia syndromes. Over the past 20 years these imaging techniques have been increasingly used for clinical characterisation and differential diagnosis, and to provide insight into the effects on functional capacity of the brain, patterns of spatial distribution of different dementia syndromes and their natural history and evolution over time. Brain imaging is also increasingly used in clinical trials, as part of inclusion criteria and/or as a surrogate outcome measure. Here we review all the relatively specific findings that can be identified with different MRI and PET techniques in each of the most frequent dementing disorders. PMID:26933232

  10. Distinguishing Parkinson's disease from atypical parkinsonian syndromes using PET data and a computer system based on support vector machines and Bayesian networks

    PubMed Central

    Segovia, Fermín; Illán, Ignacio A.; Górriz, Juan M.; Ramírez, Javier; Rominger, Axel; Levin, Johannes

    2015-01-01

    Differentiating between Parkinson's disease (PD) and atypical parkinsonian syndromes (APS) is still a challenge, specially at early stages when the patients show similar symptoms. During last years, several computer systems have been proposed in order to improve the diagnosis of PD, but their accuracy is still limited. In this work we demonstrate a full automatic computer system to assist the diagnosis of PD using 18F-DMFP PET data. First, a few regions of interest are selected by means of a two-sample t-test. The accuracy of the selected regions to separate PD from APS patients is then computed using a support vector machine classifier. The accuracy values are finally used to train a Bayesian network that can be used to predict the class of new unseen data. This methodology was evaluated using a database with 87 neuroimages, achieving accuracy rates over 78%. A fair comparison with other similar approaches is also provided. PMID:26594165

  11. Distinguishing Parkinson's disease from atypical parkinsonian syndromes using PET data and a computer system based on support vector machines and Bayesian networks.

    PubMed

    Segovia, Fermín; Illán, Ignacio A; Górriz, Juan M; Ramírez, Javier; Rominger, Axel; Levin, Johannes

    2015-01-01

    Differentiating between Parkinson's disease (PD) and atypical parkinsonian syndromes (APS) is still a challenge, specially at early stages when the patients show similar symptoms. During last years, several computer systems have been proposed in order to improve the diagnosis of PD, but their accuracy is still limited. In this work we demonstrate a full automatic computer system to assist the diagnosis of PD using (18)F-DMFP PET data. First, a few regions of interest are selected by means of a two-sample t-test. The accuracy of the selected regions to separate PD from APS patients is then computed using a support vector machine classifier. The accuracy values are finally used to train a Bayesian network that can be used to predict the class of new unseen data. This methodology was evaluated using a database with 87 neuroimages, achieving accuracy rates over 78%. A fair comparison with other similar approaches is also provided. PMID:26594165

  12. Frontotemporal dementia or frontotemporal lobar degeneration--overview of a group of proteinopathies.

    PubMed

    Haberland, Catherine

    2010-03-30

    Frontotemporal dementia is the second most common early onset dementia after Alzheimer disease. Frontotemporal dementias are a complex group of dementias. The clinical diagnosis can be perplexing because of concurring psychiatric and neurologic syndromes. Frontotemporal lobar degeneration, the underlying pathology, represents an emerging group of proteinopathies. Genetic factors play an important part in the etiologies of dementias. This article overviews current defining characteristics of frontotemporal dementias known also as frontotemporal lobar degenerations. PMID:20405665

  13. Psychiatric aspects of Parkinson's disease

    PubMed Central

    Grover, Sandeep; Somaiya, Mansi; Kumar, Santhosh; Avasthi, Ajit

    2015-01-01

    Parkinson's disease (PD) is essentially characterized by the motor symptoms in the form of resting tremor, rigidity and bradykinesia. However, over the years it has been recognized that motor symptoms are just the “tip of the iceberg” of clinical manifestations of PD. Besides motor symptoms, PD characterized by many non-motor symptoms, which include cognitive decline, psychiatric disturbances (depression, psychosis and impulse control), sleep difficulties, autonomic failures (gastrointestinal, cardiovascular, urinary, thermoregulation) and pain syndrome. This review evaluates the various aspects of psychiatric disorders including cognitive decline and sleep disturbances in patients with PD. The prevalence rate of various psychiatric disorders is high in patients with PD. In terms of risk factors, various demographic, clinical and treatment-related variables have been shown to be associated with higher risk of development of psychiatric morbidity. Evidence also suggests that the presence of psychiatric morbidity is associated with poorer outcome. Randomized controlled trials, evaluating the various pharmacological and non-pharmacological treatments for management of psychiatric morbidity in patients with PD are meager. Available evidence suggests that tricyclic antidepressants like desipramine and nortriptyline are efficacious for management of depression. Among the antipsychotics, clozapine is considered to be the best choice for management of psychosis in patients with PD. Among the various cognitive enhancers, evidence suggest efficacy of rivastigmine in management of dementia in patients with PD. To conclude, this review suggests that psychiatric morbidity is highly prevalent in patients with PD. Hence, a multidisciplinary approach must be followed to improve the overall outcome of PD. Further studies are required to evaluate the efficacy of various other measures for management of psychiatric morbidity in patients with PD. PMID:25552854

  14. Psychiatric aspects of Parkinson's disease.

    PubMed

    Grover, Sandeep; Somaiya, Mansi; Kumar, Santhosh; Avasthi, Ajit

    2015-01-01

    Parkinson's disease (PD) is essentially characterized by the motor symptoms in the form of resting tremor, rigidity and bradykinesia. However, over the years it has been recognized that motor symptoms are just the "tip of the iceberg" of clinical manifestations of PD. Besides motor symptoms, PD characterized by many non-motor symptoms, which include cognitive decline, psychiatric disturbances (depression, psychosis and impulse control), sleep difficulties, autonomic failures (gastrointestinal, cardiovascular, urinary, thermoregulation) and pain syndrome. This review evaluates the various aspects of psychiatric disorders including cognitive decline and sleep disturbances in patients with PD. The prevalence rate of various psychiatric disorders is high in patients with PD. In terms of risk factors, various demographic, clinical and treatment-related variables have been shown to be associated with higher risk of development of psychiatric morbidity. Evidence also suggests that the presence of psychiatric morbidity is associated with poorer outcome. Randomized controlled trials, evaluating the various pharmacological and non-pharmacological treatments for management of psychiatric morbidity in patients with PD are meager. Available evidence suggests that tricyclic antidepressants like desipramine and nortriptyline are efficacious for management of depression. Among the antipsychotics, clozapine is considered to be the best choice for management of psychosis in patients with PD. Among the various cognitive enhancers, evidence suggest efficacy of rivastigmine in management of dementia in patients with PD. To conclude, this review suggests that psychiatric morbidity is highly prevalent in patients with PD. Hence, a multidisciplinary approach must be followed to improve the overall outcome of PD. Further studies are required to evaluate the efficacy of various other measures for management of psychiatric morbidity in patients with PD. PMID:25552854

  15. Language Impairment in Alzheimer's Disease and Vascular Dementia.

    ERIC Educational Resources Information Center

    Lempinen, Maire; And Others

    A study of 21 patients with Alzheimer's Disease and 25 with vascular dementia, the two most common forms of dementia, investigated language impairments in the dementia syndrome to see if analysis of language disturbances is helpful in differential diagnosis. Diagnostic assessment included a neurological examination, detailed medical history,…

  16. The development of the dementia concept in 19th century.

    PubMed

    Caixeta, Leonardo; Costa, Jean Newton Lima; Vilela, Ana Caroline Marques; Nóbrega, Magno da

    2014-07-01

    The dementia concept has been reformulated through its history and the 19th century was remarkable in the construction of this concept as we understand it today. Like other syndromes, much of the history of the dementia concept comes from the attempt to separate it from other nosological conditions, giving it a unique identity. The fundamental elements for the arising of the dementia modern concept were: a) correlation of the observed syndrome with organic-cerebral lesions; b) understanding of the irreversibility of the dementia evolution; c) its relation with human ageing; and d) the choice of the cognitive dysfunction as a clinical marker of the dementia concept. PMID:25054992

  17. A Comparison of the Behavioural and Emotional Characteristics of Alzheimer's Dementia in Individuals with and without Down Syndrome

    ERIC Educational Resources Information Center

    Temple, Valerie; Konstantareas, M. Mary

    2005-01-01

    The behavioural and emotional changes associated with Alzheimer's disease (AD) are compared for individuals with Down syndrome and AD and individuals with AD from the general population (AD-only). The primary caregivers of 30 people with Down syndrome and AD and 30 people with AD-only completed the BEHAVE-AD and the Apathy subscale of the CERAD.…

  18. Biomarkers of aggression in dementia.

    PubMed

    Gotovac, Kristina; Nikolac Perković, Matea; Pivac, Nela; Borovečki, Fran

    2016-08-01

    Dementia is a clinical syndrome defined by progressive global impairment of acquired cognitive abilities. It can be caused by a number of underlying conditions. The most common types of dementia are Alzheimer's disease (AD), frontotemporal dementia (FTD), vascular cognitive impairment (VCI) and dementia with Lewy bodies (DLB). Despite the fact that cognitive impairment is central to the dementia, noncognitive symptoms, most commonly described nowadays as neuropsychiatric symptoms (NPS) exist almost always at certain point of the illness. Aggression as one of the NPS represents danger both for patients and caregivers and the rate of aggression correlates with the loss of independence, cognitive decline and poor outcome. Therefore, biomarkers of aggression in dementia patients would be of a great importance. Studies have shown that different genetic factors, including monoamine signaling and processing, can be associated with various NPS including aggression. There have been significant and multiple neurotransmitter changes identified in the brains of patients with dementia and some of these changes have been involved in the etiology of NPS. Aggression specific changes have also been observed in neuropathological studies. The current consensus is that the best approach for development of such biomarkers may be incorporation of genetics (polymorphisms), neurobiology (neurotransmitters and neuropathology) and neuroimaging techniques. PMID:26952705

  19. Parkinsonism following dystonia in three patients.

    PubMed

    Katchen, M; Duvoisin, R C

    1986-01-01

    Three patients who presented initially with dystonia and subsequently developed typical idiopathic parkinsonism were evaluated. One patient presented with a writer's cramp, one with axial dystonia, and one with Meige syndrome. All three displayed amelioration of their dystonia with progression of their parkinsonism over a period of 2 to 15 years. Treatment with levodopa gave some relief of the parkinsonism symptoms in two patients but exacerbated or reactivated the dystonia. It is suggested that both the dystonia and the parkinsonism represent the changing clinical expression of the same disorder at different times in its evolution. PMID:3504239

  20. Familial parkinsonism with depression: a clinicopathological study.

    PubMed

    Bhatia, K P; Daniel, S E; Marsden, C D

    1993-12-01

    A family with autosomal dominant inheritance of an early-onset and rapidly progressive parkinsonian syndrome and associated severe depression is reported. Three members had parkinsonism with depression, 3 had parkinsonism alone, and 2 suffered depression only. Pathological brain examination in 2 members with parkinsonism and depression revealed distinctive changes, with devastation of the substantia nigra, scarce Lewy bodies, and gliosis of the caudate nucleus and globus pallidus. The clinical and pathological findings were similar to those in four previously described families with autosomal dominant parkinsonism, depression, and alveolar hypoventilation. PMID:8250534

  1. Coronary artery disease in patients with dementia.

    PubMed

    Fowkes, Ross; Byrne, Matthew; Sinclair, Hannah; Tang, Eugene; Kunadian, Vijay

    2016-09-01

    Our population is ageing. The prevalence of dementia is increasing as the population ages. Dementia is known to share many common risk factors with coronary artery disease including age, genetics, smoking, the components of the metabolic syndrome and inflammation. Despite the growing ageing population with dementia, there is underutilization of optimal care (pharmacotherapy and interventional procedures) in this cohort. Given common risk factors and potential benefit, patients with cognitive impairment and dementia should be offered contemporary care. However, further research evaluating optimal care in this patient cohort is warranted. PMID:27159265

  2. Age-related changes in the clinical and electrophysiologic characteristics of patients with Wolff-Parkinson-White syndrome: comparative study between young and elderly patients.

    PubMed

    Fan, W; Peter, C T; Gang, E S; Mandel, W

    1991-09-01

    The natural history of patients with Wolff-Parkinson-White (WPW) syndrome remains an intriguing question with respect to clinical decision-making, since serial electrophysiologic data spread over several decades in the same patient are not available in the literature. To study the age-related changes in WPW syndrome, we compared two separate groups of patients referred to this Medical Center for electrophysiologic studies because of a clinical presentation with significant arrhythmias. An elderly group of 42 patients aged 50 years or more were compared with a younger group of 51 patients aged 15 to 30 years. The groups were comparable in terms of clinical presentation, including the number of patients who had reported syncopal episodes and those requiring cardioversion of their tachyarrhythmias. Baseline electrophysiologic variables such as sinus rate; sinoatrial conduction time; corrected sinus node recovery time; AH interval; and effective refractory periods of the right atrium, atrioventricular (AV) node, and right ventricular muscle, were significantly greater in the elderly group. Similarly, the anterograde effective refractory period of the bypass tract, the shortest atrial pacing cycle length with 1:1 anterograde conduction via the bypass tract, retrograde effective refractory period of the bypass tract, the shortest ventricular pacing cycle length with 1:1 retrograde conduction via the bypass tract, the shortest consecutive preexcited R-R interval during atrial fibrillation, and the cycle length of orthodromic atrial ventricular reciprocating tachycardia were significantly greater in the elderly group.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:1877451

  3. [Initial symptomatology of Parkinson disease].

    PubMed

    Fuhr, P; Maritz, D; Bernatik, J; Steck, A J

    1995-01-01

    The early diagnosis of Parkinson's disease (idiopathic parkinsonism) is important for two reasons. Some never drugs possibly have a neuroprotective effect, which can be utilized maximally only with early onset of treatment. Moreover, diagnostic mistakes may occur early in the course of the disease. Although the hallmark of Parkinson's disease is a syndrome of movement disorders, slight cognitive deficits, depression, or pain with or without paresthesias can be present at an early stage. Therefore, symptoms at the time of the first diagnosis and at the beginning of the first clinical manifestation of Parkinson's disease are often not identical. The diagnosis can be made only clinically, since no biological marker is available up to now. However, in unclear cases pharmacological tests constitute a valuable extension of the clinical examination. PMID:8533059

  4. Treatment of Parkinson's disease: focus on quality of life issues.

    PubMed

    Uitti, Ryan J

    2012-01-01

    The vast majority of Parkinson's disease (PD) treatment discussions focus on two areas: pharmacotherapy and surgery. Why? These treatments produce relatively easily measurable outcomes concerning traditional diagnostic criteria: motor rating scores and "on" times. However, despite the expanding number of drugs and surgery, there is dwindling incremental improvement from the best motor outcomes available even 40 years ago. Over the same time period, recognition of the clinical scope of this syndrome has expanded tremendously. Much of this scope impacts on quality of life and is treatable. Two arenas with great potential impact on quality of life are mental health and activity/exercise. Early in the course of PD, depression and expectations about impending demise impact significantly on the quality of life of patients. Initial responses to medical therapy may also dictate patients' expectations. Later, the two most devastating features may be motor instability/falls/injury and dementia. Mounting evidence for the benefits of treatment aimed at these arenas is accumulating and revolves around recognition and facilitating healthy routines that may mitigate future negative clinical phenomenon. Keys concerning mental health include education with realistic expectations and identification/treatment of mood and sleep disorders. Effective pro-active treatment regimens for instability and dementia include activity/exercise advocacy. PMID:22166448

  5. Mixed Dementia

    MedlinePlus

    ... bodies , What Is Alzheimer's? NIA-Funded Memory & Aging Project Reveals Mixed Dementia Common Data from the first ... disease. For example, in the Memory and Aging Project study involving long-term cognitive assessments followed by ...

  6. Vascular dementia

    MedlinePlus

    ... Alzheimer's Disease, and Dementia . 2nd ed. Philadelphia, PA: Elsevier; 2016:chap 6. Gorelick PB, Scuteri A, Black ... eds. Goldman's Cecil Medicine . 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 402. Peterson R, Graff-Radford ...

  7. Unraveling Parkinson's: Three Clues

    MedlinePlus

    ... Bar Home Current Issue Past Issues Unraveling Parkinson's: Three Clues Past Issues / Summer 2006 Table of Contents ... Dream Robber: Living with Parkinson's disease / Unraveling Parkinson's: Three Clues / Parkinson's Disease: The Newest Advances Summer 2006 ...

  8. Assessment of Atrial Fibrillation and Vulnerability in Patients with Wolff-Parkinson-White Syndrome Using Two-Dimensional Speckle Tracking Echocardiography

    PubMed Central

    Li, Jing-Jie; Wei, Fang; Chen, Ju-Gang; Yu, Yan-Wei; Gu, Hong-Yue; Jiang, Rui; Wu, Xiu-Li; Sun, Qian

    2014-01-01

    Purpose The aim was to assess atrial fibrillation (AF) and vulnerability in Wolff-Parkinson-White (WPW) syndrome patients using two-dimensional speckle tracking echocardiography (2D-STE). Methods All patients were examined via transthoracic echocardiography and 2D-STE in order to assess atrial function 7 days before and 10 days after RF catheter ablation. A postoperative 3-month follow-up was performed via outpatient visit or telephone calls. Results Results showed significant differences in both body mass index (BMI) and supraventricular tachycardia (SVT) duration between WPW patients and DAVNP patients (both P<0.05). Echocardiography revealed that the maximum left atrial volume (LAVmax) and the left ventricular mass index (LVMI) in diastole increased noticeably in patients with WPW compared to patients with DAVNP both before and after ablation (all P<0.05). Before ablation, there were obvious differences in the levels of SRs, SRe, and SRa from the 4-chamber view (LA) in the WPW patients group compared with patients in the DAVNP group (all P<0.05). In the AF group, there were significant differences in the levels of systolic strain rate (SRs), early diastolic strain rate (SRe), and late diastolic strain rate (SRa) from the 4-chamber view (LA) both before and after ablation (all P<0.05). In the non-AF group, there were decreased SRe levels from the 4-chamber view (LA/RA) pre-ablation compared to post-ablation (all P<0.05). Conclusion Our findings provide convincing evidence that WPW syndrome may result in increased atrial vulnerability and contribute to the development of AF. Further, RF catheter ablation of AAV pathway can potentially improve atrial function in WPW syndrome patients. Two-dimensional speckle tracking echocardiography imaging in WPW patients would be necessary in the evaluation and improvement of the overall function of RF catheter ablation in a long-term follow-up period. PMID:25397668

  9. Usefulness of severe cardiac sympathetic dysfunction to predict the occurrence of rapid atrial fibrillation in patients with Wolff-Parkinson-White syndrome.

    PubMed

    Akutsu, Yasushi; Kaneko, Kyouichi; Kodama, Yusuke; Li, Hui-Ling; Asano, Taku; Suyama, Jumpei; Tanno, Kaoru; Namiki, Atsuo; Shinozuka, Akira; Gokan, Takehiko; Kobayashi, Youichi

    2013-09-01

    Atrial fibrillation (AF) can be a potentially life-threatening arrhythmia when it conducts rapidly through the accessory pathway, which was not predicted by the noninvasive method. We evaluated the cardiac sympathetic activity for predicting the occurrence of AF in patients with Wolff-Parkinson-White (WPW) syndrome. Iodine-123 metaiodobenzylguanidine scintigraphy was performed under stable sinus rhythm conditions at rest <1 week before an electrophysiologic study (EPS) to assess the sympathetic activity using the heart/mediastinum (H/M) ratio in 45 consecutive patients with WPW who had a history of supraventricular tachycardia (mean ± SD, age: 47 ± 17 years, 42.2% women). The study also included 15 normal healthy volunteers (56 ± 17 years, 40% women). The H/M ratio was lower in patients with WPW syndrome than in the normal control group, and in the 15 patients with AF induced during EPS than in the 30 patients without AF (p <0.0001). The sensitivity of H/M ratio ≤2.8 for predicting the AF induced during EPS was 75% in 12 of 16 patients, and the specificity was 89.7% in 26 of 29 patients. The H/M ratio was positively correlated with anterograde effective refractory period (r = 0.514, p <0.0001). The sensitivity of H/M ratio ≤2.75 for predicting the AF with a short anterograde effective refractory period (≤250 ms) was 91.7% in 11 of 12 patients, and the specificity was 90.9% in 30 of 33 patients. In conclusion, the severe cardiac sympathetic dysfunction was associated with the occurrence of AF, particularly in those with rapid AF and in patients with WPW syndrome. PMID:23711805

  10. Evaluation of Screening Tools for Dementia in Older Adults with Mental Retardation

    ERIC Educational Resources Information Center

    Shultz, Jennifer; Aman, Michael; Kelbley, Thomas; Wallace, Cheryl LeClear; Burt, Diana B.; Primeaux-Hart, Sharon; Loveland, Katherine; Thorpe, Lilian; Bogos, Eleanor S.; Timon, John; Patti, Paul; Tsiouris, John

    2004-01-01

    We compared groups with and without diagnosed dementia matched on IQ, age, and presence of Down syndrome. The Dementia Scale for Down Syndrome and Dementia Questionnaire for Mentally Retarded Persons were used to assess participants. We developed two performance tasks to determine whether they were useful in separating subjects with and without…

  11. Drug development in dementia.

    PubMed

    Cunningham, Emma L; Passmore, Anthony P

    2013-11-01

    Dementia is a progressive, irreversible decline in cognition that, by definition, impacts on a patient's pre-existing level of functioning. The clinical syndrome of dementia has several aetiologies of which Alzheimer's disease (AD) is the most common. Drug development in AD is based on evolving pathophysiological theory. Disease modifying approaches include the targeting of amyloid processing, aggregation of tau, insulin signalling, neuroinflammation and neurotransmitter dysfunction, with efforts thus far yielding abandoned hopes and ongoing promise. Reflecting its dominance on the pathophysiological stage the amyloid cascade is central to many of the emerging drug therapies. The long preclinical phase of the disease requires robust biomarker means of identifying those at risk if timely intervention is to be possible. PMID:23707728

  12. Parkinson's Disease

    MedlinePlus

    Parkinson's disease (PD) is a type of movement disorder. It happens when nerve cells in the brain don't ... coordination As symptoms get worse, people with the disease may have trouble walking, talking, or doing simple ...

  13. Parkinson disease

    MedlinePlus

    Nerve cells use a brain chemical called dopamine to help control muscle movement. With Parkinson disease, the brain cells that make dopamine slowly die. Without dopamine, the cells that control movement ...

  14. Parkinson's Disease

    MedlinePlus

    ... cells make and use a brain chemical called dopamine (say: DOH-puh-meen) to send messages to ... coordinate body movements. When someone has Parkinson's disease, dopamine levels are low. So, the body doesn't ...

  15. Flavonoids and dementia: an update.

    PubMed

    Orhan, I E; Daglia, M; Nabavi, S F; Loizzo, M R; Sobarzo-Sánchez, E; Nabavi, S M

    2015-01-01

    Dementia is a strongly age-related syndrome due to cognitive decline that can be considered a typical example of the combination of physiological and pathological aging-associated changes occurring in old people; it ranges from intact cognition to mild cognitive impairment, which is an intermediate stage of cognitive deterioration, and dementia. The spread of this syndrome has induced to study and try to reduce dementia modifiable risk factors. They include insulin resistance and hyperinsulinaemia, high blood pressure, obesity, smoking, depression, cognitive inactivity or low educational attainment as well as physical inactivity and incorrect diet, which can be considered one of the most important factors. One emerging strategy to decrease the prevalence of mild cognitive impairment and dementia may be the use of nutritional interventions. In the last decade, prospective data have suggested that high fruit and vegetable intakes are related to improved cognitive functions and reduced risks of developing a neurodegenerative process. The protective effects against neurodegeneration could be in part due to the intake of flavonoids that have been associated with several health benefits such as antioxidant and anti-inflammatory activities, increased neuronal signaling, and improved metabolic functions. The present article is aimed at reviewing scientific studies that show the protective effects of flavonoid intake against mild cognitive impairment and dementia. PMID:25515512

  16. Sleepiness in Idiopathic REM Sleep Behavior Disorder and Parkinson Disease

    PubMed Central

    Arnulf, Isabelle; Neutel, Dulce; Herlin, Bastien; Golmard, Jean-Louis; Leu-Semenescu, Smaranda; Cochen de Cock, Valérie; Vidailhet, Marie

    2015-01-01

    Objective: To determine whether patients with idiopathic and symptomatic RBD were sleepier than controls, and if sleepiness in idiopathic RBD predicted earlier conversion to Parkinson disease. Methods: The Epworth Sleepiness Scale (ESS) and its determinants were compared at the time of a video-polysomnography for an RBD diagnosis in patients with idiopathic RBD, in patients with Parkinson disease, and in controls. Whether sleepiness at time of RBD diagnosis predicted an earlier conversion to neurodegenerative diseases was retrospectively analyzed in the followed-up patients. Results: The 75 patients with idiopathic RBD were sleepier (ESS: 7.8 ± 4.6) at the time of RBD diagnosis than 74 age- and sex-matched controls (ESS: 5.0 ± 3.6, P < 0.0001). They reached the levels of 114 patients with Parkinson disease (ESS: 8.7 ± 4.8), whether they had (n = 78) or did not have (n = 36) concomitant RBD. The severity of sleepiness in idiopathic RBD correlated with younger age, but not with sleep measures. Among the 69 patients with idiopathic RBD who were followed up for a median 3 years (1–15 years), 16 (23.2%) developed parkinsonism (n = 6), dementia (n = 6), dementia plus parkinsonism (n = 2), and multiple system atrophy (n = 2). An ESS greater than 8 at time of RBD diagnosis predicted a shorter time to phenoconversion to parkinsonism and dementia, from RBD onset, and from RBD diagnosis (when adjusted for age and time between RBD onset and diagnosis). Conclusions: Sleepiness is associated with idiopathic REM sleep behavior disorder and predicts more rapid conversion to parkinsonism and dementia, suggesting it is an early marker of neuronal loss in brainstem arousal systems. Citation: Arnulf I, Neutel D, Herlin B, Golmard JL, Leu-Semenescu S, Cochen de Cock V, Vidailhet M. Sleepiness in idiopathic REM sleep behavior disorder and Parkinson disease. SLEEP 2015;38(10):1529–1535. PMID:26085299

  17. Long-Term Impact of Parental Well-Being on Adult Outcomes and Dementia Status in Individuals with Down Syndrome

    ERIC Educational Resources Information Center

    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…

  18. Frontotemporal Dementia.

    PubMed

    Kelley, Roger E; El-Khoury, Ramy

    2016-02-01

    Frontotemporal dementia (FTD) is a not-uncommon explanation for progressive cognitive deficit in patients who often have a genetic susceptibility for such a neurodegenerative process. However, FTD does not seem to identify one particular pathogenetic mechanism but rather a spectrum of pathologies with particular predilection for the frontal and temporal lobes of the brain. There have been various subcategorizations of this form of dementia that have a tendency to be of earlier onset than typical Alzheimer disease and heralded by behavioral or communication manifestations. There is a behavioral variant and a language variant, referred to as primary progressive aphasia. PMID:26613998

  19. Baseline and longitudinal grey matter changes in newly diagnosed Parkinson's disease: ICICLE-PD study.

    PubMed

    Mak, Elijah; Su, Li; Williams, Guy B; Firbank, Michael J; Lawson, Rachael A; Yarnall, Alison J; Duncan, Gordon W; Owen, Adrian M; Khoo, Tien K; Brooks, David J; Rowe, James B; Barker, Roger A; Burn, David J; O'Brien, John T

    2015-10-01

    Mild cognitive impairment in Parkinson's disease is associated with progression to dementia (Parkinson's disease dementia) in a majority of patients. Determining structural imaging biomarkers associated with prodromal Parkinson's disease dementia may allow for the earlier identification of those at risk, and allow for targeted disease modifying therapies. One hundred and five non-demented subjects with newly diagnosed idiopathic Parkinson's disease and 37 healthy matched controls had serial 3 T structural magnetic resonance imaging scans with clinical and neuropsychological assessments at baseline, which were repeated after 18 months. The Movement Disorder Society Task Force criteria were used to classify the Parkinson's disease subjects into Parkinson's disease with mild cognitive impairment (n = 39) and Parkinson's disease with no cognitive impairment (n = 66). Freesurfer image processing software was used to measure cortical thickness and subcortical volumes at baseline and follow-up. We compared regional percentage change of cortical thinning and subcortical atrophy over 18 months. At baseline, cases with Parkinson's disease with mild cognitive impairment demonstrated widespread cortical thinning relative to controls and atrophy of the nucleus accumbens compared to both controls and subjects with Parkinson's disease with no cognitive impairment. Regional cortical thickness at baseline was correlated with global cognition in the combined Parkinson's disease cohort. Over 18 months, patients with Parkinson's disease with mild cognitive impairment demonstrated more severe cortical thinning in frontal and temporo-parietal cortices, including hippocampal atrophy, relative to those with Parkinson's disease and no cognitive impairment and healthy controls, whereas subjects with Parkinson's disease and no cognitive impairment showed more severe frontal cortical thinning compared to healthy controls. At baseline, Parkinson's disease with no cognitive impairment

  20. Update on Vascular Dementia.

    PubMed

    Khan, Ayesha; Kalaria, Raj N; Corbett, Anne; Ballard, Clive

    2016-09-01

    Vascular dementia (VaD) is a major contributor to the dementia syndrome and is described as having problems with reasoning, planning, judgment, and memory caused by impaired blood flow to the brain and damage to the blood vessels resulting from events such as stroke. There are a variety of etiologies that contribute to the development of vascular cognitive impairment and VaD, and these are often associated with other dementia-related pathologies such as Alzheimer disease. The diagnosis of VaD is difficult due to the number and types of lesions and their locations in the brain. Factors that increase the risk of vascular diseases such as stroke, high blood pressure, high cholesterol, and smoking also raise the risk of VaD. Therefore, controlling these risk factors can help lower the chances of developing VaD. This update describes the subtypes of VaD, with details of their complex presentation, associated pathological lesions, and issues with diagnosis, prevention, and treatment. PMID:27502303

  1. Sleep, Cognition and Dementia.

    PubMed

    Porter, Verna R; Buxton, William G; Avidan, Alon Y

    2015-12-01

    The older patient population is growing rapidly around the world and in the USA. Almost half of seniors over age 65 who live at home are dissatisfied with their sleep, and nearly two-thirds of those residing in nursing home facilities suffer from sleep disorders. Chronic and pervasive sleep complaints and disturbances are frequently associated with excessive daytime sleepiness and may result in impaired cognition, diminished intellect, poor memory, confusion, and psychomotor retardation all of which may be misinterpreted as dementia. The key sleep disorders impacting patients with dementia include insomnia, hypersomnolence, circadian rhythm misalignment, sleep disordered breathing, motor disturbances of sleep such as periodic leg movement disorder of sleep and restless leg syndrome, and parasomnias, mostly in the form of rapid eye movement (REM) sleep behavior disorder (RBD). RBD is a pre-clinical marker for a class of neurodegenerative diseases, the "synucleinopathies", and requires formal polysomnographic evaluation. Untreated sleep disorders may exacerbate cognitive and behavioral symptoms in patients with dementia and are a source of considerable stress for bed partners and family members. When left untreated, sleep disturbances may also increase the risk of injury at night, compromise health-related quality of life, and precipitate and accelerate social and economic burdens for caregivers. PMID:26478197

  2. Down Syndrome and Alzheimer's Disease

    MedlinePlus

    ... A A A Share Plus on Google Plus Alzheimer's & Dementia alz.org | IHaveAlz Overview What Is Dementia ... chapter Join our online community Down Syndrome and Alzheimer's Disease As they age, those affected by Down ...

  3. Vascular dementia

    PubMed Central

    Korczyn, Amos D; Vakhapova, Veronika; Grinberg, Lea T

    2012-01-01

    The epidemic grow of dementia causes great concern for the society. It is customary to consider Alzheimer’s disease (AD) as the most common cause of dementia, followed by vascular dementia (VaD). This dichotomous view of a neurodegenerative disease as opposed to brain damage caused by extrinsic factors led to separate lines of research in these two entities. Indeed, accumulated data suggest that the two disorders have additive effects and probably interact; however it is still unknown to what degree. Furthermore, epidemiological studies have shown “vascular” risk factors to be associated with AD. Therefore, a clear distinction between AD and VaD cannot be made in most cases, and is furthermore unhelpful. In the absence of efficacious treatment for the neurodegenerative process, special attention must be given to vascular component, even in patients with presumed mixed pathology. Symptomatic treatment of VaD and AD are similar, although the former is less effective. For prevention of dementia it is important to treat aggressively all factors, even in stroke survivors who do not show evidence of cognitive decline,. In this review, we will give a clinical and pathological picture of the processes leading to VaD and discuss it interaction with AD. PMID:22575403

  4. Hitler's parkinsonism.

    PubMed

    Boettcher, Lillian B; Bonney, Phillip A; Smitherman, Adam D; Sughrue, Michael E

    2015-07-01

    Of the multitude of medical and psychiatric conditions ascribed to Hitler both in his lifetime and since his suicide in April 1945, few are more substantiated than parkinsonism. While the timeline of the development of this condition, as well as its etiology, are debated, there is clear evidence for classic manifestations of the disease, most prominently a resting tremor but also stooped posture, bradykinesia, micrographia, and masked facial expressions, with progression steadily seen over his final years. Though ultimately speculation, some have suggested that Hitler suffered from progressive cognitive and mood disturbances, possibly due to parkinsonism, that affected the course of events in the war. Here, the authors discuss Hitler's parkinsonism in the context of the Third Reich and its eventual destruction, maintaining that ultimately his disease had little effect on the end result. PMID:26126407

  5. Neuroimaging Biomarkers of Neurodegenerative Diseases and Dementia

    PubMed Central

    Risacher, Shannon L.; Saykin, Andrew J.

    2014-01-01

    Neurodegenerative disorders leading to dementia are common diseases that affect many older and some young adults. Neuroimaging methods are important tools for assessing and monitoring pathological brain changes associated with progressive neurodegenerative conditions. In this review, the authors describe key findings from neuroimaging studies (magnetic resonance imaging and radionucleotide imaging) in neurodegenerative disorders, including Alzheimer’s disease (AD) and prodromal stages, familial and atypical AD syndromes, frontotemporal dementia, amyotrophic lateral sclerosis with and without dementia, Parkinson’s disease with and without dementia, dementia with Lewy bodies, Huntington’s disease, multiple sclerosis, HIV-associated neurocognitive disorder, and prion protein associated diseases (i.e., Creutzfeldt-Jakob disease). The authors focus on neuroimaging findings of in vivo pathology in these disorders, as well as the potential for neuroimaging to provide useful information for differential diagnosis of neurodegenerative disorders. PMID:24234359

  6. Viral Parkinsonism

    PubMed Central

    Jang, Haeman; Boltz, David A.; Webster, Robert G.; Smeyne, Richard Jay

    2015-01-01

    Parkinson's disease is a debilitating neurological disorder characterized that affects 1-2% of the adult population over 55 years of age. For the vast majority of cases, the etiology of this disorder is unknown, although it is generally accepted that there is a genetic susceptibility to any number of environmental agents. One such agent may be viruses. It has been shown that numerous viruses can enter the nervous system, i.e. they are neurotropic, and induce a number of encephalopathies. One of the secondary consequences of these encephalopathies can be parkinsonism, that is both transient as well as permanent. One of the most highlighted and controversial cases of viral parkinsonism is that which followed the 1918 influenza outbreak and the subsequent induction of von Economo's encephalopathy. In this review, we discuss the neurological sequelae of infection by influenza virus as well as that of other viruses known to induce parkinsonism including Coxsackie, Japanese encephalitis B, St. Louis, West Nile and HIV viruses. PMID:18760350

  7. Parkinson Disease.

    PubMed

    Capriotti, Teri; Terzakis, Kristina

    2016-06-01

    Parkinson disease (PD) is a progressive neurodegenerative disease that affects one million people in the United States. This article reviews the etiology and pathophysiology of PD, risk factors, clinical manifestations, diagnostic criteria, and treatment of this common disease. Implications for home care clinicians are included. PMID:27243427

  8. Milestones in Parkinson's disease therapeutics.

    PubMed

    Rascol, Olivier; Lozano, Andres; Stern, Matthew; Poewe, Werner

    2011-05-01

    In the mid-1980s, the treatment of Parkinson's disease was quite exclusively centered on dopatherapy and was focusing on dopamine systems and motor symptoms. A few dopamine agonists and a monoamine oxidase B inhibitor (selegiline) were used as adjuncts in advanced Parkinson's disease. In the early 2010s, levodopa remains the gold standard. New insights into the organization of the basal ganglia paved the way for deep brain stimulation, especially of the subthalamic nucleus, providing spectacular improvement of drug-refractory levodopa-induced motor complications. Novel dopamine agonists (pramipexole, ropinirole, rotigotine), catecholmethyltransferase inhibitors (entacapone), and monoamine oxidase B inhibitors (rasagiline) have also been developed to provide more continuous oral delivery of dopaminergic stimulation in order to improve motor outcomes. Using dopamine agonists early, before levodopa, proved to delay the onset of dyskinesia, although this is achieved at the price of potentially disabling daytime somnolence or impulse control disorders. The demonstration of an antidyskinetic effect of the glutamate antagonist amantadine opened the door for novel nondopaminergic approaches of Parkinson's disease therapy. More recently, nonmotor symptoms (depression, dementia, and psychosis) have been the focus of the first randomized controlled trials in this field. Despite therapeutic advances, Parkinson's disease continues to be a relentlessly progressive disorder leading to severe disability. Neuroprotective interventions able to modify the progression of Parkinson's disease have stood out as a failed therapeutic goal over the last 2 decades, despite potentially encouraging results with compounds like rasagiline. Newer molecular targets, new animal models, novel clinical trial designs, and biomarkers to assess disease modification have created hope for future therapeutic interventions. PMID:21626552

  9. Types of Dementia

    MedlinePlus

    ... Is Dementia Types of Dementia Chronic Traumatic Encephalopathy (CTE) Creutzfeldt-Jakob Disease Dementia with Lewy Bodies Down ... Research Traumatic Brain Injury and Chronic Traumatic Encephalopathy (CTE) Awardees Year Researcher Study Name 2015 Jesse Mez ...

  10. What to Ask: Dementia

    MedlinePlus

    ... What to Ask: Dementia Tools and Tips The memory loss and other changes seen in dementia can ... can ask your healthcare proffesional about dementia. Is memory loss a normal part of aging? If so, ...

  11. Restless Legs Syndrome/Willis-Ekbom Disease and Periodic Limb Movements in Sleep in the Elderly with and without Dementia.

    PubMed

    Figorilli, Michela; Puligheddu, Monica; Ferri, Raffaele

    2015-09-01

    There is great interest in the study of sleep in healthy and cognitively impaired elderly. Sleep disorders have been related to quality of aging. Sleep-related movements are a frequent cause of disordered sleep and daytime sleepiness. Restless legs syndrome/Willis-Ekbom disease (RLS/WED) is often unrecognized in the elderly. This review explores RLS/WED in the elderly population. The elderly population may be subdivided into 3 groups: healthy, dependent, and frail. The RLS/WED could be a predictor for lower physical function; its burden on quality of life and health care-related costs, in the elderly, should be an important clinical and public health concern. PMID:26329443

  12. Progression of Parkinson's Disease

    MedlinePlus

    ... National HelpLine Educational Publications Online Seminars Parkinson's News Parkinson's HelpLine Learn More Educational Materials Do you want ... out daily activities, and treatment complications. Severity of Parkinson's Below are some descriptions of mild, moderate and ...

  13. Parkinson's Disease Foundation

    MedlinePlus

    ... Parkinson’s View All News PDF at the 4th World Parkinson Congress Tuesday, September 20-Friday, September 23 ... the Parkinson's Disease Foundation (PDF) at the 4th World Parkinson Congress, a gathering of the international Parkinson’s ...

  14. The frontotemporal dementia-motor neuron disease continuum.

    PubMed

    Burrell, James R; Halliday, Glenda M; Kril, Jillian J; Ittner, Lars M; Götz, Jürgen; Kiernan, Matthew C; Hodges, John R

    2016-08-27

    Early reports of cognitive and behavioural deficits in motor neuron disease might have been overlooked initially, but the concept of a frontotemporal dementia-motor neuron disease continuum has emerged during the past decade. Frontotemporal dementia-motor neuron disease is now recognised as an important dementia syndrome, which presents substantial challenges for diagnosis and management. Frontotemporal dementia, motor neuron disease, and frontotemporal dementia-motor neuron disease are characterised by overlapping patterns of TAR DNA binding protein (TDP-43) pathology, while the chromosome 9 open reading frame 72 (C9orf72) repeat expansion is common across the disease spectrum. Indeed, the C9orf72 repeat expansion provides important clues to disease pathogenesis and suggests potential therapeutic targets. Variable diagnostic criteria identify motor, cognitive, and behavioural deficits, but further refinement is needed to define the clinical syndromes encountered in frontotemporal dementia-motor neuron disease. PMID:26987909

  15. Clinical features and multidisciplinary approaches to dementia care

    PubMed Central

    Grand, Jacob HG; Caspar, Sienna; MacDonald, Stuart WS

    2011-01-01

    Dementia is a clinical syndrome of widespread progressive deterioration of cognitive abilities and normal daily functioning. These cognitive and behavioral impairments pose considerable challenges to individuals with dementia, along with their family members and caregivers. Four primary dementia classifications have been defined according to clinical and research criteria: 1) Alzheimer’s disease; 2) vascular dementias; 3) frontotemporal dementias; and 4) dementia with Lewy bodies/Parkinson’s disease dementia. The cumulative efforts of multidisciplinary healthcare teams have advanced our understanding of dementia beyond basic descriptions, towards a more complete elucidation of risk factors, clinical symptoms, and neuropathological correlates. The characterization of disease subtypes has facilitated targeted management strategies, advanced treatments, and symptomatic care for individuals affected by dementia. This review briefly summarizes the current state of knowledge and directions of dementia research and clinical practice. We provide a description of the risk factors, clinical presentation, and differential diagnosis of dementia. A summary of multidisciplinary team approaches to dementia care is outlined, including management strategies for the treatment of cognitive impairments, functional deficits, and behavioral and psychological symptoms of dementia. The needs of individuals with dementia are extensive, often requiring care beyond traditional bounds of medical practice, including pharmacologic and non-pharmacologic management interventions. Finally, advanced research on the early prodromal phase of dementia is reviewed, with a focus on change-point models, trajectories of cognitive change, and threshold models of pathological burden. Future research goals are outlined, with a call to action for social policy initiatives that promote preventive lifestyle behaviors, and healthcare programs that will support the growing number of individuals affected by

  16. [The other dementias: the neuropathology of the non-Alzheimer's disease dementias].

    PubMed

    Hamilton, R L

    Alzheimer's disease (AD) is one of the most common causes of dementia, but requires neuropathological verification for a definitive diagnosis because there are a number of other neurodegenerative diseases that may present with dementia. Some of these disorders have considerable overlap both clinically and pathologically with AD, while others have distinct clinical and pathological profiles. Vascular dementia and dementia with Lewy bodies (DLB) have the greatest overlap with AD and considerable controversy still surrounds the exact contribution of the non AD pathology to the dementia syndrome. The frontotemporal dementias are loosely united by clinical presentation, but are pathologically heterogeneous and include Pick's disease, dementia lacking distinctive histopathology, motor neuron disease inclusion dementia and corticobasal degeneration (CBD). CBD can be difficult to distinguish from progressive supranuclear palsy, especially when the latter lacks the distinctive gaze palsy. Finally, Creutzfeldt Jakob disease (CJD) may be difficult to distinguish from AD when the symptoms progress at an atypically slow pace. Recently, a new variant of CJD (vCJD) that has been linked to bovine spongiform encephalopathy ('mad cow' disease) has heightened awareness of these prion protein disorders. The neuropathological criteria for the diagnosis of these non AD dementia disorders will be reviewed. PMID:12938072

  17. Compulsive singing: another aspect of punding in Parkinson's disease.

    PubMed

    Bonvin, Christophe; Horvath, Judit; Christe, Blaise; Landis, Theodor; Burkhard, Pierre R

    2007-11-01

    We report on two patients with advanced Parkinson's disease who were exhibiting a peculiar and stereotyped behavior characterized by an irrepressible need to sing compulsively when under high-dose dopamine replacement therapy. Sharing many features with punding, this singing behavior is proposed as a distinct manifestation of the dopamine dysregulation syndrome in Parkinson's disease. PMID:17696122

  18. PET/MR in dementia and other neurodegenerative diseases.

    PubMed

    Barthel, Henryk; Schroeter, Matthias L; Hoffmann, Karl-Titus; Sabri, Osama

    2015-05-01

    The spectrum of neurodegenerative diseases covers the dementias, parkinsonian syndromes, Huntington disease, amyotrophic lateral sclerosis, and prion diseases. In these entities, brain MRI is often used in clinical routine to exclude other pathologies and to demonstrate specific atrophy patterns. [18F]FDG PET delivers early and sensitive readouts of neural tissue loss, and more specific PET tracers currently in use clinically target β-amyloid plaques or dopaminergic deficiency. The recent integration of PET into MR technology offers a new chance to improve early and differential diagnosis of many neurodegenerative diseases. Initial evidence in the literature is available to support this notion. New emerging PET tracers, such as tracers that bind to tau or α-synuclein aggregates, as well as MR techniques, like diffusion-tensor imaging, resting-state functional MRI, and arterial spin labeling, have the potential to broaden the diagnostic capabilities of combined PET/MRI to image dementias, Parkinson disease, and other neurodegenerative diseases. The ultimate goal is to establish combined PET/MRI as a first-line imaging technique to provide, in a one-stop-shop fashion with improved patient comfort, all biomarker information required to increase diagnostic confidence toward specific diagnoses. The technical challenge of accurate PET data attenuation correction within PET/MRI systems needs yet to be solved. Apart from the projected clinical routine applications, future research would need to answer the questions of whether combined brain PET/MRI is able to improve basic research of neurodegenerative diseases and antineurodegeneration drug testing. PMID:25841277

  19. Incidence of Dementia in Older Adults with Intellectual Disabilities

    ERIC Educational Resources Information Center

    Strydom, Andre; Chan, Trevor; King, Michael; Hassiotis, Angela; Livingston, Gill

    2013-01-01

    Dementia may be more common in older adults with intellectual disability (ID) than in the general population. The increased risk for Alzheimer's disease in people with Down syndrome (DS) is well established, but much less is known about dementia in adults with ID who do not have DS. We estimated incidence rates from a longitudinal study of…

  20. Tests and Medical Conditions Associated with Dementia Diagnosis

    ERIC Educational Resources Information Center

    Burt, Diana B.; Primeaux-Hart, Sharon; Loveland, Katherine A.; Cleveland, Lynne A.; Lewis, Kay R.; Lesser, Jary; Pearson, Pamela L.

    2005-01-01

    Diagnosis of dementia in adults with intellectual disabilities requires documentation of clinically significant declines in memory and other cognitive skills, as well as changes in everyday and emotional functioning. To improve diagnostic accuracy in adults with Down syndrome, the authors examined conditions often associated with dementia, as well…

  1. Targeting the α7 nicotinic acetylcholine receptor to prevent progressive dementia and improve cognition in adults with Down's syndrome.

    PubMed

    Deutsch, Stephen I; Burket, Jessica A; Benson, Andrew D

    2014-10-01

    As persons with Down's syndrome (DS) age into the third decade and beyond, they develop Alzheimer's disease (AD)-like histopathological changes in brain and may manifest progressive worsening of adaptive functions. Increasingly, persons with DS have near-normal to normal life spans; thus, it has become a therapeutic imperative to preserve adaptive functions and ability to live as independently as possible in the least restrictive environment throughout adulthood. Data suggest that these histopathological changes and worsening adaptive functions result, at least in part, from the binding of the amyloidogenic Aβ1-42 peptide to α7 nicotinic acetylcholine receptors (α7nAChRs) on the surface of neurons, which can lead to the internalization of the tightly-bound complex and cell lysis. Pharmacotherapeutic targeting of the α7nAChR may inhibit the creation of the Aβ1-42-α7nAChR complex, which has been observed both intraneuronally and as a component of the amyloid plaque seen in AD. Additionally, selective α7nAChR agonists may improve memory and cognition independently of their potential ability to attenuate the cytotoxicity of Aβ1-42 and retard the deposition of amyloid plaques in adults with DS. However, there are conflicting data supporting an antagonist strategy to improve cognition in the presence of elevated levels of Aβ amyloidogenic peptides, as well as to prevent emergence of pyramidal neuron hyperexcitability. A major challenge to the implementation of clinical trials of targeted α7nAChR interventions in adults with DS will be the ability to detect medication-induced changes in cognition in the context of intellectual disability. The Review will consider some of the current evidence supporting both the role of the Aβ1-42-α7nAChR complex in the pathogenesis of the AD-like histopathology in adult persons with DS, and pharmacotherapeutic interventions with α7nAChR agonists. PMID:24865150

  2. Senile dementia.

    PubMed

    Terry, R D

    1978-12-01

    The Alzheimer type of senile dementia (SDAT) accounts for more than 50% of such cases, and is a very common disorder as well as being very costly in emotional, economic, and medical terms. It carries a markedly shortened life expectancy. Gray-to-white-matter ratios change and the brain shrinks slightly in the course of normal aging, but SDAT brains may not be significantly more atrophic than are normal controls. Cortical neurons are diminished in number in normal aging, but counts from frontal and midtemporal regions of SDAT specimens are not different from age-matched controls. There is loss of dendrites and of dendritic spines in both normal and abnormal aged specimens. Neurofibrillary tangles are made up of paired helical filaments that appear to be chemically and immunologically related to normal neurofibers. Neuritic plaques are made up of an amyloid core surrounded by abnormal axonal endings. Both plaques and tangles are to be correlated with the presence of senile dementia. There is some evidence for an infectious etiology of SDAT. Choline acetyltransferase is markedly reduced in cortical tissue of these patients, but the muscarinic receptors of acetylcholine are normal. PMID:720637

  3. Characterization of frontotemporal dementia and/or amyotrophic lateral sclerosis associated with the GGGGCC repeat expansion in C9ORF72.

    PubMed

    Boeve, Bradley F; Boylan, Kevin B; Graff-Radford, Neill R; DeJesus-Hernandez, Mariely; Knopman, David S; Pedraza, Otto; Vemuri, Prashanthi; Jones, David; Lowe, Val; Murray, Melissa E; Dickson, Dennis W; Josephs, Keith A; Rush, Beth K; Machulda, Mary M; Fields, Julie A; Ferman, Tanis J; Baker, Matthew; Rutherford, Nicola J; Adamson, Jennifer; Wszolek, Zbigniew K; Adeli, Anahita; Savica, Rodolfo; Boot, Brendon; Kuntz, Karen M; Gavrilova, Ralitza; Reeves, Andrew; Whitwell, Jennifer; Kantarci, Kejal; Jack, Clifford R; Parisi, Joseph E; Lucas, John A; Petersen, Ronald C; Rademakers, Rosa

    2012-03-01

    Numerous kindreds with familial frontotemporal dementia and/or amyotrophic lateral sclerosis have been linked to chromosome 9, and an expansion of the GGGGCC hexanucleotide repeat in the non-coding region of chromosome 9 open reading frame 72 has recently been identified as the pathogenic mechanism. We describe the key characteristics in the probands and their affected relatives who have been evaluated at Mayo Clinic Rochester or Mayo Clinic Florida in whom the hexanucleotide repeat expansion were found. Forty-three probands and 10 of their affected relatives with DNA available (total 53 subjects) were shown to carry the hexanucleotide repeat expansion. Thirty-six (84%) of the 43 probands had a familial disorder, whereas seven (16%) appeared to be sporadic. Among examined subjects from the 43 families (n = 63), the age of onset ranged from 33 to 72 years (median 52 years) and survival ranged from 1 to 17 years, with the age of onset <40 years in six (10%) and >60 in 19 (30%). Clinical diagnoses among examined subjects included behavioural variant frontotemporal dementia with or without parkinsonism (n = 30), amyotrophic lateral sclerosis (n = 18), frontotemporal dementia/amyotrophic lateral sclerosis with or without parkinsonism (n = 12), and other various syndromes (n = 3). Parkinsonism was present in 35% of examined subjects, all of whom had behavioural variant frontotemporal dementia or frontotemporal dementia/amyotrophic lateral sclerosis as the dominant clinical phenotype. No subject with a diagnosis of primary progressive aphasia was identified with this mutation. Incomplete penetrance was suggested in two kindreds, and the youngest generation had significantly earlier age of onset (>10 years) compared with the next oldest generation in 11 kindreds. Neuropsychological testing showed a profile of slowed processing speed, complex attention/executive dysfunction, and impairment in rapid word retrieval. Neuroimaging studies showed bilateral frontal abnormalities

  4. Characterization of frontotemporal dementia and/or amyotrophic lateral sclerosis associated with the GGGGCC repeat expansion in C9ORF72

    PubMed Central

    Boylan, Kevin B.; Graff-Radford, Neill R.; DeJesus-Hernandez, Mariely; Knopman, David S.; Pedraza, Otto; Vemuri, Prashanthi; Jones, David; Lowe, Val; Murray, Melissa E.; Dickson, Dennis W.; Josephs, Keith A.; Rush, Beth K.; Machulda, Mary M.; Fields, Julie A.; Ferman, Tanis J.; Baker, Matthew; Rutherford, Nicola J.; Adamson, Jennifer; Wszolek, Zbigniew K.; Adeli, Anahita; Savica, Rodolfo; Boot, Brendon; Kuntz, Karen M.; Gavrilova, Ralitza; Reeves, Andrew; Whitwell, Jennifer; Kantarci, Kejal; Jack, Clifford R.; Parisi, Joseph E.; Lucas, John A.; Petersen, Ronald C.; Rademakers, Rosa

    2012-01-01

    Numerous kindreds with familial frontotemporal dementia and/or amyotrophic lateral sclerosis have been linked to chromosome 9, and an expansion of the GGGGCC hexanucleotide repeat in the non-coding region of chromosome 9 open reading frame 72 has recently been identified as the pathogenic mechanism. We describe the key characteristics in the probands and their affected relatives who have been evaluated at Mayo Clinic Rochester or Mayo Clinic Florida in whom the hexanucleotide repeat expansion were found. Forty-three probands and 10 of their affected relatives with DNA available (total 53 subjects) were shown to carry the hexanucleotide repeat expansion. Thirty-six (84%) of the 43 probands had a familial disorder, whereas seven (16%) appeared to be sporadic. Among examined subjects from the 43 families (n = 63), the age of onset ranged from 33 to 72 years (median 52 years) and survival ranged from 1 to 17 years, with the age of onset <40 years in six (10%) and >60 in 19 (30%). Clinical diagnoses among examined subjects included behavioural variant frontotemporal dementia with or without parkinsonism (n = 30), amyotrophic lateral sclerosis (n = 18), frontotemporal dementia/amyotrophic lateral sclerosis with or without parkinsonism (n = 12), and other various syndromes (n = 3). Parkinsonism was present in 35% of examined subjects, all of whom had behavioural variant frontotemporal dementia or frontotemporal dementia/amyotrophic lateral sclerosis as the dominant clinical phenotype. No subject with a diagnosis of primary progressive aphasia was identified with this mutation. Incomplete penetrance was suggested in two kindreds, and the youngest generation had significantly earlier age of onset (>10 years) compared with the next oldest generation in 11 kindreds. Neuropsychological testing showed a profile of slowed processing speed, complex attention/executive dysfunction, and impairment in rapid word retrieval. Neuroimaging studies showed bilateral

  5. Hallucinations in Parkinson disease.

    PubMed

    Diederich, Nico J; Fénelon, Gilles; Stebbins, Glenn; Goetz, Christopher G

    2009-06-01

    Patients with Parkinson disease (PD) can experience hallucinations (spontaneous aberrant perceptions) and illusions (misinterpretations of real perceptual stimuli). Of such phenomena, visual hallucinations (VHs) and illusions are the most frequently encountered, although auditory, olfactory and tactile hallucinations can also occur. In cross-sectional studies, VHs occur in approximately one-third of patients, but up to three-quarters of patients might develop VHs during a 20-year period. Hallucinations can have substantial psychosocial effects and, historically, were the main reason for placing patients in nursing homes. Concomitant or overlapping mechanisms are probably active during VHs, and these include the following: central dopaminergic overactivity and an imbalance with cholinergic neurotransmission; dysfunction of the visual pathways, including specific PD-associated retinopathy and functional alterations of the extrastriate visual pathways; alterations of brainstem sleep-wake and dream regulation; and impaired attentional focus. Possible treatments include patient-initiated coping strategies, a reduction of antiparkinson medications, atypical neuroleptics and, potentially, cholinesterase inhibitors. Evidence-based studies, however, only support the use of one atypical neuroleptic, clozapine, and only in patients without dementia. Better phenomenological discrimination, combined with neuroimaging tools, should refine therapeutic options and improve prognosis. The aim of this Review is to present epidemiological, phenomenological, pathophysiological and therapeutic aspects of hallucinations in PD. PMID:19498436

  6. Parkinson's disease and insomnia.

    PubMed

    Ylikoski, Ari; Martikainen, Kirsti; Sieminski, Mariusz; Partinen, Markku

    2015-11-01

    There is a broad spectrum of sleep disturbances observed in Parkinson's disease (PD). The prevalence of symptoms of insomnia and chronic inability to sleep and their association with other sleep disorders were studied. Altogether 1447 randomly selected Parkinson patients, aged 43-89 years, participated in a questionnaire study. A structured questionnaire with 207 items was based on the Basic Nordic Sleep questionnaire. Questions on demographics, PD, REM Sleep Behavior Disorder, and other issues were included. The response rate was 59 % (N = 854), and of these 81 % returned fully answered questionnaire (N = 689). Prevalence of chronic inability to sleep was 36.9 % (95 % CI 33.3-40.5). Difficulty of initiating sleep was 18.0 % (95 % CI 15.1-20.9), disrupted sleep 81.54 % (78.5-84.4), awakenings during night 31.3 % (27.8-34.8), early morning awakenings 40.4 % (36.8-44.1) and non-restorative sleep 38.5 % (34.8-42.1). In the logistic regression models, poor quality of life and restless legs syndrome correlated significantly with chronic insomnia disorder. Disrupted sleep and early morning awakenings were the most common insomnia symptoms. PD patients do not seem to have difficulties in sleep initiation. Insomnia symptoms including disruptive sleep and non-restorative sleep are common in patients with Parkinson's disease. Inability to sleep is more common as comorbidity than a single sleep problem. PMID:26099862

  7. Auditory object cognition in dementia.

    PubMed

    Goll, Johanna C; Kim, Lois G; Hailstone, Julia C; Lehmann, Manja; Buckley, Aisling; Crutch, Sebastian J; Warren, Jason D

    2011-07-01

    The cognition of nonverbal sounds in dementia has been relatively little explored. Here we undertook a systematic study of nonverbal sound processing in patient groups with canonical dementia syndromes comprising clinically diagnosed typical amnestic Alzheimer's disease (AD; n=21), progressive nonfluent aphasia (PNFA; n=5), logopenic progressive aphasia (LPA; n=7) and aphasia in association with a progranulin gene mutation (GAA; n=1), and in healthy age-matched controls (n=20). Based on a cognitive framework treating complex sounds as 'auditory objects', we designed a novel neuropsychological battery to probe auditory object cognition at early perceptual (sub-object), object representational (apperceptive) and semantic levels. All patients had assessments of peripheral hearing and general neuropsychological functions in addition to the experimental auditory battery. While a number of aspects of auditory object analysis were impaired across patient groups and were influenced by general executive (working memory) capacity, certain auditory deficits had some specificity for particular dementia syndromes. Patients with AD had a disproportionate deficit of auditory apperception but preserved timbre processing. Patients with PNFA had salient deficits of timbre and auditory semantic processing, but intact auditory size and apperceptive processing. Patients with LPA had a generalised auditory deficit that was influenced by working memory function. In contrast, the patient with GAA showed substantial preservation of auditory function, but a mild deficit of pitch direction processing and a more severe deficit of auditory apperception. The findings provide evidence for separable stages of auditory object analysis and separable profiles of impaired auditory object cognition in different dementia syndromes. PMID:21689671

  8. An update on pharmacological, pharmacokinetic properties and drug-drug interactions of rotigotine transdermal system in Parkinson's disease and restless legs syndrome.

    PubMed

    Elshoff, Jan-Peer; Cawello, Willi; Andreas, Jens-Otto; Mathy, Francois-Xavier; Braun, Marina

    2015-04-01

    This narrative review reports on the pharmacological and pharmacokinetic properties of rotigotine, a non-ergolinic D₃/D₂/D₁ dopamine receptor agonist approved for the treatment of early- and advanced-stage Parkinson's disease (PD) and moderate to severe restless legs syndrome (RLS). Rotigotine is formulated as a transdermal patch providing continuous drug delivery over 24 h, with a plasma concentration profile similar to that of administration via continuous intravenous infusion. Absolute bioavailability after 24 h transdermal delivery is 37 % of the applied rotigotine dose. Following a single administration of rotigotine transdermal system (24-h patch-on period), most of the absorbed drug is eliminated in urine and feces as sulphated and glucuronidated conjugates within 24 h of patch removal. The drug shows a high apparent volume of distribution (>2500 L) and a total body clearance of 300-600 L/h. Rotigotine transdermal system provides dose-proportional pharmacokinetics up to supratherapeutic dose rates of 24 mg/24 h, with steady-state plasma drug concentrations attained within 1-2 days of daily dosing. The pharmacokinetics of rotigotine transdermal patch are similar in healthy subjects, patients with early- or advanced-stage PD, and patients with RLS when comparing dose-normalized area under the plasma concentration-time curve (AUC) and maximum plasma drug concentration (Cmax), as well as half-life and other pharmacokinetic parameters. Also, it is not influenced in a relevant manner by age, sex, ethnicity, advanced renal insufficiency, or moderate hepatic impairment. No clinically relevant drug-drug interactions were observed following co-administration of rotigotine with levodopa/carbidopa, domperidone, or the CYP450 inhibitors cimetidine or omeprazole. Also, pharmacodynamics and pharmacokinetics of an oral hormonal contraceptive were not influenced by rotigotine co-administration. Rotigotine was generally well tolerated, with an adverse event profile

  9. Orthostatic Hypotension in Patients with Parkinson's Disease and Atypical Parkinsonism

    PubMed Central

    Lökk, Johan

    2014-01-01

    Orthostatic hypotension (OH) is one of the commonly occurring nonmotor symptoms in patients with idiopathic Parkinson's disease (IPD) and atypical parkinsonism (AP). We aimed to review current evidences on epidemiology, diagnosis, treatment, and prognosis of OH in patients with IPD and AP. Major electronic medical databases were assessed including PubMed/MEDLINE and Embase up to February 2013. English-written original or review articles with keywords such as “Parkinson's disease,” “atypical parkinsonism,” and “orthostatic hypotension” were searched for relevant evidences. We addressed different issues such as OH definition, epidemiologic characteristics, pathophysiology, testing and diagnosis, risk factors for symptomatic OH, OH as an early sign of IPD, prognosis, and treatment options of OH in parkinsonian syndromes. Symptomatic OH is present in up to 30% of IPD, 80% of multiple system atrophy (MSA), and 27% of other AP patients. OH may herald the onset of PD before cardinal motor symptoms and our review emphasises the importance of its timely diagnosis (even as one preclinical marker) and multifactorial treatment, starting with patient education and lifestyle approach. Advancing age, male sex, disease severity, and duration and subtype of motor symptoms are predisposing factors. OH increases the risk of falls, which affects the quality of life in PD patients. PMID:24634790

  10. Epigenetic Treatment of Neurodegenerative Disorders: Alzheimer and Parkinson Diseases.

    PubMed

    Irwin, Michael H; Moos, Walter H; Faller, Douglas V; Steliou, Kosta; Pinkert, Carl A

    2016-05-01

    Preclinical Research In this review, we discuss epigenetic-driven methods for treating neurodegenerative disorders associated with mitochondrial dysfunction, focusing on carnitinoid antioxidant-histone deacetylase inhibitors that show an ability to reinvigorate synaptic plasticity and protect against neuromotor decline in vivo. Aging remains a major risk factor in patients who progress to dementia, a clinical syndrome typified by decreased mental capacity, including impairments in memory, language skills, and executive function. Energy metabolism and mitochondrial dysfunction are viewed as determinants in the aging process that may afford therapeutic targets for a host of disease conditions, the brain being primary in such thinking. Mitochondrial dysfunction is a core feature in the pathophysiology of both Alzheimer and Parkinson diseases and rare mitochondrial diseases. The potential of new therapies in this area extends to glaucoma and other ophthalmic disorders, migraine, Creutzfeldt-Jakob disease, post-traumatic stress disorder, systemic exertion intolerance disease, and chemotherapy-induced cognitive impairment. An emerging and hopefully more promising approach to addressing these hard-to-treat diseases leverages their sensitivity to activation of master regulators of antioxidant and cytoprotective genes, antioxidant response elements, and mitophagy. Drug Dev Res 77 : 109-123, 2016. © 2016 Wiley Periodicals, Inc. PMID:26899010

  11. Personality and dementia.

    PubMed

    Cipriani, Gabriele; Borin, Gemma; Del Debbio, Alessandro; Di Fiorino, Mario

    2015-03-01

    Personality describes persistent human behavioral responses to broad classes of environmental stimuli. Change in personality may be an early sign of dementia. Our goal was to review scientific literature on the association between personality and dementia. Medline and Google Scholar searches were conducted for relevant articles, chapters, and books published since 1980. Search terms used included personality, dementia, Alzheimer's disease, frontotemporal dementia, dementia with Lewy bodies. People with dementia commonly exhibit changes in personality that sometimes precede the other early clinical manifestations of the condition, such as cognitive impairment. Premorbid personality might be a determining factor so that caricature or exaggeration of original personality emerges as dementia progresses. Although it is generally accepted that these personality changes reflect the impact of progressive brain damage, there are several possible patterns of personality alterations with dementia. Early identification of personality modifications might assist with the timely diagnosis of dementia. PMID:25714255

  12. The Brain in AIDS: Central Nervous System HIV-1 Infection and AIDS Dementia Complex.

    ERIC Educational Resources Information Center

    Price, Richard W.; And Others

    1988-01-01

    Discusses the complicated infection of human immunodeficiency virus type 1 (HIV-1) in its late stages of the acquired immune deficiency syndrome (AIDS) dementia complex. Explains the syndrome's development of abnormalities in cognition, motor performance, and behavior. (TW)

  13. Case Studies Illustrating Focal Alzheimer's, Fluent Aphasia, Late-Onset Memory Loss, and Rapid Dementia.

    PubMed

    Camsari, Gamze Balci; Murray, Melissa E; Graff-Radford, Neill R

    2016-08-01

    Many dementia subtypes have more shared signs and symptoms than defining ones. We review 8 cases with 4 overlapping syndromes and demonstrate how to distinguish the cases. These include focal cortical presentations of Alzheimer's disease (AD; posterior cortical atrophy and corticobasal syndrome [CBS]), fluent aphasia (semantic dementia and logopenic aphasia), late-onset slowly progressive dementia (hippocampal sclerosis and limbic predominant AD) and rapidly progressive dementia (Creutzfeldt-Jakob disease and limbic encephalitis). Recognizing the different syndromes can help the clinician to improve their diagnostic skills, leading to improved patient outcomes by early and accurate diagnosis, prompt treatment, and appropriate counseling and guidance. PMID:27445249

  14. Recognition and management of neuropsychiatric complications in Parkinson's disease

    PubMed Central

    Ferreri, Florian; Agbokou, Catherine; Gauthier, Serge

    2006-01-01

    Parkinson's disease is primarily considered a motor disease characterized by rest tremor, rigidity, bradykinesia and postural disturbances. However, neuropsychiatric complications, including mood and anxiety disorders, fatigue, apathy, psychosis, cognitive impairment, dementia, sleep disorders and addictions, frequently complicate the course of the illness. The pathophysiologic features of these complications are multifaceted and include neuropathophysiologic changes of a degenerative disease, exposure to antiparkinsonian treatments and emotional reactions to having a disabling chronic illness. Changes in mental status have profound implications for the well-being of patients with Parkinson's disease and of their caregivers. Treatment is often efficacious but becomes a challenge in advanced stages of Parkinson's disease. In this article, we review the key clinical features of neuropsychiatric complications in Parkinson's disease as well as what is known about their epidemiologic characteristics, risk factors, pathophysiologic features and management. PMID:17146092

  15. [What is dementia? 2. A fuzzy construct].

    PubMed

    Derouesné, Christian

    2003-03-01

    Since the publication of the third edition of the classification of mental disorders by the American Psychiatric Association, APA (DSM-III) in 1980, a large international consensus has been reached to define dementia as a multiple cognitive deficit centered by memory disturbances, interfering with the daily life activities and related to organic brain lesions. The DSM-III defined a diagnostic strategy in two steps. First to make the diagnosis of dementia according to the clinical criteria and then to specify its etiology. The more recent editions of the APA classification did not significantly modify these recommendations. The use of the DSM criteria has allowed important advances in epidemiological, clinical and therapeutic research. However, several criticisms should be addressed to the clinical diagnostic criteria as well as to the current concept of dementia. First, from a theoretical point of view, dementia is not a disease, not even a disorder. It only corresponds to a conventional collection of symptoms. Actually, clinical symptoms of dementia basically depend of the localization of brain lesions. Therefore, there could not be one single dementia syndrome. Similarly, the basis of the search for one "antidementia drug" should be questioned. From a clinical point of view, the DSM-IV criteria present some inaccuracies regarding the description of deficits in memory and executive functions as well as in the assessment of the interference of the cognitive decline with the activities of daily living. The definition of dementia as a purely cognitive deficit results in neglecting its psychological and relational manifestations which play a large part in the detection of dementia and its acceptance by the family. A major criticism concerns the definition of dementia as a sincle clinical entity with memory deficits as the core symptom. This definition is well adapted to Alzheimer's disease but results in delayed diagnosis or misclassification of dementias such as

  16. Two cases of food aversion with semantic dementia.

    PubMed

    Thompson, Alexandra E; Clark, Camilla N; Hardy, Christopher J; Fletcher, Phillip D; Greene, John; Rohrer, Jonathan D; Warren, Jason D

    2016-06-01

    Accounts of altered eating behavior in semantic dementia generally emphasize gluttony and abnormal food preferences. Here we describe two female patients with no past history of eating disorders who developed early prominent aversion to food in the context of an otherwise typical semantic dementia syndrome. One patient (aged 57) presented features in line with anorexia nervosa while the second patient (aged 58) presented with a syndrome more suggestive of bulimia nervosa. These cases add to the growing spectrum of apparently dichotomous behavior patterns in the frontotemporal dementias and illustrate a potentially under-recognized cause of eating disorders presenting in later life. PMID:26963051

  17. Two cases of food aversion with semantic dementia

    PubMed Central

    Thompson, Alexandra E.; Clark, Camilla N.; Hardy, Christopher J.; Fletcher, Phillip D.; Greene, John; Rohrer, Jonathan D.; Warren, Jason D.

    2016-01-01

    ABSTRACT Accounts of altered eating behavior in semantic dementia generally emphasize gluttony and abnormal food preferences. Here we describe two female patients with no past history of eating disorders who developed early prominent aversion to food in the context of an otherwise typical semantic dementia syndrome. One patient (aged 57) presented features in line with anorexia nervosa while the second patient (aged 58) presented with a syndrome more suggestive of bulimia nervosa. These cases add to the growing spectrum of apparently dichotomous behavior patterns in the frontotemporal dementias and illustrate a potentially under-recognized cause of eating disorders presenting in later life. PMID:26963051

  18. Neuroeconomic dissociation of semantic dementia and behavioural variant frontotemporal dementia.

    PubMed

    Chiong, Winston; Wood, Kristie A; Beagle, Alexander J; Hsu, Ming; Kayser, Andrew S; Miller, Bruce L; Kramer, Joel H

    2016-02-01

    Many neuropsychiatric disorders are marked by abnormal behaviour and decision-making, but prevailing diagnostic criteria for such behaviours are typically qualitative and often ambiguous. Behavioural variant frontotemporal dementia and semantic variant primary progressive aphasia (also called semantic dementia) are two clinical variants of frontotemporal dementia with overlapping but distinct anatomical substrates known to cause profound changes in decision-making. We investigated whether abnormal decision-making in these syndromes could be more precisely characterized in terms of dissociable abnormalities in patients' subjective evaluations of valence (positive versus negative outcome) and of time (present versus future outcome). We presented 28 patients with behavioural variant frontotemporal dementia, 14 patients with semantic variant primary progressive aphasia, 25 patients with Alzheimer's disease (as disease controls), and 61 healthy older control subjects with experimental tasks assaying loss aversion and delay discounting. In general linear models controlling for age, gender, education and Mini-Mental State Examination score, patients with behavioural variant frontotemporal dementia were less averse to losses than control subjects (P < 0.001), while patients with semantic variant primary progressive aphasia discounted delayed rewards more steeply than controls (P = 0.019). There was no relationship between loss aversion and delay discounting across the sample, nor in any of the subgroups. These findings suggest that abnormal behaviours in neurodegenerative disease may result from the disruption of either of two dissociable neural processes for evaluating the outcomes of action. More broadly, these findings suggest a role for computational methods to supplement traditional qualitative characterizations in the differential diagnosis of neuropsychiatric disorders. PMID:26667277

  19. Diabetes, Dementia and Hypoglycemia.

    PubMed

    Meneilly, Graydon S; Tessier, Daniel M

    2016-02-01

    We are experiencing an epidemic of both diabetes and dementia among older adults in this country. The risk for dementia appears to be increased in patients with diabetes, and patients with dementia and diabetes appear to be at greater risk for severe hypoglycemia. In addition, there may be an increased risk for developing dementia by older patients with diabetes who have had episodes of severe hypoglycemia, although this issue is controversial. In this article, we review the factors that contribute to the increased risk for dementia in older adults with diabetes and outline the complex relationships between hypoglycemia and dementia. PMID:26778684

  20. Anosognosia in Dementia.

    PubMed

    Wilson, Robert S; Sytsma, Joel; Barnes, Lisa L; Boyle, Patricia A

    2016-09-01

    Progressive decline in memory (and other functions) is the defining feature of late-life dementia but affected individuals are often unaware of this impairment. This article reviews recent research on anosognosia in dementia, including methods of assessing anosognosia, its prevalence and developmental course in dementia, its occurrence in different forms of dementia, neuroimaging findings, and hypothesized component mechanisms. The results suggest that anosognosia is eventually exhibited by nearly all persons with dementia. Its occurrence is robustly associated with common dementia-related pathologies and damage to memory and self-referential brain networks and their interconnections. PMID:27438597

  1. Cholinergic dysfunction in Parkinson's disease.

    PubMed

    Müller, Martijn L T M; Bohnen, Nicolaas I

    2013-09-01

    There is increasing interest in the clinical effects of cholinergic basal forebrain and tegmental pedunculopontine complex (PPN) projection degeneration in Parkinson's disease (PD). Recent evidence supports an expanded role beyond cognitive impairment, including effects on olfaction, mood, REM sleep behavior disorder, and motor functions. Cholinergic denervation is variable in PD without dementia and may contribute to clinical symptom heterogeneity. Early in vivo imaging evidence that impaired cholinergic integrity of the PPN associates with frequent falling in PD is now confirmed by human post-mortem evidence. Brainstem cholinergic lesioning studies in primates confirm the role of the PPN in mobility impairment. Degeneration of basal forebrain cholinergic projections correlates with decreased walking speed. Cumulatively, these findings provide evidence for a new paradigm to explain dopamine-resistant features of mobility impairments in PD. Recognition of the increased clinical role of cholinergic system degeneration may motivate new research to expand indications for cholinergic therapy in PD. PMID:23943367

  2. Prevalence of Alzheimer's disease and vascular dementia: association with education. The Rotterdam study.

    PubMed Central

    Ott, A.; Breteler, M. M.; van Harskamp, F.; Claus, J. J.; van der Cammen, T. J.; Grobbee, D. E.; Hofman, A.

    1995-01-01

    OBJECTIVE--To estimate the prevalence of dementia and its subtypes in the general population and examine the relation of the disease to education. DESIGN--Population based cross sectional study. SETTING--Ommoord, a suburb of Rotterdam. SUBJECTS--7528 participants of the Rotterdam study aged 55-106 years. RESULTS--474 cases of dementia were detected, giving an overall prevalence of 6.3%. Prevalence ranged from 0.4% (5/1181 subjects) at age 55-59 years to 43.2% (19/44) at 95 years and over. Alzheimer's disease was the main subdiagnosis (339 cases; 72%); it was also the main cause of the pronounced increase in dementia with age. The relative proportion of vascular dementia (76 cases; 16%), Parkinson's disease dementia (30; 6%), and other dementias (24; 5%) decreased with age. A substantially higher prevalence of dementia was found in subjects with a low level of education. The association with education was not due to confounding by cardiovascular disease. CONCLUSIONS--The prevalence of dementia increases exponentially with age. About one third of the population aged 85 and over has dementia. Three quarters of all dementia is due to Alzheimer's disease. In this study an inverse dose-response relation was found between education and dementia--in particular, Alzheimer's disease. PMID:7728032

  3. Accuracy of the National Institute for Neurological Disorders and Stroke/Society for Progressive Supranuclear Palsy and neuroprotection and natural history in Parkinson plus syndromes criteria for the diagnosis of progressive supranuclear palsy.

    PubMed

    Respondek, Gesine; Roeber, Sigrun; Kretzschmar, Hans; Troakes, Claire; Al-Sarraj, Safa; Gelpi, Ellen; Gaig, Carles; Chiu, Wang Zheng; van Swieten, John C; Oertel, Wolfgang H; Höglinger, Günter U

    2013-04-01

    Autopsy is the diagnostic gold standard for progressive supranuclear palsy (PSP). The National Institute of Neurological Disorders and Stroke and Society for Progressive Supranuclear Palsy (NINDS-SPSP) criteria for the clinical diagnosis of "probable" PSP are thought to possess high specificity and low sensitivity. The NINDS-SPSP criteria for "possible" PSP are considered to increase sensitivity at the expense of specificity. The Neuroprotection and Natural History in Parkinson Plus Syndromes (NNIPPS) criteria are intended to improve sensitivity while maintaining high specificity. The aim of this study was to conduct a clinicopathological evaluation of the NINDS-SPSP and NNIPPS criteria in tertiary neurological centers. Defined clinical features and their year of onset were recorded by chart review in neuropathologically diagnosed patients with PSP, Parkinsons's disease (PD), MSA parkinsonism and corticobasal degeneration from four European brain banks. Fulfillment of the clinical diagnostic criteria was verified for each year after disease onset and for the final antemortem record. We analyzed 98 PSP patients and 46 disease controls. The NINDS-SPSP "probable" criteria yielded shorter time to diagnosis, slightly higher specificity and positive predictive value (PPV), and similar sensitivity, compared with the NNIPPS criteria. Unexpectedly, the NINDS-SPSP "possible" criteria yielded the lowest sensitivity, specificity, and PPV. A combination of NINDS-SPSP possible and probable criteria yielded the highest sensitivity. We suggest that the NINDS-SPSP probable criteria might be preferred for recruitment of patients for clinical trials, where an early and specific diagnosis is important. For routine clinical care, where high sensitivity is crucial, a combination of NINDS possible and probable criteria might be preferred. PMID:23436751

  4. [Differential diagnosis of dementia with lewy bodies].

    PubMed

    Orimo, Satoshi

    2015-04-01

    Kosaka and colleagues first reported dementia with Lewy bodies (DLB) in 1976. They have also established the concept of DLB. It is important to differentiate DLB from other dementia, especially Alzheimer disease (AD), because the medical treatment, management, and prognosis of DLB and AD are different. We have used several clinical features and imaging tools to differentiate between DLB and AD. With regard to clinical features, patients with DLB have relatively mild memory disturbances and fluctuating cognition. However, compared to patients with AD they have more severe disturbances of attention and executive, visuospatial functions, visual hallucination, depression, autonomic symptoms. In addition, they show the presence of REM sleep behavior disorder and idiopathic parkinsonism. On performing imaging analysis, patients with DLB showed milder atrophy in the medial temporal lobe on brain MRI, reduced occipital activity on SPECT or PET, reduced MIBG uptake on MIBG cardiac scintigraphy, and low dopamine transporter activity in the basal ganglia on SPECT or PET. PMID:25846590

  5. UPDATE ON DEMENTIA WITH LEWY BODIES

    PubMed Central

    Karantzoulis, Stella; Galvin, James E.

    2014-01-01

    Dementia with Lewy bodies (DLB) is the second most common form of dementia after Alzheimer disease (AD). DLB is characterized pathologically by Lewy body and Lewy neuritic pathology, often with variable levels of Alzheimer-type pathology. Core clinical features include fluctuating cognition, visual hallucinations, and parkinsonism resulting in greater impairments of quality of life, more caregiver burden, and higher health-related costs compared with AD. These issues, together with a high sensitivity to adverse events with treatment with antipsychotic agents, make the need for an early and accurate diagnosis of DLB essential. Unfortunately, current consensus criteria are highly specific but lack sufficient sensitivity. Use of composite risk scores may improve accuracy of clinical diagnosis. Imaging findings, particularly targeting dopaminergic systems have shown promise as potential markers to differentiate DLB from AD. A combination of non-pharmacologic treatments and pharmacotherapy interventions may maximize cognitive function and overall quality of life in DLB patients. PMID:25379359

  6. Prodromal dementia with Lewy bodies.

    PubMed

    Fujishiro, Hiroshige; Nakamura, Shinichiro; Sato, Kiyoshi; Iseki, Eizo

    2015-07-01

    Dementia with Lewy bodies (DLB) is the second most common neurodegenerative dementing disorder after Alzheimer's disease (AD), but there is limited information regarding the prodromal DLB state compared with that of AD. Parkinson's disease (PD) and DLB share common prodromal symptoms with Lewy body disease (LBD), allowing us to use a common strategy for identifying the individuals with an underlying pathophysiology of LBD. Dysautonomia, olfactory dysfunction, rapid eye movement sleep behavior disorder (RBD) and psychiatric symptoms antedate the onset of dementia by years or even decades in patients with DLB. Although RBD is the most potentially accurate prodromal predictor of DLB, disease progression before the onset of dementia could differ between the prodromal DLB state with and without RBD. Experts who specialize in idiopathic RBD and DLB might need communication in order to clarify the clinical relevance of RBD with the disease progression of DLB. The presence of prodromal LBD symptoms or findings of occipital hypoperfusion/hypometabolism helps us to predict the possible pathophysiological process of LBD in non-demented patients. This approach might provide the opportunity for additional neuroimaging, including cardiac (123) I-metaiodobenzylguanidine scintigraphy and dopamine transporter imaging. Although limited radiological findings in patients with prodromal DLB states have been reported, there is now a need for larger clinical multisite studies with pathological verification. The long prodromal phase of DLB provides a critical opportunity for potential intervention with disease-modifying therapy, but only if we are able to clearly identify the diversity in the clinical courses of DLB. In the present article, we reviewed the limited literature regarding the clinical profiles of prodromal DLB. PMID:25690399

  7. Frontotemporal dementia: An updated overview

    PubMed Central

    Mohandas, E.; Rajmohan, V.

    2009-01-01

    Frontotemporal dementia (FTD) is a progressive neurodegenerative syndrome occurring between 45 and 65 years. The syndrome is also called frontotemporal lobar degeneration (FTLD). However, FTLD refers to a larger group of disorders FTD being one of its subgroups. The other subgroups of FTLD are progressive nonfluent aphasia (PFNA), and semantic dementia (SD). FTLD is characterized by atrophy of prefrontal and anterior temporal cortices. FTD occurs in 5-15% of patients with dementia and it is the third most common degenerative dementia. FTD occurs with equal frequency in both sexes. The age of onset is usually between 45 and 65 years though it may range anywhere from 21 to 81 years. The usual course is one of progressive clinicopathological deterioration with mortality within 6-8 years. Unlike Alzheimer’s disease (AD), this condition has a strong genetic basis and family history of FTD is seen in 40-50% of cases. FTD is a genetically complex disorder inherited as an autosomal dominant trait with high penetrance in majority of cases. Genetic linkage studies have revealed FTLD loci on chromosome 3p, 9, 9p, and 17q. The most prevalent genes are PGRN (progranulin) and MAPT (microtubule-associated protein tau), both located on chromosome 17q21. More than 15 different pathologies can underlie FTD and related disorders and it has four major types of pathological features: (1) microvacuolation without neuronal inclusions, (2) microvacuolation with ubiquitinated rounded intraneuronal inclusions and dystrophic neurites FTLD-ubiquitinated (FTLD-U), (3) transcortical gliosis with tau-reactive rounded intraneuronal inclusions, (4) microvacuolation and taupositive neurofibrillary tangles. Behavior changes are the most common initial symptom of FTD (62%), whereas speech and language problems are most common in NFPA (100%) and SD (58%). There are no approved drugs for the management of FTD and trials are needed to find effective agents. Non-pharmacological treatment and caregiver

  8. Frontotemporal dementia: An updated overview.

    PubMed

    Mohandas, E; Rajmohan, V

    2009-01-01

    Frontotemporal dementia (FTD) is a progressive neurodegenerative syndrome occurring between 45 and 65 years. The syndrome is also called frontotemporal lobar degeneration (FTLD). However, FTLD refers to a larger group of disorders FTD being one of its subgroups. The other subgroups of FTLD are progressive nonfluent aphasia (PFNA), and semantic dementia (SD). FTLD is characterized by atrophy of prefrontal and anterior temporal cortices. FTD occurs in 5-15% of patients with dementia and it is the third most common degenerative dementia. FTD occurs with equal frequency in both sexes. The age of onset is usually between 45 and 65 years though it may range anywhere from 21 to 81 years. The usual course is one of progressive clinicopathological deterioration with mortality within 6-8 years. Unlike Alzheimer's disease (AD), this condition has a strong genetic basis and family history of FTD is seen in 40-50% of cases. FTD is a genetically complex disorder inherited as an autosomal dominant trait with high penetrance in majority of cases. Genetic linkage studies have revealed FTLD loci on chromosome 3p, 9, 9p, and 17q. The most prevalent genes are PGRN (progranulin) and MAPT (microtubule-associated protein tau), both located on chromosome 17q21. More than 15 different pathologies can underlie FTD and related disorders and it has four major types of pathological features: (1) microvacuolation without neuronal inclusions, (2) microvacuolation with ubiquitinated rounded intraneuronal inclusions and dystrophic neurites FTLD-ubiquitinated (FTLD-U), (3) transcortical gliosis with tau-reactive rounded intraneuronal inclusions, (4) microvacuolation and taupositive neurofibrillary tangles. Behavior changes are the most common initial symptom of FTD (62%), whereas speech and language problems are most common in NFPA (100%) and SD (58%). There are no approved drugs for the management of FTD and trials are needed to find effective agents. Non-pharmacological treatment and caregiver

  9. Cholinergic imaging in dementia spectrum disorders.

    PubMed

    Roy, Roman; Niccolini, Flavia; Pagano, Gennaro; Politis, Marios

    2016-07-01

    The multifaceted nature of the pathology of dementia spectrum disorders has complicated their management and the development of effective treatments. This is despite the fact that they are far from uncommon, with Alzheimer's disease (AD) alone affecting 35 million people worldwide. The cholinergic system has been found to be crucially involved in cognitive function, with cholinergic dysfunction playing a pivotal role in the pathophysiology of dementia. The use of molecular imaging such as SPECT and PET for tagging targets within the cholinergic system has shown promise for elucidating key aspects of underlying pathology in dementia spectrum disorders, including AD or parkinsonian dementias. SPECT and PET studies using selective radioligands for cholinergic markers, such as [(11)C]MP4A and [(11)C]PMP PET for acetylcholinesterase (AChE), [(123)I]5IA SPECT for the α4β2 nicotinic acetylcholine receptor and [(123)I]IBVM SPECT for the vesicular acetylcholine transporter, have been developed in an attempt to clarify those aspects of the diseases that remain unclear. This has led to a variety of findings, such as cortical AChE being significantly reduced in Parkinson's disease (PD), PD with dementia (PDD) and AD, as well as correlating with certain aspects of cognitive function such as attention and working memory. Thalamic AChE is significantly reduced in progressive supranuclear palsy (PSP) and multiple system atrophy, whilst it is not affected in PD. Some of these findings have brought about suggestions for the improvement of clinical practice, such as the use of a thalamic/cortical AChE ratio to differentiate between PD and PSP, two diseases that could overlap in terms of initial clinical presentation. Here, we review the findings from molecular imaging studies that have investigated the role of the cholinergic system in dementia spectrum disorders. PMID:26984612

  10. Neuroimaging characteristics of dementia with Lewy bodies.

    PubMed

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

    2014-01-01

    This review summarises the findings and applications from neuroimaging studies in dementia with Lewy bodies (DLB), highlighting key differences between DLB and other subtypes of dementia. We also discuss the increasingly important role of imaging biomarkers in differential diagnosis and outline promising areas for future research in DLB. DLB shares common clinical, neuropsychological and pathological features with Parkinson's disease dementia and other dementia subtypes, such as Alzheimer's disease. Despite the development of consensus diagnostic criteria, the sensitivity for differential diagnosis of DLB in clinical practice remains low and many DLB patients will be misdiagnosed. The importance of developing accurate imaging markers in dementia is highlighted by the potential for treatments targeting specific molecular abnormalities as well as the responsiveness to cholinesterase inhibitors and marked neuroleptic sensitivity of DLB. We review various brain imaging techniques that have been applied to investigate DLB, including the characteristic nigrostriatal degeneration in DLB using positron emission tomography (PET) and single-photon emission computed tomography (SPECT) tracers. Dopamine transporter loss has proven to reliably differentiate DLB from other dementias and has been incorporated into the revised clinical diagnostic criteria for DLB. To date, this remains the 'gold standard' for diagnostic imaging of DLB. Regional cerebral blood flow, 18 F-fluorodeoxygluclose-PET and SPECT have also identified marked deficits in the occipital regions with relative sparing of the medial temporal lobe when compared to Alzheimer's disease. In addition, structural, diffusion, and functional magnetic resonance imaging techniques have shown alterations in structure, white matter integrity, and functional activity in DLB. We argue that the multimodal identification of DLB-specific biomarkers has the potential to improve ante-mortem diagnosis and contribute to our

  11. Dementia: Diagnosis and Tests

    MedlinePlus

    ... our e-newsletter! Aging & Health A to Z Dementia Diagnosis & Tests If you or someone you care ... To determine whether an older adult might have dementia, a healthcare professional will: Ask about the person’s ...

  12. Lewy Body Dementia Diagnosis

    MedlinePlus

    ... individuals, it may also be due to the natural course of the disease. All Rights Reserved Lewy Body Dementia Association, Inc. 912 Killian Hill Road S.W., Lilburn, GA 30047 © 2016 Lewy Body Dementia Association, Inc. Connect ...

  13. Precision Medicine: Clarity for the Complexity of Dementia.

    PubMed

    Cholerton, Brenna; Larson, Eric B; Quinn, Joseph F; Zabetian, Cyrus P; Mata, Ignacio F; Keene, C Dirk; Flanagan, Margaret; Crane, Paul K; Grabowski, Thomas J; Montine, Kathleen S; Montine, Thomas J

    2016-03-01

    Three key elements to precision medicine are stratification by risk, detection of pathophysiological processes as early as possible (even before clinical presentation), and alignment of mechanism of action of intervention(s) with an individual's molecular driver(s) of disease. Used for decades in the management of some rare diseases and now gaining broad currency in cancer care, a precision medicine approach is beginning to be adapted to cognitive impairment and dementia. This review focuses on the application of precision medicine to address the clinical and biological complexity of two common neurodegenerative causes of dementia: Alzheimer disease and Parkinson disease. PMID:26724389

  14. Dysphagia in stroke, neurodegenerative disease, and advanced dementia.

    PubMed

    Altman, Kenneth W; Richards, Amanda; Goldberg, Leanne; Frucht, Steven; McCabe, Daniel J

    2013-12-01

    Aspiration risk from dysphagia increases with central and peripheral neurologic disease. Stroke, microvascular ischemic disease, a spectrum of neurodegenerative diseases, and advancing dementia all have unique aspects. However, there are distinct commonalities in this population. Increasing nutritional requirements to stave off oropharyngeal muscular atrophy and a sedentary lifestyle further tax the patient's abilities to safely swallow. This article reviews stroke, muscular dystrophy, myasthenia gravis, multiple sclerosis, amyotrophic lateral sclerosis, Parkinson's disease, and advanced dementia. Approaches to screening and evaluation, recognizing sentinel indicators of decline that increase aspiration risk, and options for managing global laryngeal dysfunction are also presented. PMID:24262965

  15. [The Competence Network Parkinson (CNP)].

    PubMed

    Oertel, Wolfgang H; Deuschl, Guenther; Eggert, Karla

    2016-04-01

    The Competence Network Parkinson (CNP) is a research infrastructure for disease-oriented translational and clinical research in the field of Parkinson syndromes (PS). It was initiated in 1999 and funded until 2008 by the German Ministry for Education and Research (BMBF). The CNP created a highly frequented website with information on PS for the general public and for experts. The CNP designed and established one of the first electronic internet-based data entry systems (secuTrial®) - fulfilling the legal standards of data safety and security - a material bank for genetic research on Parkinson's disease (PD), implemented and investigated new methods for early diagnosis of PD and related atypical PS including in vivo dopamine transporter imaging (DAT SPECT), established the German Parkinson Study Group (GPS-Pharma) with 40 certified trial centres for pharmacotherapeutical trials and the German interdisciplinary Parkinson Study Group (neurology and neurosurgery) for deep brain stimulation (GPS-DBS), and carried out several pharmacoeconomic and health care studies on PD in Germany. Sustainability of the infrastructure CNP has in part been achieved in form of the GPS-Pharma and the GPS-DBS, as well as in the German Study Group on REM Sleep Behaviour Disorder (RBD), a prodromal phase of PD. Part of the CNP activities, such as genetic research and research on cohorts of PD patients, have been incorporated into the German Center for Neurodegenerative Disorders (DZNE). Furthermore, topics such as health care research are funded within projects of the EU research program. The article describes problems in setting up a competence network from scratch and contains recommendations how to avoid them in the future. PMID:26961981

  16. [Personality and Dementia].

    PubMed

    Masui, Yukie

    2016-07-01

    Previous studies have looked into the relationships between personality and dementia from three hypothetical points of views: 1) that personality type is a risk factor for dementia, 2) that personality changes occur before receiving a diagnosis of dementia, and 3) that premorbid personality traits define behavioral and psychological symptoms of dementia (BPSD) after receiving a diagnosis. This article overviews all three perspectives of the studies, after explaining the character and characteristic attributes of each perspective. PMID:27395463

  17. Wolff-Parkinson-White syndrome

    MedlinePlus

    ... normal. The condition may be diagnosed with an ECG or with continuous or person triggered ambulatory ECG monitoring, such as a Holter monitor . A test ... chap 64. Read More Cardiac ablation procedures Heart pacemaker Pulse Pulse - bounding Update Date 2/24/2016 ...

  18. The History of Parkinson's Disease: Early Clinical Descriptions and Neurological Therapies

    PubMed Central

    Goetz, Christopher G.

    2011-01-01

    Although components of possible Parkinson's disease can be found in very early documents, the first clear medical description was written in 1817 by James Parkinson. In the mid-1800s, Jean-Martin Charcot was particularly influential in refining and expanding this early description and in disseminating information internationally about Parkinson's disease. He separated Parkinson's disease from multiple sclerosis and other disorders characterized by tremor, and he recognized cases that later would likely be classified among the Parkinsonism-plus syndromes. Early treatments of Parkinson's disease were based on empirical observation, and anticholinergic drugs were used as early as the nineteenth century. The discovery of dopaminergic deficits in Parkinson's disease and the synthetic pathway of dopamine led to the first human trials of levodopa. Further historically important anatomical, biochemical, and physiological studies identified additional pharmacological and neurosurgical targets for Parkinson's disease and allow modern clinicians to offer an array of therapies aimed at improving function in this still incurable disease. PMID:22229124

  19. The history of Parkinson's disease: early clinical descriptions and neurological therapies.

    PubMed

    Goetz, Christopher G

    2011-09-01

    Although components of possible Parkinson's disease can be found in very early documents, the first clear medical description was written in 1817 by James Parkinson. In the mid-1800s, Jean-Martin Charcot was particularly influential in refining and expanding this early description and in disseminating information internationally about Parkinson's disease. He separated Parkinson's disease from multiple sclerosis and other disorders characterized by tremor, and he recognized cases that later would likely be classified among the Parkinsonism-plus syndromes. Early treatments of Parkinson's disease were based on empirical observation, and anticholinergic drugs were used as early as the nineteenth century. The discovery of dopaminergic deficits in Parkinson's disease and the synthetic pathway of dopamine led to the first human trials of levodopa. Further historically important anatomical, biochemical, and physiological studies identified additional pharmacological and neurosurgical targets for Parkinson's disease and allow modern clinicians to offer an array of therapies aimed at improving function in this still incurable disease. PMID:22229124

  20. Symptoms of Parkinson's

    MedlinePlus

    ... HelpLine Educational Publications Online Seminars Parkinson's News Educational Materials Do you need to know more about Parkinson's? PDF's materials provide information about symptoms, medications, resources & more. Order ...

  1. Managing Your Parkinson's Disease

    MedlinePlus

    ... Team Finding Resources Parkinson's HelpLine Learn More Educational Materials Do you want to know more about Parkinson's? PDF's materials provide information about symptoms, medications, resources & more. Order ...

  2. Parkinson disease - resources

    MedlinePlus

    Resources - Parkinson disease ... The following organizations are good resources for information on Parkinson disease : The Michael J. Fox Foundation -- www.michaeljfox.org National Institute of Neurological Disorders and Stroke -- www. ...

  3. Parkinson disease - discharge

    MedlinePlus

    Your doctor has told you that you have Parkinson disease . This disease affects the brain and leads ... have you take different medicines to treat your Parkinson disease and many of the problems that may ...

  4. Parkinson disease - discharge

    MedlinePlus

    Your doctor has told you that you have Parkinson disease . This disease affects the brain and leads to ... have you take different medicines to treat your Parkinson disease and many of the problems that may come ...

  5. [Metronome therapy in patients with Parkinson disease].

    PubMed

    Enzensberger, W; Oberländer, U; Stecker, K

    1997-12-01

    We studied 10 patients with Parkinson's disease and 12 patients with Parkinson-plus-syndrome, trying to improve patients' gait by application of various external rhythmic stimuli, including metronome stimulation (96 beats per minute = middle andante). The test course of the patients was 4 x 10 meters and 3 U-turns. The patients' gait quality under stimulation was compared with their free walk (velocity, number of steps, number of freezing episodes). Metronome stimulation significantly reduced the time and number of steps needed for the test course and also diminished the number of freezing episodes. March music stimulation was less effective and tactile stimulation (rhythmically tapping on the patient's shoulder) even produced negative results. The positive effect of metronome stimulation was also found, when the tests were not performed inside the hospital building, but outside in the hospital parc. Metronome stimulation was comparably effective in both patient sub-groups examined in this study (M. Parkinson, Parkinson-plus-syndrome) and seems to be an important additional help in the treatment of these patients. Electronical metronomes are not expensive, easy in handling, and portable. A theoretical explanation of metronome stimulation effectivity in patients with Parkinson's disease still needs to be elucidated. PMID:9465340

  6. Parkinsonism in FMR1 premutation carriers may be indistinguishable from Parkinson disease

    PubMed Central

    Hall, Deborah A.; Howard, Katherine; Hagerman, Randi; Leehey, Maureen A.

    2009-01-01

    Premutation carriers of repeat expansions in the fragile X mental retardation (FMR1) gene develop kinetic tremor and ataxia or the ‘fragile X associated tremor/ataxia syndrome’ (FXTAS). Affected FMR1 premutation carriers also have parkinsonism, but have not been reported to meet criteria for Parkinson disease. This case series illustrates that some patients who are FMR1 premutation carriers may appear by history and examination to have idiopathic Parkinson disease. Based on previous studies, it is likely that the genetic mutation and parkinsonism are associated. Although screening all PD patients is likely to be low yield, genetic testing of FMR1 in individuals with PD and a family history of fragile X syndrome, autism or developmental delay, or other related FMR1 phenotypes is warranted. PMID:18565783

  7. Manganese-Induced Parkinsonism and Parkinson's Disease: Shared and Distinguishable Features.

    PubMed

    Kwakye, Gunnar F; Paoliello, Monica M B; Mukhopadhyay, Somshuvra; Bowman, Aaron B; Aschner, Michael

    2015-07-01

    Manganese (Mn) is an essential trace element necessary for physiological processes that support development, growth and neuronal function. Secondary to elevated exposure or decreased excretion, Mn accumulates in the basal ganglia region of the brain and may cause a parkinsonian-like syndrome, referred to as manganism. The present review discusses the advances made in understanding the essentiality and neurotoxicity of Mn. We review occupational Mn-induced parkinsonism and the dynamic modes of Mn transport in biological systems, as well as the detection and pharmacokinetic modeling of Mn trafficking. In addition, we review some of the shared similarities, pathologic and clinical distinctions between Mn-induced parkinsonism and Parkinson's disease. Where possible, we review the influence of Mn toxicity on dopamine, gamma aminobutyric acid (GABA), and glutamate neurotransmitter levels and function. We conclude with a survey of the preventive and treatment strategies for manganism and idiopathic Parkinson's disease (PD). PMID:26154659

  8. Sudden Death: An Uncommon Occurrence in Dementia with Lewy Bodies.

    PubMed

    Molenaar, Joery P; Wilbers, Joyce; Aerts, Marjolein B; Leijten, Quinten H; van Dijk, Jan G; Esselink, Rianne A; Bloem, Bastiaan R

    2016-01-01

    We present a 75-year-old woman with dementia and parkinsonism who developed severe orthostatic hypotension and eventually died. Autopsy revealed extensive Lewy body formation in the midbrain, limbic system, intermediate spinal cord, and medulla oblongata. Furthermore, a vast amount of Lewy bodies was seen in the paravertebral sympathetic ganglia which likely explained the severe autonomic failure. We speculate that this autonomic failure caused sudden death through dysregulation of respiration or heart rhythm, reminiscent of sudden death in multiple system atrophy (MSA). Clinicians should be aware of this complication in patients presenting with parkinsonism and autonomic dysfunction, and that sudden death may occur in dementia with Lewy bodies (DLB) as it does in MSA. PMID:26891177

  9. Dementia Resulting From Traumatic Brain Injury

    PubMed Central

    Shively, Sharon; Scher, Ann I.; Perl, Daniel P.; Diaz-Arrastia, Ramon

    2013-01-01

    Traumatic brain injury (TBI) is among the earliest illnesses described in human history and remains a major source of morbidity and mortality in the modern era. It is estimated that 2% of the US population lives with long-term disabilities due to a prior TBI, and incidence and prevalence rates are even higher in developing countries. One of the most feared long-term consequences of TBIs is dementia, as multiple epidemiologic studies show that experiencing a TBI in early or midlife is associated with an increased risk of dementia in late life. The best data indicate that moderate and severe TBIs increase risk of dementia between 2-and 4-fold. It is less clear whether mild TBIs such as brief concussions result in increased dementia risk, in part because mild head injuries are often not well documented and retrospective studies have recall bias. However, it has been observed for many years that multiple mild TBIs as experienced by professional boxers are associated with a high risk of chronic traumatic encephalopathy (CTE), a type of dementia with distinctive clinical and pathologic features. The recent recognition that CTE is common in retired professional football and hockey players has rekindled interest in this condition, as has the recognition that military personnel also experience high rates of mild TBIs and may have a similar syndrome. It is presently unknown whether dementia in TBI survivors is pathophysiologically similar to Alzheimer disease, CTE, or some other entity. Such information is critical for developing preventive and treatment strategies for a common cause of acquired dementia. Herein, we will review the epidemiologic data linking TBI and dementia, existing clinical and pathologic data, and will identify areas where future research is needed. PMID:22776913

  10. The burden of dementia.

    PubMed

    Cotter, Valerie T

    2007-12-01

    Dementia care is a significant and growing healthcare need that will have major economic and medical impact as the prevalence of Alzheimer's disease (AD) and related dementias continues to increase in the United States during the next 50 years. The ability to differentiate the signs and symptoms of the most common dementing illnesses - AD, vascular dementia, frontotemporal dementia, and dementia with Lewy bodies - is essential to dementia care and management. Additionally, dementia patients in longterm care (LTC) facilities are prone to significantly greater risk of negative outcomes compared with nondemented residents as a result of a decline in activities of daily living, physical capacities, and behavioral manifestations. Careful and active assessment of risk factors and their management provides opportunities for improving outcomes. These include behavioral manifestations of pain, wandering, and risks of malnutrition, falls and injuries, and restraint use. Specific nonpharmacologic interventions to promote restraint- and pain-free care in LTC are highlighted. PMID:18095782

  11. Competency and consent in dementia.

    PubMed

    Fellows, L K

    1998-07-01

    Health care for demented older persons presents a range of ethical dilemmas. The disease process affects cognitive abilities, making competency a central issue. The syndrome of dementia carries a complex social overlay that colors perceptions of these patients and of their capacity for making decisions. An argument is made for a coherent, ethically based decision-making process that can be applied across the whole spectrum of dementia severity. The major ethical principles implicated in assessing a patient's ability to consent to treatment are reviewed. A sliding scale model of capacity is presented, in which the patient's ability to decide is weighed against the risk associated with the treatment decision in question. This model preserves the autonomy of the demented patient while minimizing the potential for harm. In situations where the patient is deemed incapable, two approaches that can be applied to making treatment decisions are contrasted. The 'prior competent choice' standard stresses the values that the patient held while competent. The 'best interests' standard moves the focus to the patient's subjective experience at the time the treatment is considered. The relative merits of these two concepts are evaluated in the context of dementia. Surveys of actual decision-making practice are contrasted with ethical and legal principles. The challenges inherent to applying the best interests standard are discussed. Despite the pitfalls, this standard offers an opportunity to restore the demented patient's sense of self. PMID:9670887

  12. International Rett Syndrome Foundation

    MedlinePlus

    ... your state! State Resources Rettsyndrome.org is the world's leading Rett syndrome research funding organization We have ... toward treatments for millions of people around the world with Autism, Parkinson's, Alzheimers, Schizophrenia and Traumatic Brain ...

  13. Dementia Coding, Workup, and Treatment in the VA New England Healthcare System

    PubMed Central

    Gagnon, David R.; Driver, Jane A.; Altincatal, Arman; Kosik, Nicole; Lanes, Stephan; Lawler, Elizabeth V.

    2014-01-01

    Growing evidence suggests that Alzheimer's disease and other types of dementia are underdiagnosed and poorly documented. In our study, we describe patterns of dementia coding and treatment in the Veteran's Administration New England Healthcare System. We conducted a retrospective cohort study with new outpatient ICD-9 codes for several types of dementia between 2002 and 2009. We examined healthcare utilization, medication use, initial dementia diagnoses, and changes in diagnoses over time by provider type. 8,999 veterans received new dementia diagnoses during the study period. Only 18.3% received a code for cognitive impairment other than dementia, most often “memory loss” (65.2%) prior to dementia diagnosis. Two-thirds of patients received their initial code from a PCP. The etiology of dementia was often never specified by ICD-9 code, even by specialists. Patients followed up exclusively by PCPs had lower rates of neuroimaging and were less likely to receive dementia medication. Emergency room visits and hospitalizations were frequent in all patients but highest in those seen by dementia specialists. Dementia medications are commonly used off-label. Our results suggest that, for the majority the patients, no prodrome of the dementia syndrome is documented with diagnostic code, and patients who do not see dementia specialists have less extensive diagnostic assessment and treatment. PMID:24701364

  14. Nuclear medicine imaging in dementia: a practical overview for hospitalists.

    PubMed

    Toney, Lauren Kay; McCue, Tim J; Minoshima, Satoshi; Lewis, David H

    2011-08-01

    Dementia is a clinical syndrome with diverse presentation, a challenging differential diagnosis, and time-sensitive therapy. The most common cause of dementia in patients aged > 65 years is Alzheimer's disease, which now affects 4 million people in the United States, but is often underrecognized, especially in the inpatient population. The hospitalist may have the opportunity to evaluate a patient's initial presentation of dementia. Addressing the inpatient's dementia symptoms can improve overall care and outcomes, so it is imperative that the hospitalist is abreast of recent developments in the dementia workup. The focus of this article is to overview how nuclear medicine imaging of the brain can aid in this process, with perfusion single-photon emission computed tomography (SPECT) and fludeoxyglucose F 18 ((18)F-FDG) positron emission tomography (PET) as the 2 most common modalities. Our discussion focuses on Alzheimer's disease, as this the most common etiology of dementia in patients aged > 65 years; however, we also touch on the other common neurodegenerative dementias (eg, dementia with Lewy bodies, vascular dementia, and frontotemporal dementia) for completeness. We begin with a summary of the most recent published guidelines for each of these neurodegenerative diseases, and then expand on the role that nuclear imaging plays in each. We provide a basic overview of the principles of these nuclear medicine techniques, and then illustrate findings in perfusion SPECT and (18)F-FDG PET for typical patterns of dementia, with emphasis on evidence regarding diagnostic accuracy of each modality, in comparison with accepted gold standards. Finally, we outline some future research topics within the field of nuclear medicine in dementia, including amyloid plaque imaging and dopamine transporter imaging. PMID:21881402

  15. Music and dementia.

    PubMed

    Baird, Amee; Samson, Séverine

    2015-01-01

    There is an increasing incidence of dementia in our aging population, and consequently an urgent need to develop treatments and activities that may alleviate the symptoms of dementia. Accumulating evidence shows that persons with dementia enjoy music, and their ability to respond to music is potentially preserved even in the late or severe stages of dementia when verbal communication may have ceased. Media interest in this topic has contributed to the public perception that music abilities are an "island of preservation" in an otherwise cognitively impaired person with dementia. In this chapter, we review the current literature on music cognition in dementia and show that there has been very scarce rigorous scientific investigation of this issue, and that various types of music memory exist and are differentially impaired in the different types of dementia. Furthermore, we discuss the recent development of music activities as a nonpharmacological treatment for dementia and highlight the methodological limitations of the current literature on this topic. While it has been reported that music activities can improve behavior, (particularly agitation), mood, and cognition in persons with dementia, recent large-scale randomized control studies have questioned the specificity of the effect of music and found that it is no more beneficial than other pleasant activities. Nevertheless, music is unique in its powerful ability to elicit both memories and emotions. This can provide an important link to individual's past and a means of nonverbal communication with carers, which make it an ideal stimulus for persons with dementia. PMID:25725917

  16. Clinico-Pathological Correlations of the Most Common Neurodegenerative Dementias

    PubMed Central

    Taipa, Ricardo; Pinho, João; Melo-Pires, Manuel

    2012-01-01

    Neurodegenerative dementias are a group of neurological disorders characterized by deterioration in several cognitive domains in which there is selective and progressive loss of specific populations of neurons. The precise neurobiological basis for the different neurodegenerative dementias remains unknown. It is expected that different pathologies reflect different mechanisms, at least early in the neurodegeneration process. The next decades promise treatments directed to causes and mechanisms, bringing an outstanding challenge to clinicians due to heterogeneous clinical presentations with the same molecular pathology. The purpose of this brief review is to describe the key neuropathological features of the most common neurodegenerative dementias (Alzheimer disease, dementia with Lewy bodies and Parkinson’s disease dementia, and frontotemporal lobar degeneration) and the relationship with the clinical syndromes described in clinico-pathological studies. We expect this overview contributes for the understanding of this broad topic integrating the two ends of the spectrum: clinical and pathological. PMID:22557993

  17. Exercise for Individuals with Lewy Body Dementia: A Systematic Review

    PubMed Central

    Inskip, Michael; Mavros, Yorgi; Sachdev, Perminder S.; Fiatarone Singh, Maria A.

    2016-01-01

    Background Individuals with Lewy body Dementia (LBD), which encompasses both Parkinson disease dementia (PDD) and Dementia with Lewy Bodies (DLB) experience functional decline through Parkinsonism and sedentariness exacerbated by motor, psychiatric and cognitive symptoms. Exercise may improve functional outcomes in Parkinson’s disease (PD), and Alzheimer’s disease (AD). However, the multi-domain nature of the LBD cluster of symptoms (physical, cognitive, psychiatric, autonomic) results in vulnerable individuals often being excluded from exercise studies evaluating physical function in PD or cognitive function in dementia to avoid confounding results. This review evaluated existing literature reporting the effects of exercise interventions or physical activity (PA) exposure on cluster symptoms in LBD. Methods A high-sensitivity search was executed across 19 databases. Full-length articles of any language and quality, published or unpublished, that analysed effects of isolated exercise/physical activity on indicative Dementia with Lewy Bodies or PD-dementia cohorts were evaluated for outcomes inclusive of physical, cognitive, psychiatric, physiological and quality of life measures. The protocol for this review (Reg. #: CRD42015019002) is accessible at http://www.crd.york.ac.uk/PROSPERO/. Results 111,485 articles were initially retrieved; 288 full articles were reviewed and 89.6% subsequently deemed ineligible due to exclusion of participants with co-existence of dementia and Parkinsonism. Five studies (1 uncontrolled trial, 1 randomized controlled trial and 3 case reports) evaluating 16 participants were included. Interventions were diverse and outcome homogeneity was low. Habitual gait speed outcomes were measured in 13 participants and increased (0.18m/s, 95% CI -0.02, 0.38m/s), exceeding moderate important change (0.14m/s) for PD cohorts. Other outcomes appeared to improve modestly in most participants. Discussion Scarce research investigating exercise in LBD

  18. [Neuroepigenetics: Desoxyribonucleic acid methylation in Alzheimer's disease and other dementias].

    PubMed

    Mendioroz Iriarte, Maite; Pulido Fontes, Laura; Méndez-López, Iván

    2015-05-21

    DNA methylation is an epigenetic mechanism that controls gene expression. In Alzheimer's disease (AD), global DNA hypomethylation of neurons has been described in the human cerebral cortex. Moreover, several variants in the methylation pattern of candidate genes have been identified in brain tissue when comparing AD patients and controls. Specifically, DNA methylation changes have been observed in PSEN1 and APOE, both genes previously being involved in the pathophysiology of AD. In other degenerative dementias, methylation variants have also been described in key genes, such as hypomethylation of the SNCA gene in Parkinson's disease and dementia with Lewy bodies or hypermethylation of the GRN gene promoter in frontotemporal dementia. The finding of aberrant DNA methylation patterns shared by brain tissue and peripheral blood opens the door to use those variants as epigenetic biomarkers in the diagnosis of neurodegenerative diseases. PMID:24907105

  19. Dementia with Lewy bodies: disease concept and genetics.

    PubMed

    Graeber, Manuel B; Müller, Ulrich

    2003-08-01

    Dementia with Lewy bodies (DLB) was first recognized as a clinicopathological entity about 20 years ago. It is the second most-common degenerative dementia after Alzheimer's disease. Clinically, DLB differs from Alzheimer's disease in that disease symptoms are prone to fluctuate and patients often suffer from visual hallucinations, while short-term memory is relatively preserved. As many as 70% of patients have parkinsonism and up to 50% are sensitive to the extrapyramidal side effects of neuroleptic drugs. About 3 million Europeans will be affected by DLB in 2020 if no cure or effective treatment is found. This article reviews the current disease concept, as well as existing problems concerning classification and delineation of DLB from other conditions with dementia. The literature on genetic findings in this complex disease is critically discussed. PMID:12898286

  20. Imaging neuroinflammation in Alzheimer's disease and other dementias: Recent advances and future directions.

    PubMed

    Varley, James; Brooks, David J; Edison, Paul

    2015-09-01

    Alzheimer's disease (AD), dementia with Lewy bodies, frontotemporal dementia (FTD), and Huntington's disease (HD) are the main neurodegenerative causes of dementia. Causes and mechanisms of these diseases remain elusive. Neuroinflammation is increasingly emerging as an important pathological factor in their development. Positron emission tomography (PET) using [11C]PK11195 represents a method of visualizing the microglial component of neuroinflammation via the translocator protein (TSPO) and we discuss the valuable insights this has yielded in neurodegenerative diseases. We discuss the limitations of this method and the development of second generation TSPO PET ligands which hope to overcome these limitations. We also discuss other methods of visualizing neuroinflammation and review the state of current dementia treatments targeted at neuroinflammation. It is our view that a multimodal investigation into neuroinflammation in AD, Parkinson's disease dementia, FTD and HD will yield valuable pathological insights which will usefully inform development of therapeutic targets and biomarkers. PMID:25449529

  1. Mutations in presenilin 2 and its implications in Alzheimer's disease and other dementia-associated disorders.

    PubMed

    Cai, Yan; An, Seong Soo A; Kim, SangYun

    2015-01-01

    Alzheimer's disease (AD) is the most common form of dementia. Mutations in the genes encoding presenilin 1 (PSEN1), presenilin 2 (PSEN2), and amyloid precursor protein have been identified as the main genetic causes of familial AD. To date, more than 200 mutations have been described worldwide in PSEN1, which is highly homologous with PSEN2, while mutations in PSEN2 have been rarely reported. We performed a systematic review of studies describing the mutations identified in PSEN2. Most PSEN2 mutations were detected in European and in African populations. Only two were found in Korean populations. Interestingly, PSEN2 mutations appeared not only in AD patients but also in patients with other disorders, including frontotemporal dementia, dementia with Lewy bodies, breast cancer, dilated cardiomyopathy, and Parkinson's disease with dementia. Here, we have summarized the PSEN2 mutations and the potential implications of these mutations in dementia-associated disorders. PMID:26203236

  2. [Carotid atherosclerosis and dementia].

    PubMed

    Harlé, Louise-Marine; Plichart, Matthieu

    2015-09-01

    Over the past decade a growing interest has been devoted to exploring the role of atherosclerosis in the development of dementia. Despite a well-known association between atherosclerosis risk factors in middle-life with later cognitive decline, the pathophysiological pathways underlying this association remain unclear. The current hypothesis is that neurodegenerative and vascular lesions coexist and have a synergistic role in the development of cognitive impairment and dementia. Carotid atherosclerosis (e.g. carotid plaques and intima-media thickness as measured by carotid ultrasonography) has been associated with cognitive decline and dementia and may help to better understand the complex interaction between the vascular and neurodegenerative processes. Furthermore, carotid atherosclerosis has been used in the recent field for dementia risk prediction. In this review, we discuss the physiopathological implications from the current available data on the relationship between carotid atherosclerosis and dementia as well as the interest of carotid biomarkers for individual dementia risk prediction. PMID:26395304

  3. Autosomal dominant Parkinson's disease caused by SNCA duplications.

    PubMed

    Konno, Takuya; Ross, Owen A; Puschmann, Andreas; Dickson, Dennis W; Wszolek, Zbigniew K

    2016-01-01

    The discovery in 1997 that mutations in the SNCA gene cause Parkinson's disease (PD) greatly advanced our understanding of this illness. There are pathogenic missense mutations and multiplication mutations in SNCA. Thus, not only a mutant protein, but also an increased dose of wild-type protein can produce autosomal dominant parkinsonism. We review the literature on SNCA duplications and focus on pathologically-confirmed cases. We also report a newly-identified American family with SNCA duplication whose proband was autopsied. We found that over half of the reported cases with SNCA duplication had early-onset parkinsonism and non-motor features, such as dysautonomia, rapid eye movement sleep behavior disorder (RBD), hallucinations (usually visual) and cognitive deficits leading to dementia. Only a few cases have presented with typical features of PD. Our case presented with depression and RBD that preceded parkinsonism, and dysautonomia that led to an initial diagnosis of multiple system atrophy. Dementia and visual hallucinations followed. Our patient and the other reported cases with SNCA duplications had widespread cortical Lewy pathology. Neuronal loss in the hippocampal cornu ammonis 2/3 regions were seen in about half of the autopsied SNCA duplication cases. Similar pathology was also observed in SNCA missense mutation and triplication carriers. PMID:26350119

  4. Dementia paralytica: deterioration from communicating hydrocephalus.

    PubMed Central

    Giménez-Roldán, S; Benito, C; Martin, M

    1979-01-01

    Five patients suffering from dementia paralytica who failed to improve or deteriorated after one or several high dosage courses of penicillin, had pneumoencephalographic patterns suggesting communicating hydrocephalus. Measurements of the ventricular index, ratio of cella media to width of the temporal horn, and the callosal angle differed from that in seven cases of dementia paralytica with associated cerebral atrophy. An isotope cisternogram in three cases with communicating hydrocephalus further confirmed a blockage of the cerebrospinal fluid (CSF) at the parasagittal subarachnoid space. Three patients exhibited the full syndrome of gait apraxia, incontinence, and pyramidal tract signs associated with a severe degree of dementia. Shunting of the CSF in three cases was followed by immediate improvement in two, one in a longlasting way. No active parenchymal inflammation was observed in any of three brain biopsy samples taken during surgery, except for leptomeningeal fibrosis in one. Chronic leptomeningitis in dementia paralytica may impair subarachnoid CSF absorption with subsequent communicating hydrocephalus. Progression or inadequate responses after therapeutic dose of penicillin in dementia paralytica should prompt investigation for this complication as an alternative, effective treatment could be offered. Images PMID:469557

  5. Trends in the Incidence of Parkinson Disease in the General Population: The Rotterdam Study.

    PubMed

    Darweesh, Sirwan K L; Koudstaal, Peter J; Stricker, Bruno H; Hofman, Albert; Ikram, M Arfan

    2016-06-01

    We investigated trends in the incidence of parkinsonism and Parkinson disease (PD) by comparing data from the first 2 subcohorts of the Rotterdam Study, a prospective, population-based cohort study (first subcohort: baseline 1990 with 10 years of follow-up; second subcohort, baseline 2000 with 10 years of follow-up). From the baseline years, we observed differences in the second subcohort that were associated with a lower risk of PD for some but not all baseline risk factors. Participants in both subcohorts were followed for a maximum of 10 years and monitored for the onset of parkinsonism, the onset of dementia, or death, until January 1, 2011. We used Poisson regression models to compare the incidences of parkinsonism, both overall and by cause (PD and secondary causes), and competitive events (incident dementia and death) as well as the mortality of parkinsonism patients in the 2 subcohorts. In the 1990 subcohort, there were 182 cases of parkinsonism (84 of which were PD) during 57,052 person-years. In the 2000 subcohort, we observed 28 cases of parkinsonism (10 with PD) during 22,307 person-years. The overall age- and sex-adjusted incidence of parkinsonism was lower in the 2000 subcohort (incidence rate ratio = 0.55, 95% confidence interval: 0.36, 0.81), and PD incidence declined sharply (incidence rate ratio = 0.39, 95% confidence interval: 0.19, 0.72). Competitive event rates were lower in the 2000 subcohort, and mortality rates among persons with parkinsonism remained stable. These findings suggest that the incidence of parkinsonism in general, and of PD in particular, decreased between 1990 and 2011. PMID:27188952

  6. Neurochemical Profile of Dementia Pugilistica

    PubMed Central

    Kokjohn, Tyler A.; Maarouf, Chera L.; Daugs, Ian D.; Hunter, Jesse M.; Whiteside, Charisse M.; Malek-Ahmadi, Michael; Rodriguez, Emma; Kalback, Walter; Jacobson, Sandra A.; Sabbagh, Marwan N.; Beach, Thomas G.

    2013-01-01

    Abstract Dementia pugilistica (DP), a suite of neuropathological and cognitive function declines after chronic traumatic brain injury (TBI), is present in approximately 20% of retired boxers. Epidemiological studies indicate TBI is a risk factor for neurodegenerative disorders including Alzheimer disease (AD) and Parkinson disease (PD). Some biochemical alterations observed in AD and PD may be recapitulated in DP and other TBI persons. In this report, we investigate long-term biochemical changes in the brains of former boxers with neuropathologically confirmed DP. Our experiments revealed biochemical and cellular alterations in DP that are complementary to and extend information already provided by histological methods. ELISA and one-dimensional and two dimensional Western blot techniques revealed differential expression of select molecules between three patients with DP and three age-matched non-demented control (NDC) persons without a history of TBI. Structural changes such as disturbances in the expression and processing of glial fibrillary acidic protein, tau, and α-synuclein were evident. The levels of the Aβ–degrading enzyme neprilysin were reduced in the patients with DP. Amyloid-β levels were elevated in the DP participant with the concomitant diagnosis of AD. In addition, the levels of brain-derived neurotrophic factor and the axonal transport proteins kinesin and dynein were substantially decreased in DP relative to NDC participants. Traumatic brain injury is a risk factor for dementia development, and our findings are consistent with permanent structural and functional damage in the cerebral cortex and white matter of boxers. Understanding the precise threshold of damage needed for the induction of pathology in DP and TBI is vital. PMID:23268705

  7. Neurochemical profile of dementia pugilistica.

    PubMed

    Kokjohn, Tyler A; Maarouf, Chera L; Daugs, Ian D; Hunter, Jesse M; Whiteside, Charisse M; Malek-Ahmadi, Michael; Rodriguez, Emma; Kalback, Walter; Jacobson, Sandra A; Sabbagh, Marwan N; Beach, Thomas G; Roher, Alex E

    2013-06-01

    Dementia pugilistica (DP), a suite of neuropathological and cognitive function declines after chronic traumatic brain injury (TBI), is present in approximately 20% of retired boxers. Epidemiological studies indicate TBI is a risk factor for neurodegenerative disorders including Alzheimer disease (AD) and Parkinson disease (PD). Some biochemical alterations observed in AD and PD may be recapitulated in DP and other TBI persons. In this report, we investigate long-term biochemical changes in the brains of former boxers with neuropathologically confirmed DP. Our experiments revealed biochemical and cellular alterations in DP that are complementary to and extend information already provided by histological methods. ELISA and one-dimensional and two dimensional Western blot techniques revealed differential expression of select molecules between three patients with DP and three age-matched non-demented control (NDC) persons without a history of TBI. Structural changes such as disturbances in the expression and processing of glial fibrillary acidic protein, tau, and α-synuclein were evident. The levels of the Aβ-degrading enzyme neprilysin were reduced in the patients with DP. Amyloid-β levels were elevated in the DP participant with the concomitant diagnosis of AD. In addition, the levels of brain-derived neurotrophic factor and the axonal transport proteins kinesin and dynein were substantially decreased in DP relative to NDC participants. Traumatic brain injury is a risk factor for dementia development, and our findings are consistent with permanent structural and functional damage in the cerebral cortex and white matter of boxers. Understanding the precise threshold of damage needed for the induction of pathology in DP and TBI is vital. PMID:23268705

  8. Immunologically Mediated Dementias

    PubMed Central

    Rosenbloom, Michael H.; Smith, Sallie; Akdal, Gulden; Geschwind, Michael D.

    2009-01-01

    Although most dementias are due to neurodegenerative or vascular disease, it is important to diagnose immunologically mediated dementias quickly because they can be both rapidly progressive and readily treatable. They usually affect function of limbic and cortical structures, but subcortical involvement can also occur. Because of the variety of symptoms and the rapid course, these dementias present a particular challenge to the clinician and may require evaluation and intervention in the inpatient setting. Diagnostic workup typically reveals evidence of an autoimmune process and, in some cases, cancer. In contrast to the neurodegenerative processes, many of the immunologically mediated dementias respond to immunomodulatory therapy. PMID:19664365

  9. Sexual disinhibition and dementia.

    PubMed

    Cipriani, Gabriele; Ulivi, Martina; Danti, Sabrina; Lucetti, Claudio; Nuti, Angelo

    2016-03-01

    To describe inappropriate sexual behaviour (ISB) observed in patients with dementia, we conducted searches using the Cochrane Library, PubMed, and Web of Science to find relevant articles, chapters, and books published from 1950 to 2014. Search terms used included 'hypersexuality', 'inappropriate sexual behaviors', and 'dementia'. Publications found through this indexed search were reviewed for further relevant references. Sexuality is a human's need to express intimacy, but persons with dementia may not know how to appropriately meet their needs for closeness and intimacy due to their decline in cognition. Generally, the interaction among brain, physical, psychological, and environmental factors can create what we call ISB. The most likely change in the sexual behaviour of a person with dementia is indifference. However, ISB in dementia appear to be of two types--intimacy-seeking and disinhibited--that differ in their association with dementia type, dementia severity and, possibly, other concurrent behavioural disorder. Tensions develop from uncertainties regarding which, or when, behaviours are to be considered 'inappropriate' (i.e. improper) or abnormal. While most ISB occur in the moderate to severe stages of Alzheimer's dementia, they may also be seen in early stages of frontotemporal dementia because of the lack of insight and disinhibition. ISB are often better managed by non-pharmacological means, as patients may be less responsive to psychoactive therapies, but non-pharmacological interventions do not always stop the behaviour. PMID:26215977

  10. Peripheral neuropathy and parkinsonism: a large clinical and pathogenic spectrum.

    PubMed

    Vital, Anne; Lepreux, Sebastien; Vital, Claude

    2014-12-01

    Peripheral neuropathy (PN) has been reported in idiopathic and hereditary forms of parkinsonism, but the pathogenic mechanisms are unclear and likely heterogeneous. Levodopa-induced vitamin B12 deficiency has been discussed as a causal factor of PN in idiopathic Parkinson's disease, but peripheral nervous system involvement might also be a consequence of the underlying neurodegenerative process. Occurrence of PN with parkinsonism has been associated with a panel of mitochondrial cytopathies, more frequently related to a nuclear gene defect and mainly polymerase gamma (POLG1) gene. Parkin (PARK2) gene mutations are responsible for juvenile parkinsonism, and possible peripheral nervous system involvement has been reported. Rarely, an association of parkinsonism with PN may be encountered in other neurodegenerative diseases such as fragile X-associated tremor and ataxia syndrome related to premutation CGG repeat expansion in the fragile X mental retardation (FMR1) gene, Machado-Joseph disease related to an abnormal CAG repeat expansion in ataxin-3 (ATXN3) gene, Kufor-Rakeb syndrome caused by mutations in ATP13A2 gene, or in hereditary systemic disorders such as Gaucher disease due to mutations in the β-glucocerebrosidase (GBA) gene and Chediak-Higashi syndrome due to LYST gene mutations. This article reviews conditions in which PN may coexist with parkinsonism. PMID:25582874

  11. Welding and parkinsonism.

    PubMed

    Furbee, Brent

    2011-08-01

    Manganese-induced parkinsonism has been recognized since 1837. It has been reported primarily in miners, grinders, and smelters since that time. More recently, isolated case reports involving welders have appeared in the medical literature. Manganism can be distinguished from other forms of parkinsonism by clinical presentation with support from laboratory and radiologic findings. The controversy regarding the risk of parkinsonism in welders is reviewed. PMID:21803214

  12. TREM2 p.R47H substitution is not associated with dementia with Lewy bodies.

    PubMed

    Walton, Ronald L; Soto-Ortolaza, Alexandra I; Murray, Melissa E; Lorenzo-Betancor, Oswaldo; Ogaki, Kotaro; Heckman, Michael G; Rayaprolu, Sruti; Rademakers, Rosa; Ertekin-Taner, Nilüfer; Uitti, Ryan J; van Gerpen, Jay A; Wszolek, Zbigniew K; Smith, Glenn E; Kantarci, Kejal; Lowe, Val J; Parisi, Joseph E; Jones, David T; Savica, Rodolfo; Graff-Radford, Jonathan; Knopman, David S; Petersen, Ronald C; Graff-Radford, Neill R; Ferman, Tanis J; Dickson, Dennis W; Boeve, Bradley F; Ross, Owen A; Labbé, Catherine

    2016-08-01

    Dementia with Lewy bodies (DLB) is the second leading cause of neurodegenerative dementia in the elderly and is clinically characterized by the presence of cognitive decline, parkinsonism, REM sleep behavior disorder, and visual hallucinations.(1,2) At autopsy, α-synuclein-positive Lewy-related pathology is observed throughout the brain. Concomitant Alzheimer disease-related pathology including amyloid plaques and, to a lesser degree, neurofibrillary tangles are often present.(2) The clinical characteristics of DLB share overlapping features with Alzheimer disease dementia (AD) and Parkinson disease (PD). A recent genetic association study examining known hits from PD and AD identified variants at both the α-synuclein (SNCA) and APOE loci as influencing the individual risk to DLB.(3) These findings would suggest that DLB may be a distinct disease with shared genetic risk factors with PD and AD. PMID:27458607

  13. Parkinson's disease with camptocormia

    PubMed Central

    Bloch, F; Houeto, J L; du Montcel, S Tezenas; Bonneville, F; Etchepare, F; Welter, M L; Rivaud‐Pechoux, S; Hahn‐Barma, V; Maisonobe, T; Behar, C; Lazennec, J Y; Kurys, E; Arnulf, I; Bonnet, A M; Agid, Y

    2006-01-01

    Background Camptocormia is defined as an abnormal flexion of the trunk that appears when standing or walking and disappears in the supine position. The origin of the disorder is unknown, but it is usually attributed either to a primary or a secondary paravertebral muscle myopathy or a motor neurone disorder. Camptocormia is also observed in a minority of patients with parkinsonism. Objective To characterise the clinical and electrophysiological features of camptocormia and parkinsonian symptoms in patients with Parkinson's disease and camptocormia compared with patients with Parkinson's disease without camptocormia. Methods Patients with parkinsonism and camptocormia (excluding patients with multiple system atrophy) prospectively underwent a multidisciplinary clinical (neurological, neuropsychological, psychological, rheumatological) and neurophysiological (electromyogram, ocular movement recording) examination and were compared with age‐matched patients with Parkinson's disease without camptocormia. Results The camptocormia developed after 8.5 (SD 5.3) years of parkinsonism, responded poorly to levodopa treatment (20%) and displayed features consistent with axial dystonia. Patients with camptocormia were characterised by prominent levodopa‐unresponsive axial symptoms (ie, axial rigidity, gait disorder and postural instability), along with a tendency for greater error in the antisaccade paradigm. Conclusion We suggest that (1) the salient features of parkinsonism observed in patients with camptocormia are likely to represent a specific form of Parkinson's disease and camptocormia is an axial dystonia and (2) both camptocormia and parkinsonism in these patients might result from additional, non‐dopaminergic neuronal dysfunction in the basal ganglia. PMID:16754693

  14. Clinical subtypes of chronic traumatic encephalopathy: literature review and proposed research diagnostic criteria for traumatic encephalopathy syndrome

    PubMed Central

    2014-01-01

    The long-term consequences of repetitive head impacts have been described since the early 20th century. Terms such as punch drunk and dementia pugilistica were first used to describe the clinical syndromes experienced by boxers. A more generic designation, chronic traumatic encephalopathy (CTE), has been employed since the mid-1900s and has been used in recent years to describe a neurodegenerative disease found not just in boxers but in American football players, other contact sport athletes, military veterans, and others with histories of repetitive brain trauma, including concussions and subconcussive trauma. This article reviews the literature of the clinical manifestations of CTE from 202 published cases. The clinical features include impairments in mood (for example, depression and hopelessness), behavior (for example, explosivity and violence), cognition (for example, impaired memory, executive functioning, attention, and dementia), and, less commonly, motor functioning (for example, parkinsonism, ataxia, and dysarthria). We present proposed research criteria for traumatic encephalopathy syndrome (TES) which consist of four variants or subtypes (TES behavioral/mood variant, TES cognitive variant, TES mixed variant, and TES dementia) as well as classifications of ‘probable CTE’ and ‘possible CTE’. These proposed criteria are expected to be modified and updated as new research findings become available. They are not meant to be used for a clinical diagnosis. Rather, they should be viewed as research criteria that can be employed in studies of the underlying causes, risk factors, differential diagnosis, prevention, and treatment of CTE and related disorders. PMID:25580160

  15. Tauopathies with parkinsonism: clinical spectrum, neuropathologic basis, biological markers, and treatment options

    PubMed Central

    Ludolph, A. C.; Kassubek, J.; Landwehrmeyer, B. G.; Mandelkow, E.; Mandelkow, E.-M.; Burn, D. J.; Caparros-Lefebvre, D.; Frey, K. A.; de Yebenes, J. G.; Gasser, T.; Heutink, P.; Höglinger, G.; Jamrozik, Z.; Jellinger, K. A.; Kazantsev, A.; Kretzschmar, H.; Lang, A. E.; Litvan, I.; Lucas, J. J.; McGeer, P. L.; Melquist, S.; Oertel, W.; Otto, M.; Paviour, D.; Reum, T.; Saint-Raymond, A.; Steele, J. C.; Tolnay, M.; Tumani, H.; van Swieten, J. C.; Vanier, M. T.; Vonsattel, J.-P.; Wagner, S.; Wszolek, Z. K.

    2009-01-01

    Tauopathies with parkinsonism represent a spectrum of disease entities unified by the pathologic accumulation of hyperphosphorylated tau protein fragments within the central nervous system. These pathologic characteristics suggest shared pathogenetic pathways and possible molecular targets for disease-modifying therapeutic interventions. Natural history studies, for instance, in progressive supranuclear palsy, frontotemporal dementia with parkinsonism linked to chromosome 17, corticobasal degeneration, and Niemann-Pick disease type C as well as in amyotrophic lateral sclerosis/Parkinson–dementia complex permit clinical characterization of the disease phenotypes and are crucial to the development and validation of biological markers for differential diagnostics and disease monitoring, for example, by use of neuroimaging or proteomic approaches. The wide pathologic and clinical spectrum of the tauopathies with parkinsonism is reviewed in this article, and perspectives on future advances in the understanding of the pathogenesis are given, together with potential therapeutic strategies. PMID:19364361

  16. [Speech changes in dementia].

    PubMed

    Benke, T; Andree, B; Hittmair, M; Gerstenbrand, F

    1990-06-01

    This review analyzes the spectrum of language deficits commonly encountered in dementia. A specific communication profile is found in dementia of the "cortical" type, such as Alzheimer's disease. With advancing disease lexical, comprehension and pragmatic functions deteriorate, whereas syntax and phonology tend to be preserved. This pattern bears some resemblance to aphasia types like transcortical and Wernicke's aphasia, however, a much broader range of communicative functions is impaired in Alzheimer's disease than in aphasia. Differentiation of dementia and aphasia, especially in elderly patients requires careful neuropsychological assessment of language, memory and other psychological functions. "Subcortical" dementia commonly presents with dysarthria as the leading symptom and linguistic impairment is rarely of crucial importance until late stages. Thus, the interetiologic dissociation of language and speech impairment can be used for dementia differentiation. Aphasia batteries are not sufficient to comprehend the range of language deficits in demented patients. Testing the communication impairment in dementia requires specific tasks for spontaneous speech, naming, comprehension, reading, writing, repetition and motor speech functions. Tasks for verbal learning and metalinguistic abilities should also be performed. Language deficits are frequent initial symptoms of dementia, thus language assessment may be of diagnostic relevance. Many data support the concept that the communication deficit in dementia results from a particular impairment of semantic memory. PMID:1695887

  17. Dealing with Dementia

    MedlinePlus

    ... an NIH-supported Alzheimer’s disease center at the University of Wisconsin. “Symptoms of dementia can include problems with memory, thinking, and language, along with impairments to social skills and some behavioral symptoms.” Several factors can raise your risk for developing dementia. These ...

  18. Psychiatric aspects of Parkinson's disease--an update.

    PubMed

    Schrag, Anette

    2004-07-01

    In patients with Parkinson's disease (PD) disturbances of mental state constitute some of the most difficult treatment challenges of advanced disease, often limiting effective treatment of motor symptoms and leading to increased disability and poor quality of life. This article provides an update on the current knowledge of these complications and the use of old and new drugs in their management. Mental state alterations in PD include depression, anxiety, cognitive impairment, apathy, and treatment-related psychiatric symptoms. The latter range from vivid dreams and hallucinations to delusions, manic symptoms, hypersexuality, dopamine dysregulation syndrome and delirium. While some of these symptoms may be alleviated by anti-parkinsonian medication, especially if they are off-period related, treatment-related phenomena are usually exacerbated by increasing the number or dosage of antiparkinsonian drugs. Elimination of exacerbating factors and simplification of drug regimes are the first and most important steps in improvement of such symptoms. However, the advent of atypical antipsychotics such as clozapine has dramatically helped the management of treatment-related psychiatric complications in PD. In patients with dementia associated with PD cognitive functioning and behavioural problems appear to respond to cholinesterase inhibitors, such as rivastigmine or donepezil. Depression is a common problem in early as well as advanced PD, and selective serotonin reuptake inhibitors, reboxetine, and tricyclic antidepressants have been reported to be effective and well tolerated antidepressants. Randomised, controlled studies are required to assess the differential efficacy and tolerability of antidepressants in patients with PD, including the newer antidepressants with serotonergic and noradrenergic properties. PMID:15258780

  19. Ageing and Parkinson's disease: substantia nigra regional selectivity.

    PubMed

    Fearnley, J M; Lees, A J

    1991-10-01

    The micro-architecture of the substantia nigra was studied in control cases of varying age and patients with parkinsonism. A single 7 mu section stained with haematoxylin and eosin was examined at a specific level within the caudal nigra using strict criteria. The pars compacta was divided into a ventral and a dorsal tier, and each tier was further subdivided into 3 regions. In 36 control cases there was a linear fallout of pigmented neurons with advancing age in the pars compacta of the caudal substantia nigra at a rate of 4.7% per decade. Regionally, the lateral ventral tier was relatively spared (2.1% loss per decade) compared with the medial ventral tier (5.4%) and the dorsal tier (6.9%). In 20 Parkinson's disease (PD) cases of varying disease duration there was an exponential loss of pigmented neurons with a 45% loss in the first decade. Regionally, the pattern was opposite to ageing. Loss was greatest in the lateral ventral tier (average loss 91%) followed by the medial ventral tier (71%) and the dorsal tier (56%). The presymptomatic phase of PD from the onset of neuronal loss was estimated to be about 5 yrs. This phase is represented by incidental Lewy body cases: individuals who die without clinical signs of PD or dementia, but who are found to have Lewy bodies at post-mortem. In 7 cases cell loss was confined to the lateral ventral tier (average loss 52%) congruent with the lateral ventral selectivity of symptomatic PD. It was calculated that at the onset of symptoms there was a 68% cell loss in the lateral ventral tier and a 48% loss in the caudal nigra as a whole. The regional selectivity of PD is relatively specific. In 15 cases of striatonigral degeneration the distribution of cell loss was similar, but the loss in the dorsal tier was greater than PD by 21%. In 14 cases of Steele-Richardson-Olszewski syndrome (SRO) there was no predilection for the lateral ventral tier, but a tendency to involve the medial nigra and spare the lateral. These findings

  20. Living With Semantic Dementia

    PubMed Central

    Sage, Karen; Wilkinson, Ray; Keady, John

    2014-01-01

    Semantic dementia is a variant of frontotemporal dementia and is a recently recognized diagnostic condition. There has been some research quantitatively examining care partner stress and burden in frontotemporal dementia. There are, however, few studies exploring the subjective experiences of family members caring for those with frontotemporal dementia. Increased knowledge of such experiences would allow service providers to tailor intervention, support, and information better. We used a case study design, with thematic narrative analysis applied to interview data, to describe the experiences of a wife and son caring for a husband/father with semantic dementia. Using this approach, we identified four themes: (a) living with routines, (b) policing and protecting, (c) making connections, and (d) being adaptive and flexible. Each of these themes were shared and extended, with the importance of routines in everyday life highlighted. The implications for policy, practice, and research are discussed. PMID:24532121

  1. Depression, Dementia, and Social Supports.

    ERIC Educational Resources Information Center

    Esser, Sally R.; Vitaliano, Peter P.

    1988-01-01

    Reviews recent literature on the relationships among dementia, depression, and social support, emphasizing the diagnostic differentiation of dementia and depression, and the role of these three entities in elderly with cognitive impairment. Discusses dementia-like symptoms arising in depression and the coexistence of dementia and depression.…

  2. Dementia With Lewy Bodies: Diagnosis and Management for Primary Care Providers

    PubMed Central

    Mahajan, Aman; Handa, Kamna

    2011-01-01

    Objective: The purpose of this review is to aid primary care providers in distinguishing dementia with Lewy bodies (DLB) from Alzheimer's disease and from Parkinson's disease with dementia. Differentiating these entities has important treatment implications. Data Sources: A PubMed search was undertaken using the keywords Lewy body dementia, dementia with Lewy bodies, and Lewy body disease. There were no date restrictions. Only articles in the English language were reviewed. References of selected articles were reviewed for additional sources. Data Selection and Extraction: Initially, 2,967 articles were retrieved. All 3 authors participated in data selection and extraction. Articles were further selected for content specific to epidemiology, clinical presentation, diagnostic studies, treatment, and prognosis. For articles with repetitive information, the most current article was used. This resulted in a total of 62 articles included in the review. Data Synthesis: Dementia with Lewy bodies is the second leading cause of dementia after Alzheimer's disease. The core symptoms of DLB, including cognitive fluctuations, visual hallucinations, and parkinsonism, may not always be present as a triad, and clinicians may be unaware of associated symptoms. Thus, this diagnosis is frequently missed by primary care providers. Often, DLB is misdiagnosed as Alzheimer's disease, Parkinson's disease, or a primary psychiatric illness. Treatments for DLB include cholinesterase inhibitors and N-methyl-D-aspartate antagonists. Antipsychotics should be avoided or used with caution. Conclusions: Dementia with Lewy bodies is an often missed diagnosis. Symptoms are often attributed to other disorders. A high clinical suspicion is helpful in accurate diagnosis, and presence of any of the core symptoms should initiate clinical suspicion of DLB. Distinguishing DLB from other disorders has important treatment implications. PMID:22295275

  3. Prescribing patterns in dementia: a multicentre observational study in a German network of CAM physicians

    PubMed Central

    2011-01-01

    Background Dementia is a major and increasing health problem worldwide. This study aims to investigate dementia treatment strategies among physicians specialised in complementary and alternative medicine (CAM) by analysing prescribing patterns and comparing them to current treatment guidelines in Germany. Methods Twenty-two primary care physicians in Germany participated in this prospective, multicentre observational study. Prescriptions and diagnoses were reported for each consecutive patient. Data were included if patients had at least one diagnosis of dementia according to the 10th revision of the International Classification of Diseases during the study period. Multiple logistic regression was used to determine factors associated with a prescription of any anti-dementia drug including Ginkgo biloba. Results During the 5-year study period (2004-2008), 577 patients with dementia were included (median age: 81 years (IQR: 74-87); 69% female). Dementia was classified as unspecified dementia (57.2%), vascular dementia (25.1%), dementia in Alzheimer's disease (10.4%), and dementia in Parkinson's disease (7.3%). The prevalence of anti-dementia drugs was 25.6%. The phytopharmaceutical Ginkgo biloba was the most frequently prescribed anti-dementia drug overall (67.6% of all) followed by cholinesterase inhibitors (17.6%). The adjusted odds ratio (AOR) for receiving any anti-dementia drug was greater than 1 for neurologists (AOR = 2.34; CI: 1.59-3.47), the diagnosis of Alzheimer's disease (AOR = 3.28; CI: 1.96-5.50), neuroleptic therapy (AOR = 1.87; CI: 1.22-2.88), co-morbidities hypertension (AOR = 2.03; CI: 1.41-2.90), and heart failure (AOR = 4.85; CI: 3.42-6.88). The chance for a prescription of any anti-dementia drug decreased with the diagnosis of vascular dementia (AOR = 0.64; CI: 0.43-0.95) and diabetes mellitus (AOR = 0.55; CI: 0.36-0.86). The prescription of Ginkgo biloba was associated with sex (female: AOR = 0.41; CI: 0.19-0.89), patient age (AOR = 1.06; CI: 1

  4. Neural correlates of attention‐executive dysfunction in lewy body dementia and Alzheimer's disease

    PubMed Central

    Kobeleva, Xenia; Cherry, George; Killen, Alison; Gallagher, Peter; Burn, David J.; Thomas, Alan J.; O'Brien, John T.; Taylor, John‐Paul

    2015-01-01

    Abstract Attentional and executive dysfunction contribute to cognitive impairment in both Lewy body dementia and Alzheimer's disease. Using functional MRI, we examined the neural correlates of three components of attention (alerting, orienting, and executive/conflict function) in 23 patients with Alzheimer's disease, 32 patients with Lewy body dementia (19 with dementia with Lewy bodies and 13 with Parkinson's disease with dementia), and 23 healthy controls using a modified Attention Network Test. Although the functional MRI demonstrated a similar fronto‐parieto‐occipital network activation in all groups, Alzheimer's disease and Lewy body dementia patients had greater activation of this network for incongruent and more difficult trials, which were also accompanied by slower reaction times. There was no recruitment of additional brain regions or, conversely, regional deficits in brain activation. The default mode network, however, displayed diverging activity patterns in the dementia groups. The Alzheimer's disease group had limited task related deactivations of the default mode network, whereas patients with Lewy body dementia showed heightened deactivation to all trials, which might be an attempt to allocate neural resources to impaired attentional networks. We posit that, despite a common endpoint of attention‐executive disturbances in both dementias, the pathophysiological basis of these is very different between these diseases. Hum Brain Mapp 37:1254–1270, 2016. © 2015 The Authors Human Brain Mapping Published by Wiley Periodicals, Inc. PMID:26705763

  5. Dementia and amputation

    PubMed Central

    Schuch, Vera; Moysidis, Theodoros; Weiland, Dorothea; Santosa, Frans

    2012-01-01

    Objectives To our experience dementia seems to play an increasing role for major amputation in patients suffering from peripheral arterial disease (PAD). To confirm our impression, we analysed the rate of dementia associated with different surgical procedures using the information of the federal statistics in Germany. Patients and Methods Detailed lists of cases hospitalized with the principal diagnosis (PAD), abdominal aortic aneurysm (AAA), myocardial infarction (MI) and hip fracture (HF), and of the procedures minor or major amputation, endovascular aortic repair (EVAR), total endoprosthesis for hip replacement (THR) and coronary aortic bypass graft (CABG) in Germany in the years 2008 to 2010 were provided by the Federal Statistical Office. Results Dementia is documented as additional diagnosis in approximately one fourth of cases having the principal diagnosis HF, 5% to 6% of cases with the principal diagnosis MI and PAD, but only in approximately 2% of AAA cases. Dementia is documented as principal or additional diagnosis in one fourth of amputation procedures (major amputation approximately 18% and minor amputation approximately 8%), in approximately 5% THR, 2% of EVAR and only 0.3% of CABG. The rate of documentation of dementia is higher in patients treated by major amputation than in the hospitalized PAD population. Vice versa, the rate of documentation of dementia is lower in patients getting THR than in the hospitalized HF population. Conclusion The presented analysis supports the assumption that dementia plays a relevant role in older patients suffering from PAD receiving major amputation in Germany. PMID:24265873

  6. Similarities of cerebral glucose metabolism in Alzheimer's and Parkinsonian dementia

    SciTech Connect

    Kuhl, D.E.; Metter, E.J.; Benson, D.F.; Ashford, J.W.; Riege, W.H.; Fujikawa, D.G.; Markham, C.H.; Maltese, A.

    1985-05-01

    In the dementia of probable Alzheimer's Disease (AD), there is a decrease in the metabolic ratio of parietal cortex/caudate-thalamus which relates measures in the most and in the least severely affected locations. Since some demented patients with Parkinson's Disease (PDD) are known to share pathological and neurochemical features with AD patients, the authors evaluated if the distribution of cerebral hypometabolism in PDD and AD were the same. Local cerebral metabolic rates were determined using the FDG method and positron tomography in subjects with AD (N=23), and PDD (N=7), multiple infarct dementia (MID)(N=6), and controls (N=10). In MID, the mean par/caudthal ratio was normal (0.79 +- 0.9, N=6). In AD and PDD patients, this ratio correlated negatively with both the severity (r=-0.624, rho=0.001) and duration (r=-0.657, rho=0.001) of dementia. The ratio was markedly decreased in subjects with mild to severe dementia (0.46 +- 0.09, N=21) and with dementia duration greater than two years (0.44 +- 0.08, N=18), but the ratio was also significantly decreased in patients with less advanced disease, i.e., when dementia was only questionable (0.64 +- 0.14, N=9) (t=2.27, rho<0.037) and when duration was two years or less (0.62 +- 0.13, N=12)(t=2.88, rho<0.009). This similarity of hypometabolism in AD and PDD is additional evidence that a common mechanism may operate in both disorders. The par/caud-thal metabolic ratio may be an index useful in the differential diagnosis of early dementia.

  7. Clinicians' ability to diagnose dementia with Lewy bodies is not affected by β-amyloid load

    PubMed Central

    Attems, Johannes; Thomas, Alan; Brown, Andrew; Jaros, Evelyn; Lett, Debbie J.; Ossola, Maria; Perry, Robert H.; Ramsay, Lynne; Walker, Lauren; McKeith, Ian G.

    2015-01-01

    Objective: To investigate whether an increasing load of β-amyloid and/or neuritic plaques influences the phenotype, and thus the clinical diagnostic accuracy, of dementia with Lewy bodies (DLB). Methods: A series of 64 subjects with autopsy-proven DLB was studied. Last diagnosis before death was used to determine the clinical diagnostic accuracy of DLB in relation to Lewy body distribution and extent of Alzheimer β-amyloid and/or neuritic pathology. DLB pathologic diagnosis was made according to consensus criteria, using α-synuclein immunostaining for Lewy body identification. β-Amyloid immunostaining was used for quantifying β-amyloid deposits. The Consortium to Establish a Registry for Alzheimer's Disease criteria and Braak stage were applied for semiquantitative grading of neuritic plaque and neurofibrillary tangle pathology. Results: Overall clinical diagnostic accuracy for the entire DLB cohort was high (80%), reflecting the high prevalence of core clinical features (fluctuations [81%], parkinsonism [77%], visual hallucinations [70%]). Lower frequencies of core clinical features of DLB, resulting in lower accuracy of its clinical diagnosis, were associated with decreasing Lewy body distribution (p < 0.0001) and with increasing neuritic plaque pathology (p = 0.035), but not with the number of β-amyloid plaque deposits. Conclusions: The likelihood of occurrence of the DLB clinical syndrome is positively related to the extent of Lewy body pathology and negatively related to the severity of Alzheimer neuritic pathology, while β-amyloid load has no effect. PMID:25552579

  8. Weight variation before and after surgery in Parkinson's disease: a noradrenergic modulation?

    PubMed

    Guimarães, Joana; Moura, Eduardo; Vieira-Coelho, Maria Augusta; Garrett, Carolina

    2012-08-01

    Changes in the nutritional profile of patients with Parkinson's disease have been reported before and after deep brain stimulation surgery. The major determinants of the weight variation in Parkinson's disease are not yet understood, and the mechanism seems complex. Based on the influence of the sympathetic nervous system in metabolic syndrome obesity, the intent of the present review is to consider the role of noradrenergic modulation on weight variations in Parkinson's disease. In this review the authors raise the following hypothesis: weight variation in Parkinson's disease before and after deep brain stimulation of the subthalamic nucleus could be influenced by noradrenergic interaction between the locus coeruleus, subthalamic nucleus, and hypothalamic nucleus. PMID:22700383

  9. [Dementia: management and prevention].

    PubMed

    Daher, Oscar; Nguyen, Sylvain; Smith, Cindi; Büla, Christophe; Démonet, Jean-François

    2016-04-20

    Dementia represents a great challenge for health care providers. Detection of cognitive impairment is critical for early diagnosis of dementia. Early diagnosis allows to initiate individualized management that focuses on maintaining patient's autonomy and supporting their caregivers. Proposed multimodal interventions include physical activity, cognitive training, mediterranean diet, and management of cardiovascular risk factors. Before the initiation of pro-cognitive therapy, medication review is essential to evaluate current treament and determine specific therapeutic objectives, based on patient's overall health and preferences. Overall risk reduction for dementia revolves around similar measures that target physical activity, cognition, diet and management of cardiovascular risk factors. PMID:27276724

  10. Cellular function and pathological role of ATP13A2 and related P-type transport ATPases in Parkinson's disease and other neurological disorders

    PubMed Central

    van Veen, Sarah; Sørensen, Danny M.; Holemans, Tine; Holen, Henrik W.; Palmgren, Michael G.; Vangheluwe, Peter

    2014-01-01

    Mutations in ATP13A2 lead to Kufor-Rakeb syndrome, a parkinsonism with dementia. ATP13A2 belongs to the P-type transport ATPases, a large family of primary active transporters that exert vital cellular functions. However, the cellular function and transported substrate of ATP13A2 remain unknown. To discuss the role of ATP13A2 in neurodegeneration, we first provide a short description of the architecture and transport mechanism of P-type transport ATPases. Then, we briefly highlight key P-type ATPases involved in neuronal disorders such as the copper transporters ATP7A (Menkes disease), ATP7B (Wilson disease), the Na+/K+-ATPases ATP1A2 (familial hemiplegic migraine) and ATP1A3 (rapid-onset dystonia parkinsonism). Finally, we review the recent literature of ATP13A2 and discuss ATP13A2's putative cellular function in the light of what is known concerning the functions of other, better-studied P-type ATPases. We critically review the available data concerning the role of ATP13A2 in heavy metal transport and propose a possible alternative hypothesis that ATP13A2 might be a flippase. As a flippase, ATP13A2 may transport an organic molecule, such as a lipid or a peptide, from one membrane leaflet to the other. A flippase might control local lipid dynamics during vesicle formation and membrane fusion events. PMID:24904274

  11. Clinical-pathologic correlations in vascular cognitive impairment and dementia.

    PubMed

    Flanagan, Margaret; Larson, Eric B; Latimer, Caitlin S; Cholerton, Brenna; Crane, Paul K; Montine, Kathleen S; White, Lon R; Keene, C Dirk; Montine, Thomas J

    2016-05-01

    The most common causes of cognitive impairment and dementia are Alzheimer's disease (AD) and vascular brain injury (VBI), either independently, in combination, or in conjunction with other neurodegenerative disorders. The contribution of VBI to cognitive impairment and dementia, particularly in the context of AD pathology, has been examined extensively yet remains difficult to characterize due to conflicting results. Describing the relative contribution and mechanisms of VBI in dementia is important because of the profound impact of dementia on individuals, caregivers, families, and society, particularly the stability of health care systems with the rapidly increasing age of our population. Here we discuss relationships between pathologic processes of VBI and clinical expression of dementia, specific subtypes of VBI including microvascular brain injury, and what is currently known regarding contributions of VBI to the development and pathogenesis of the dementia syndrome. This article is part of a Special Issue entitled: Vascular Contributions to Cognitive Impairment and Dementia edited by M. Paul Murphy, Roderick A. Corriveau and Donna M. Wilcock. PMID:26319420

  12. TDP-43 and frontotemporal dementia.

    PubMed

    Hu, William T; Grossman, Murray

    2009-09-01

    TAR DNA-binding protein of about 43 kDa (TDP-43) is the main ubiquitinated peptide in tau-negative frontotemporal lobar degeneration (FTLD). TDP-43 is typically a nuclear protein, and its aggregation and cytoplasmic translocation are thought to represent major steps in the pathogenesis of FTLD due to TDP-43 proteinopathy (FTLD-TDP). Certain clinical syndromes of frontotemporal dementia are preferentially associated with pathologic findings of FTLD-TDP, and TDP-43 pathology represents the connection between FTLD-TDP and amyotrophic lateral sclerosis. Recent advances in clinical, genetic, and pathologic studies of FTLD-TDP and amyotrophic lateral sclerosis have shed light on the potentially pathogenic role of TDP-43 and identified TDP-43 itself as a candidate biomarker for antemortem diagnosis of FTLD-TDP. PMID:19664364

  13. The history of parkinsonism: descriptions in ancient Indian medical literature.

    PubMed

    Ovallath, Sujith; Deepa, P

    2013-05-01

    The clinical syndrome of parkinsonism was identified in ancient India even before the period of Christ and was treated methodically. The earliest reference to bradykinesia dates to 600 bc. Evidences prove that as early as 300 bc, Charaka proposed a coherent picture of parkinsonism by describing tremor, rigidity, bradykinesia, and gait disturbances as its components. The scenario was further developed by Madhava, Vagbhata, and Dalhana all through history. The 15th-century classic "Bhasava rajyam" introduced the term kampavata, which may be regarded as an ayurvedic analogue of parkinsonism. The pathogenesis of kampavata centered on the concept of imbalance in the vata factor, which controls psychomotor activities. The essential element in therapy was the administration of powdered seed of Mucuna pruriens, or atmagupta, which as per reports, contains 4%-6% of levodopa. In addition to proving the existence and identification of parkinsonism in ancient India, the study points to the significance of ancient Indian Sanskrit works in medical history. PMID:23483637

  14. Dementia with Lewy Bodies

    MedlinePlus

    ... DLB and Parkinson’s disease, and between DLB and Alzheimer’s disease, can often make it difficult for a ... in the brains of people with Parkinson's and Alzheimer’s diseases. These findings suggest that either DLB is ...

  15. Pharmacological management of behavioral symptoms associated with dementia

    PubMed Central

    Madhusoodanan, Subramoniam; Ting, Mark Bryan

    2014-01-01

    Dementia is a clinical syndrome with features of neurocognitive decline. Subtypes of dementia include Alzheimer’s, frontotemporal, Parkinson’s, Lewy body disease, and vascular type. Dementia is associated with a variety of neuropsychiatric symptoms that may include agitation, psychosis, depression, and apathy. These symptoms can lead to dangerousness to self or others and are the main source for caregiver burnout. Treatment of these symptoms consists of nonpharmacological and pharmacological interventions. However, there are no Food and Drug Administration-approved medications for the treatment of behavioral and psychological symptoms of dementia. Pharmacological interventions are used off-label. This article reviews the current evidence supporting or negating the use of psychotropic medications along with safety concerns, monitoring, regulations, and recommendations. PMID:25540722

  16. Tau PET: the next frontier in molecular imaging of dementia.

    PubMed

    Xia, Chenjie; Dickerson, Bradford C

    2016-09-01

    We have arrived at an exciting juncture in dementia research: the second major pathological hallmark of Alzheimer's disease (AD)-tau-can now be seen for the first time in the living human brain. The major proteinopathies in AD include amyloid-β plaques and neurofibrillary tangles (NFTs) made of hyperphosphorylated paired helical filament (PHF) tau. Since its advent more than a decade ago, amyloid PET imaging has revolutionized the field of dementia research, enabling more confident diagnosis of the likely pathology in patients with a variety of clinical dementia syndromes, paving the way for the identification of people with preclinical or prodromal AD pathology, and serving as a minimally invasive molecular readout in clinical trials of putative disease-modifying interventions. Now that we are on the brink of a second revolution in molecular imaging in dementia, it is worth considering the likely potential impact of this development on the field. PMID:27334648

  17. A clinical overview of non-motor symptoms in Parkinson's Disease.

    PubMed

    Modugno, Nicola; Lena, Francesco; Di Biasio, Francesca; Cerrone, Gloria; Ruggieri, Stefano; Fornai, Francesco

    2013-12-01

    Although Parkinson's disease (PD) is diagnosed on the basis of motor symptoms, including slowness of movement, tremor, rigidity and difficulties with balance and walking, now we are aware that non-motor symptoms are highly prevalent, since they can anticipate motor symptoms and can cause severe consequences. Several studies have shown that non-motor symptoms, such as depression, anxiety and apathy, psychosis (e.g., hallucinations, delusions), sleep disturbance, and pain may have a greater adverse impact on quality of life and health economics compared with motor symptoms. Non-motor symptoms can be divided into four domains: neuropsychiatric (e.g., depression, anxiety, apathy, hallucinations, dementia), autonomic (e.g., constipation, orthostatic hypotension, urinary changes, sweating abnormalities), sleep (e.g., insomnia, sleep fragmentation, excessive daytime sleepiness, rapid eye movement, sleep behavioural disorder, restless leg syndrome), and sensory dysfunction (e.g., pain, olfactory dysfunction). This review addresses diagnosis and treatment of these disorders. The causative mechanisms remain complex, since they reflect the widespread brainstem and cortical pathology of PD, with involvement of several neurotransmitters, including dopamine (DA), serotonin, norepinephrine, and acetylcholine. The diagnosis is often challenging, especially for psychiatric disorders, and in particular affective disorders, because somatic features of psychopathology may overlap with the movement disorder itself. Treatments used are limited and psychiatric drugs may not be as effective as in general population. Evidence based medicine is quite poor and it still requires well-designed clinical studies. PMID:24873924

  18. Midlife migraine and late-life parkinsonism

    PubMed Central

    Ross, G. Webster; Sigurdsson, Sigurdur; Garcia, Melissa; Gudmundsson, Larus S.; Sveinbjörnsdóttir, Sigurlaug; Wagner, Amy K.; Gudnason, Vilmundur; Launer, Lenore J.

    2014-01-01

    Objective: In the present study, we tested the hypothesis that having migraine in middle age is related to late-life parkinsonism and a related disorder, restless legs syndrome (RLS), also known as Willis-Ekbom disease (WED). Methods: The AGES-Reykjavik cohort (born 1907–1935) has been followed since 1967. Headaches were classified based on symptoms assessed in middle age. From 2002 to 2006, 5,764 participants were reexamined to assess symptoms of parkinsonism, diagnosis of Parkinson disease (PD), family history of PD, and RLS/WED. Results: Subjects with midlife migraine, particularly migraine with aura (MA), were in later life more likely than others to report parkinsonian symptoms (odds ratio [OR]MA = 3.6 [95% CI 2.7–4.8]) and diagnosed PD (ORMA = 2.5 [95% CI 1.2–5.2]). Women with MA were more likely than others to have a parent (ORMA = 2.26 [95% CI 1.3–4.0]) or sibling (ORMA = 1.78 [95% CI 1.1–2.9]) with PD. Late-life RLS/WED was increased for headache generally. Associations were independent of cardiovascular disease and MRI-evident presumed ischemic lesions. Conclusions: These findings suggest there may be a common vulnerability to, or consequences of, migraine and multiple indicators of parkinsonism. Additional genetic and longitudinal observational studies are needed to identify candidate pathways that may account for the comorbid constellation of symptoms. PMID:25230997

  19. Burden of Dementia

    MedlinePlus

    ... Us FAQs Stay Connected You are here Home Global Health and Aging The Burden of Dementia The ... this total in less developed countries ( Figure 9 ). Global efforts are underway to understand and find cures ...

  20. Dementia - home care

    MedlinePlus

    ... help improve communication skills and prevent wandering. Calming music may reduce wandering and restlessness, ease anxiety, and ... prevent falls Ways to improve bathroom safety The Alzheimer's Association's Safe Return Program requires people with dementia ...

  1. Preventing and diagnosing dementia.

    PubMed

    Keenan, Bernie; Jenkins, Catharine; Ginesi, Laura

    While dementia is an umbrella term for a range of degenerative brain disorders, many share similar presentations. Nurses are ideally placed to identify those at risk and empower them to access treatment and plan and prepare for their future needs--as such, they need up-to-date knowledge of the signs and symptoms of the different types of dementia to identify risk factors and make an informed diagnosis. This article, the third in a four-part series on dementia, examines the risk factors, signs, symptoms and diagnosis of dementia, as well as outlining lifestyle factors such as diet and exercise that may help to prevent the development of the condition. PMID:27544960

  2. Multi-Infarct Dementia

    MedlinePlus

    ... Funding Information Research Programs Training & Career Awards Enhancing Diversity Find People About NINDS NINDS Multi-Infarct Dementia ... News From NINDS | Find People | Training | Research | Enhancing Diversity Careers@NINDS | FOIA | Accessibility Policy | Contact Us | Privacy ...

  3. Young onset dementia

    PubMed Central

    Sampson, E; Warren, J; Rossor, M

    2004-01-01

    Young onset dementia is a challenging clinical problem with potentially devastating medical and social consequences. The differential diagnosis is wide, and includes a number of rare sporadic and hereditary diseases. However, accurate diagnosis is often possible, and all patients should be thoroughly investigated to identify treatable processes. This review presents an approach to the diagnosis, investigation, and management of patients with young onset dementia, with particular reference to common and treatable causes. PMID:15016933

  4. Neuroimaging and dementia

    SciTech Connect

    Benson, D.F.

    1986-05-01

    The tremendous increase in dementia has created a need for improved diagnostic techniques, and each of the newly established brain imaging techniques has been applied to this problem. Several, particularly computerized tomography (CT), magnetic resonance imaging (MRI), and isotope emission tomography, have proved valuable. Each procedure has strengths--specific disorders that can be diagnosed--and weaknesses--types of dementia that cannot be demonstrated.

  5. Hereditary Parkinson s Disease Natural History Protocol

    ClinicalTrials.gov

    2016-08-31

    Parkinson Disease 6, Early-Onset; Parkinson Disease (Autosomal Recessive, Early Onset) 7, Human; Parkinson Disease Autosomal Recessive, Early Onset; Parkinson Disease, Autosomal Recessive Early-Onset, Digenic, Pink1/Dj1

  6. Autophagy in dementias.

    PubMed

    Kragh, Christine Lund; Ubhi, Kiren; Wyss-Coray, Tony; Wyss-Corey, Tony; Masliah, Eliezer

    2012-01-01

    Dementias are a varied group of disorders typically associated with memory loss, impaired judgment and/or language and by symptoms affecting other cognitive and social abilities to a degree that interferes with daily functioning. Alzheimer's disease (AD) is the most common cause of a progressive dementia, followed by dementia with Lewy bodies (DLB), frontotemporal dementia (FTD), (VaD) and HIV-associated neurocognitive disorders (HAND). The pathogenesis of this group of disorders has been linked to the abnormal accumulation of proteins in the brains of affected individuals, which in turn has been related to deficits in protein clearance. Autophagy is a key cellular protein clearance pathway with proteolytic cleavage and degradation via the ubiquitin-proteasome pathway representing another important clearance mechanism. Alterations in the levels of autophagy and the proteins associated with the autophagocytic pathway have been reported in various types of dementias. This review will examine recent literature across these disorders and highlight a common theme of altered autophagy across the spectrum of the dementias. PMID:22150925

  7. Early Dementia Screening.

    PubMed

    Panegyres, Peter K; Berry, Renee; Burchell, Jennifer

    2016-01-01

    As the population of the world increases, there will be larger numbers of people with dementia and an emerging need for prompt diagnosis and treatment. Early dementia screening is the process by which a patient who might be in the prodromal phases of a dementing illness is determined as having, or not having, the hallmarks of a neurodegenerative condition. The concepts of mild cognitive impairment, or mild neurocognitive disorder, are useful in analyzing the patient in the prodromal phase of a dementing disease; however, the transformation to dementia may be as low as 10% per annum. The search for early dementia requires a comprehensive clinical evaluation, cognitive assessment, determination of functional status, corroborative history and imaging (including MRI, FDG-PET and maybe amyloid PET), cerebrospinal fluid (CSF) examination assaying Aβ1-42, T-τ and P-τ might also be helpful. Primary care physicians are fundamental in the screening process and are vital in initiating specialist investigation and treatment. Early dementia screening is especially important in an age where there is a search for disease modifying therapies, where there is mounting evidence that treatment, if given early, might influence the natural history-hence the need for cost-effective screening measures for early dementia. PMID:26838803

  8. [Post Stroke Dementia].

    PubMed

    Ihara, Masafumi

    2016-07-01

    Post-stroke dementia (PSD) is a clinical entity that encompasses all types of dementia following an index stroke. Current evidence suggests that 25-30% of ischemic stroke survivors develop immediate or delayed vascular cognitive impairment or vascular dementia. The type of stroke can be either ischemic, hemorrhagic or hypoperfusive. There are multiple risk factors for PSD including older age, family history, genetic variants, low educational status, vascular comorbidities, prior transient ischemic attack or recurrent stroke and depressive illness. Pre-stroke dementia refers to the occurrence of cognitive impairment before the index stroke, which may be caused by a vascular burden as well as insidious neurodegenerative changes. Neuroimaging determinants of dementia after stroke include silent brain infarcts, white matter changes, lacunar infarcts and medial temporal lobe atrophy. Published clinical trials have not been promising and there is little information on whether PSD can be prevented using pharmacological agents. Control of vascular disease risk and prevention of recurrent strokes are key to reducing the burden of cognitive decline and post-stroke dementia. Modern imaging and analysis techniques will help to elucidate the mechanism of PSD and establish better treatment. PMID:27395459

  9. Early Dementia Screening

    PubMed Central

    Panegyres, Peter K.; Berry, Renee; Burchell, Jennifer

    2016-01-01

    As the population of the world increases, there will be larger numbers of people with dementia and an emerging need for prompt diagnosis and treatment. Early dementia screening is the process by which a patient who might be in the prodromal phases of a dementing illness is determined as having, or not having, the hallmarks of a neurodegenerative condition. The concepts of mild cognitive impairment, or mild neurocognitive disorder, are useful in analyzing the patient in the prodromal phase of a dementing disease; however, the transformation to dementia may be as low as 10% per annum. The search for early dementia requires a comprehensive clinical evaluation, cognitive assessment, determination of functional status, corroborative history and imaging (including MRI, FDG-PET and maybe amyloid PET), cerebrospinal fluid (CSF) examination assaying Aβ1–42, T-τ and P-τ might also be helpful. Primary care physicians are fundamental in the screening process and are vital in initiating specialist investigation and treatment. Early dementia screening is especially important in an age where there is a search for disease modifying therapies, where there is mounting evidence that treatment, if given early, might influence the natural history—hence the need for cost-effective screening measures for early dementia. PMID:26838803

  10. Young onset dementia.

    PubMed

    Draper, B; Withall, A

    2016-07-01

    Young onset dementia (YOD), where symptoms of dementia have an onset before the age of 65, has become more prominent due to the population increase from the Baby Boomer generation. This clinical perspective examines key issues in the assessment, diagnosis and management of YOD. Challenges in the assessment and diagnosis of YOD are partly due to the diverse range of types of YOD, where degenerative dementias are less common and secondary dementias more common than in late onset dementia. Early symptoms are broad and include depression, behavioural change, neurological disorders, systemic disorders and mild cognitive impairment (MCI). Perceived diagnostic delay may result in frustration and distress in people with YOD and their families. Chronic depression and MCI are associated with longer time to diagnosis, and in these situations, clinicians need to establish appropriate review processes and communicate clearly. A diagnosis of YOD may have marked consequences for a younger person, including early retirement, financial impacts and the psychological challenge of coming to grips with cognitive decline. Partners, children and other supporters often have unmet needs, feel burdened by care and are at high risk of physical and emotional consequences. Concerns about the heritability of dementia may add to family distress. Recent community service developments in Australia for YOD are outlined and the challenges of residential care described. PMID:27405890

  11. Genetics Home Reference: frontotemporal dementia with parkinsonism-17

    MedlinePlus

    ... FTDP-17 also have restricted up-and-down eye movement (vertical gaze palsy) and rapid abnormal movements of ... characterized by the gradual death of cells in areas of the brain called the frontal and temporal ...

  12. Parkinson's Disease Foundation Newsletter

    MedlinePlus

    ... Newsletters These include monthly e-newsletters and quarterly science-specific e-newsletters. Read the latest issue below or browse the archives. National Parkinson Foundation and the Parkinson’s Disease Foundation Complete Merger to ...

  13. [Depression in Parkinson's disease].

    PubMed

    Murata, Miho; Okamoto, Tomoko

    2013-01-01

    The frequency of depression in patients with Parkinson's disease is approximately 30-40%. Depression has a significantly negative impact on the QOL in Parkinson's disease patients. It leads to the worsening of tremors and frozen gait without disease progression and decreases the patient's motivation to participate in rehabilitation. The distinguishing feature of depression in patients with Parkinson's disease is that guilt, self-blame and suicidal ideation are rarely seen compared to that observed in patients with major depression. Depression can occur in the pre-motor, diagnostic and advanced stages of Parkinson's disease. In particular, patients with wearing-off symptoms are apt to develop anxiety. As for treatment, it is very important to optimize dopamine replacement therapy. Antiparkinsonian drugs may have beneficial effects not only on the motor symptoms of the disease, but also the patient's mood. Cognitive behavioral therapy (CBT) and peer counseling may also be beneficial. PMID:24622217

  14. Dementia and Cognitive Impairment: Epidemiology, Diagnosis, and Treatment

    PubMed Central

    Hugo, Julie; Ganguli, Mary

    2014-01-01

    Synopsis Clinicians can diagnose the syndromes of dementia (major neurocognitive disorder) and mild cognitive impairment (mild neurocognitive disorder) based on history, examination, and appropriate objective assessments, using standard criteria such as DSM-5. They can then diagnose the etiological subtypes of these syndromes using standard criteria for each of them. Brain imaging and biomarkers are gaining ground for the differential diagnoses among the different disorders. Treatments for the most part are still symptomatic. PMID:25037289

  15. [The Parkinson puzzle].

    PubMed

    Guseo, András

    2012-12-30

    Parkinson's disease is one of the most frequent progressive degenerative disorders with unknown origin of the nervous system. The commutation of the disease on Guam led to the discovery of a neurotoxin which was also found in other continents. This neurotoxin was identified in the common cyanobacteria (blue-green algae). Early clinical observations suggested some loose correlations with gastric and duodenal ulcer and Parkinson's disease, while recent studies revealed a toxin, almost identical to that found in cyanobacteria in one strain of Helicobacter pylori, which proved to cause Parkinson like symptoms in animals. Therefore, it cannot be ruled out that there is a slowly progressive poisoning in Parkinson's disease. The disease specific alpha-sinuclein inclusions can be found in nerve cells of the intestinal mucosa far before the appearance of clinical symptoms indicating that the disease may start in the intestines. These results are strengthened by the results of Borody's fecal transplants, after which in Parkinson patients showed a symptomatic improvement. Based on these observations the Parkinson puzzle is getting complete. Although these observations are not evidence based, they may indicate a new way for basic clinical research, as well as a new way of thinking for clinicians. These new observations in psycho-neuro-immunology strengthen the fact that immunological factors may also play a critical factor facilitating local cell necrosis which may be influenced easily. PMID:23261994

  16. Behavioral and Psychological Symptoms of Dementia

    PubMed Central

    Cerejeira, J.; Lagarto, L.; Mukaetova-Ladinska, E. B.

    2012-01-01

    Behavioral and psychological symptoms of dementia (BPSD), also known as neuropsychiatric symptoms, represent a heterogeneous group of non-cognitive symptoms and behaviors occurring in subjects with dementia. BPSD constitute a major component of the dementia syndrome irrespective of its subtype. They are as clinically relevant as cognitive symptoms as they strongly correlate with the degree of functional and cognitive impairment. BPSD include agitation, aberrant motor behavior, anxiety, elation, irritability, depression, apathy, disinhibition, delusions, hallucinations, and sleep or appetite changes. It is estimated that BPSD affect up to 90% of all dementia subjects over the course of their illness, and is independently associated with poor outcomes, including distress among patients and caregivers, long-term hospitalization, misuse of medication, and increased health care costs. Although these symptoms can be present individually it is more common that various psychopathological features co-occur simultaneously in the same patient. Thus, categorization of BPSD in clusters taking into account their natural course, prognosis, and treatment response may be useful in the clinical practice. The pathogenesis of BPSD has not been clearly delineated but it is probably the result of a complex interplay of psychological, social, and biological factors. Recent studies have emphasized the role of neurochemical, neuropathological, and genetic factors underlying the clinical manifestations of BPSD. A high degree of clinical expertise is crucial to appropriately recognize and manage the neuropsychiatric symptoms in a patient with dementia. Combination of non-pharmacological and careful use of pharmacological interventions is the recommended therapeutic for managing BPSD. Given the modest efficacy of current strategies, there is an urgent need to identify novel pharmacological targets and develop new non-pharmacological approaches to improve the adverse outcomes associated with

  17. Therapeutic interventions in parkinsonism: Corticobasal degeneration.

    PubMed

    Marsili, Luca; Suppa, Antonio; Berardelli, Alfredo; Colosimo, Carlo

    2016-01-01

    Corticobasal degeneration (CBD) is a progressive neurodegenerative disorder resulting from pathological accumulation of tau protein and is included in the spectrum of Atypical Parkinsonism. The typical clinical phenotype of CBD is characterized by the Corticobasal syndrome (CBS). In recent years it has become clear that the clinical picture of CBS may be caused by different pathological conditions, resulting in frequent misdiagnosis. CBD has high morbidity and poor prognosis with no effective therapies. In this review, we will discuss the symptomatic treatment, the palliative care and the disease modifying strategies currently in use. Symptomatic treatment in patients with CBD may sometimes be useful for improving motor (parkinsonism, dystonia and myoclonus) and non-motor (cognitive-behavioral) symptoms, but the effects are often unsatisfactory. In addition, non-pharmacological strategies and palliative care are useful integrating components of the multidisciplinary therapeutic approach for patients with CBD. Despite many efforts, a disease-modifying treatment is still unavailable for CBD. PMID:26382843

  18. Extrapyramidal features in advanced Down's syndrome: clinical evaluation and family history.

    PubMed Central

    Vieregge, P; Ziemens, G; Freudenberg, M; Piosinski, A; Muysers, A; Schulze, B

    1991-01-01

    Extrapyramidal, frontal release, and other neurological signs were studied in 54 demented and non-demented patients with Down's syndrome (DS). Fourteen patients were demented and five showed extrapyramidal signs, mainly of the rigid-hypokinetic spectrum and similar to Parkinsonian features in advanced Alzheimer's disease (AD). None of the non-demented patients had Parkinsonian signs. The mean age of the demented DS patients with extrapyramidal signs was significantly higher than that of the patients without. Frontal release signs were present in demented and non-demented patients. A questionnaire showed no increase in either the proportion of early- or senile-onset dementia or Parkinsonism among first- and second-degree relatives of DS patients. Parkinsonian signs appear to be present at a lower frequency in DS than in advanced AD. A speculative hypothesis about a gene dosage effect of Cu/Zn-superoxide dismutase in preventing toxic radical formation in the substantia nigra of DS patients is presented. PMID:1826326

  19. Clinical course and prognosis in patients with Gaucher disease and parkinsonism

    PubMed Central

    Lopez, Grisel; Kim, Jenny; Wiggs, Edythe; Cintron, Dahima; Groden, Catherine; Tayebi, Nahid; Mistry, Pramod K.; Pastores, Gregory M.; Zimran, Ari; Goker-Alpan, Ozlem

    2016-01-01

    Objective: The goal of this study was to characterize the parkinsonian phenotype in patients with Gaucher disease (GD) who developed parkinsonism in order to evaluate clinical course and prognosis. Methods: This is a retrospective observational study conducted at the Clinical Center of the NIH, Bethesda, MD, over a period of 10 years. The study included 19 patients with GD and parkinsonism. The severity of Gaucher and parkinsonian symptoms was determined from clinical data including physical, neurologic, pathologic, and neurocognitive evaluations, family histories, imaging studies, olfactory testing, and validated questionnaires. Results: We found an earlier age at onset of parkinsonism and evidence of mild cognitive dysfunction in our cohort. Although the clinical course in some patients was similar to that of idiopathic Parkinson disease with a favorable levodopa response, others exhibited features characteristic of dementia with Lewy bodies. When we examined the patients as a group, we did not observe a uniformly aggressive form of parkinsonism after the initial onset of symptoms, contrary to other published reports. Conclusions: Appreciable clinical variation was seen in this cohort with GD and parkinsonism. Although some patients had early onset and prominent cognitive changes, others had a later, slower course, indicating that GBA1 mutations may not be a reliable prognostic indicator in Parkinson disease in clinical settings. PMID:27123476

  20. Computerised brain electrical activity findings of parkinson patients suffering from hyperkinetic side effects (hypersensitive dopamine syndrome) and a review of possible sources.

    PubMed

    Fünfgeld, E W

    1995-01-01

    Among 2,000 Park. pat. hospitalised during the years 1988 till 1990 we found 61 pat. with hyperkinetic side-effects in the sequence of a long-term L-dopa treatment. 43 (mean age 64y.) had a complete clinical data set and hyperkinesia ratings (AIMS scale). Compared to the system's data base--none of these patients showed a strickly normal CEEG. Our standard parkinson treatment was established in 1986: L-dopa as low as possible, dopamine agonists low, amantadines as high as necessary, L-deprenyl till 10 mg and anticholinergics--no, if possible. Our pat. group got initially a mean of 508 mg L-dopa, at the end of the study 296 mg. Conventional EEG (Picker-Schwarzer) and CEEG data were recorded (Dynamic Brain Mapping, Itil). Clinical and CEEG follow-up investigations were done after 2 weeks and 2 years. At follow-up, a reduction of the hyperkinesias was found in 43 patients (AIMS scale). 28 pat. received an additional treatment with nootropic drugs, 15 pat. were without this additional therapy. The visually evaluated CEEG at the latter group showed an acceleration ("response") in 33%, but among the patients with nootropic drugs the acceleration was seen in 66%. In patients without nootropics the mean delta power increased, patients additionally treated showed a reduction of delta. In cases of high L-dopa dosage--associated with severe hyperkinetic side effects--more slow waves were registered. The non-nootropic treated patients were divided into two groups--non-responders and the responders: a) Non-responders (n = 10) showed a significant reduction of alpha and a significant increase of delta and theta, b) Responders (5 pat.) without nootropics had a significant reduction of theta and a increase of alpha, but the acceleration is less pronounced than in the responder group with nootropics. Without co-medication a high L-dopa dosage may provoke/facilitate an organic cerebral dysfunction. The nootropic-treated but non-responder group showed no significant changes (n = 13