Pan, Fen; Wang, Jun-Yuan; Xu, Yi; Huang, Man-Li
It is widely believed that structural abnormalities of the brain contribute to the pathophysiology of schizophrenia. The parietal lobe is a central hub of multisensory integration, and abnormities in this region might account for the clinical features of schizophrenia. However, few cases of parietal encephalomalacia associated with schizophrenia have been described. In this paper, we present a case of a 25-year-old schizophrenia patient with abnormal parietal encephalomalacia. The patient had poor nutrition and frequently had upper respiratory infections during childhood and adolescence. She showed severe schizophrenic symptoms such as visual hallucinations for 2 years. After examining all her possible medical conditions, we found that the patient had a lesion consistent with the diagnosis of encephalomalacia in her right parietal lobe and slight brain atrophy. The patient was prescribed olanzapine (10 mg per day). Her symptoms significantly improved after antipsychotic treatment and were still well controlled 1 year later. This case suggested that parietal encephalomalacia, which might be caused by inflammatory and infectious conditions in early life and be aggravated by undernutrition, might be implicated in the etiology of schizophrenia.
Love, Rozalyn; Lee, Amy; Matiasek, April; Carter, William; Ylagan, Marissa
Introduction Encephalomalacia in a developing fetus is a rare and devastating neurological finding on radiologic imaging. Maternal diabetic ketoacidosis (DKA) can lead to metabolic and vascular derangements which can cause fetal encephalomalacia. Case We report the case of a 27-year-old pregnant woman with White's Class C diabetes mellitus who presented in the 25th week of gestation with DKA. Four weeks after her discharge, marked fetal cerebral ventriculomegaly was noted on ultrasound. A subsequent fetal magnetic resonance imaging (MRI) demonstrated extensive, symmetric cystic encephalomalacia, primarily involving both cerebral hemispheres. The pregnancy was continued with close fetal and maternal surveillance. The patient underwent a repeat cesarean delivery in her 37th week. The infant had a 1 month neonatal intensive care unit stay with care rendered by a multiple disciplinary team of pediatric subspecialists. The postnatal course was complicated by global hypotonia, poor feeding, delayed development and ultimately required anticonvulsants for recurrent seizures. He died at the age of 9 months from aspiration during a seizure. Discussion Although the maternal mortality from DKA has declined, DKA still confers significant neurological fetal morbidity to its survivors. PMID:25452892
Love, Rozalyn; Lee, Amy; Matiasek, April; Carter, William; Ylagan, Marissa
Introduction Encephalomalacia in a developing fetus is a rare and devastating neurological finding on radiologic imaging. Maternal diabetic ketoacidosis (DKA) can lead to metabolic and vascular derangements which can cause fetal encephalomalacia. Case We report the case of a 27-year-old pregnant woman with White's Class C diabetes mellitus who presented in the 25th week of gestation with DKA. Four weeks after her discharge, marked fetal cerebral ventriculomegaly was noted on ultrasound. A subsequent fetal magnetic resonance imaging (MRI) demonstrated extensive, symmetric cystic encephalomalacia, primarily involving both cerebral hemispheres. The pregnancy was continued with close fetal and maternal surveillance. The patient underwent a repeat cesarean delivery in her 37th week. The infant had a 1 month neonatal intensive care unit stay with care rendered by a multiple disciplinary team of pediatric subspecialists. The postnatal course was complicated by global hypotonia, poor feeding, delayed development and ultimately required anticonvulsants for recurrent seizures. He died at the age of 9 months from aspiration during a seizure. Discussion Although the maternal mortality from DKA has declined, DKA still confers significant neurological fetal morbidity to its survivors.
Folio, Les; Solomon, Jeffrey; Biassou, Nadia; Fischer, Tatjana; Dworzak, Jenny; Raymont, Vanessa; Sinaii, Ninet; Wassermann, Eric M.; Grafman, Jordan
Background Penetrating head injuries (PHIs) are common in combat operations and most have visible wound paths on computed tomography (CT). Objective We assess agreement between an automated trajectory analysis-based assessment of brain injury and manual tracings of encephalomalacia on CT. Methods We analyzed 80 head CTs with ballistic PHI from the Institutional Review Board approved Vietnam head injury registry. Anatomic reports were generated from spatial coordinates of projectile entrance and terminal fragment location. These were compared to manual tracings of the regions of encephalomalacia. Dice’s similarity coefficients, kappa, sensitivities, and specificities were calculated to assess agreement. Times required for case analysis were also compared. Results Results show high specificity of anatomic regions identified on CT with semiautomated anatomical estimates and manual tracings of tissue damage. Radiologist’s and medical students’ anatomic region reports were similar (Kappa 0.8, t-test p < 0.001). Region of probable injury modeling of involved brain structures was sensitive (0.7) and specific (0.9) compared with manually traced structures. Semiautomated analysis was 9-fold faster than manual tracings. Conclusion Our region of probable injury spatial model approximates anatomical regions of encephalomalacia from ballistic PHI with time-saving over manual methods. Results show potential for automated anatomical reporting as an adjunct to current practice of radiologist/neurosurgical review of brain injury by penetrating projectiles. PMID:23707123
Bovine spongiform encephalopathy was not confirmed histologically in 225 of 829 bovine brains submitted for diagnosis. Several previously described disorders of the central nervous system were observed in these brains as well as disorders not previously recognised in Britain, including bilateral vacuolation of the substantia nigra, hippocampal sclerosis with brainstem neuronal chromatolysis and necrosis, focal symmetrical encephalomalacia and meningio-angiomatosis. Severe cerebellar dysplasia consistent with pre-natal bovine viral diarrhoea--mucosal disease virus infection or mineralisation of the blood vessels of the basal ganglia were interpreted respectively as congenital changes or changes due to ageing and were considered to be of no clinical significance.
Reynolds, Thomas Q.; Roy, Asim
Cataplexy is a complex neurologic phenomenon during wakefulness probably resulting from impairment of pontine and hypothalamic control over muscle tone. REM sleep behavior disorder (RSBD) is characterized by the presence of REM sleep without atonia manifesting clinically as disruptive or injurious behaviors. We present here a patient with both cataplexy and RSBD following pontine encephalomalacia. The clinical presentation provides insight into the possible pathobiology of both waking and sleeping disorders of REM sleep regulation. Citation: Reynolds TQ; Roy A. Isolated cataplexy and REM sleep behavior disorder after pontine stroke. J Clin Sleep Med 2011;7(2):211-213. PMID:21509338
Tulloch, Isabel; Palmer, Siobhan; Scott, Richard; Lozsadi, Dora; Martin, Andrew J
We report the case of a 55-year-old woman presenting with progressive memory impairment secondary to a transsphenoidal encephalocele involving her dominant medial temporal lobe. Her clinical deterioration was accompanied by radiological progression in the encephalocele's size and associated encephalomalacia. Through a temporal craniotomy, her encephalocele was resected and the defect closed. Baseline neuropsychological assessment indicated global cognitive impairment, but post-operatively, she reported improved memory and concentration. Standardized assessment reflected an improvement in perceptual skills and an associated improved recall of a complex figure. This is the first case report to date of a patient's memory improving following treatment of a basal encephalocele.
Forrester, K R; Keegan, K M; Schmidt, J W
It has been established that twin pregnancies are at an increased risk for complications, including the risk of morbidity or mortality for one or both of the infants. Cerebral palsy and other associated neurological deficits also occur at higher rates in twin pregnancies. This report examines two cases of intrauterine demise of one twin with subsequent survival of the co-twin. In both cases, the surviving infant suffered significant neurological sequelae. Impairments observed in these two cases include multicystic encephalomalacia and periventricular leukomalacia as well as the subsequent development of cerebral palsy. This case study explores the predisposing factors, incidence, pathophysiology, consequences, and future research implications of these findings.
Mo, Andrew Z; Asemota, Anthony O; Venkatesan, Arun; Ritzl, Eva K; Njoku, Dolores B; Sponseller, Paul D
Intraoperative neuromonitoring (IONM) is widely used to reduce postoperative neurological complications during scoliosis correction. IONM allows intraoperative detection of neurological insults to the spinal cord and enables surgeons to react in real time. IONM failure rates can reach 61% in patients with cerebral palsy (CP). Factors decreasing the quality of IONM signals or making IONM impossible in CP patients undergoing scoliosis correction have not been well described. We categorized IONM data from 206 children with CP who underwent surgical scoliosis correction at a single institution from 2002 through 2013 into 3 groups: (1) "no signals," if neither somatosensory-evoked potentials (SSEP) nor transcranial motor-evoked potentials (TcMEP) could be obtained; (2) "no sensory," if no interpretable SSEP were obtained regardless of interpretable TcMEP; and (3) "no motor," if no interpretable TcMEP were obtained regardless of interpretable SSEP. We analyzed preexisting neuroimaging, available for 93 patients, and neurological status of the full cohort against these categories. Statistical analysis of univariate and multivariate associations was performed using logistic regression. Odds ratios (ORs) were calculated with significance set at P<0.05. Multivariate analysis showed significant associations of periventricular leukomalacia (PVL), hydrocephalus, and encephalomalacia with lack of meaningful and interpretable signals. Focal PVL (Fig. 1) was associated with no motor (OR=39.95; P=0.04). Moderate hydrocephalus was associated with no signals (OR=32.35; P<0.01), no motor (OR=10.14; P=0.04), and no sensory (OR=8.44; P=0.03). Marked hydrocephalus (Fig. 2) was associated with no motor (OR=20.46; P<0.01) and no signals (OR=8.83; P=0.01). Finally, encephalomalacia (Fig. 3) was associated with no motor (OR=6.99; P=0.01) and no signals (OR=4.26; P=0.03). Neuroanatomic findings of PVL, hydrocephalus, and encephalomalacia are significant predictors of limited IONM signals
Passantino, Giuseppe; Marino, Fabio; Gaglio, Gabriella; Patruno, Rosa; Lanteri, Giovanni; Zizzo, Nicola
Severe lung strongylosis was detected in a wild red fox (Vulpes vulpes) (1/12) from Apulia (Italy). We performed routine diagnostics on 12 foxes found dead in Apulia. Eleven of them showed lesions consistent with a vehicle collision. However, the remaining fox appeared to have died from other causes. At necropsy we observed, catarrhal enteritis, fatty liver, lung congestion with some areas rm in consistence and brain haemorrhages and malacia. Histopathology revealed lung brosis with mononucleate cells in ltration, thrombosis a several larval nematodes spread in the parenchyma, interstitial nephritis, interstitial myocarditis, encephalitis, encephalomalacia, and a brain granuloma. The larvae recovered from the lung parenchyma were identi ed as the rst stage larvae of Angiostrongylus vasorum. This is the rst documented report of angiostrongylosis in a fox in Southern Italy.
Cho, Yang-Je; Han, Sang-Don; Song, Sook Keun; Lee, Byung In; Heo, Kyoung
Palilalia is a relatively rare pathologic speech behavior and has been reported in various neurologic and psychiatric disorders. We encountered a case of palilalia, echolalia, and echopraxia-palipraxia as ictal phenomena of left frontal lobe epilepsy. A 55-year-old, right-handed man was admitted because of frequent episodes of rapid reiteration of syllables. Video-electroencephalography monitoring revealed stereotypical episodes of palilalia accompanied by rhythmic head nodding and right-arm posturing with ictal discharges over the left frontocentral area. He also displayed echolalia or echopraxia-palipraxia, partially responding to an examiner's stimulus. Magnetic resonance imaging revealed encephalomalacia on the left superior frontal gyrus and ictal single photon emission computed tomography showed hyperperfusion just above the lesion, corresponding to the left supplementary motor area (SMA), and subcortical nuclei. This result suggests that the neuroanatomic substrate involved in the generation of these behaviors as ictal phenomena might exist in the SMA of the left frontal lobe.
Xiao, Lianxiang; Lin, Xiangtao; Cao, Jinfeng; Wang, Xueyu; Wu, Lebin
Compound diphenoxylate (diphenoxylate-atropine) poisoning can cause toxic encephalopathy in children, and magnetic resonance imaging (MRI) of the brain in this condition has not been reported. This study is to analyze brain MRI findings and to investigate the relations between MRI features and possible pathophysiological changes in children. Six children accidentally swallowed compound diphenoxylate, 4 males, 2 females, aged 20-46 months, average 33 months. Quantity of ingested diphenoxylate-atropine was from 6 to 30 tablets, each tablet contains diphenoxylate 2.5mg and atropine 0.025 mg. These patients were referred to our hospital within 24h after diphenoxylate-atropine ingestion, and underwent brain MRI scan within 24-72 h after emergency treatment. The characteristics of conventional MRI were analyzed. These pediatric patients had various symptoms of opioid intoxication and atropine toxicity. Brain MRI showed abnormal low signal intensity on T1-weighted images (T1WI) and abnormal high signal intensity on T2-weighted images (T2WI) and fluid-attenuated inversion recovery (FLAIR) imaging in bilateral in all cases; abnormal high signal intensity on T1WI, T2WI and FLAIR in 4 cases. Encephalomalacia was observed in 3 cases during follow-up. In the early stage of compound diphenoxylate poisoning in children, multiple extensive edema-necrosis and hemorrhagic-necrosis focus were observed in basic nucleus, pallium and cerebellum, these resulted in the corresponding brain dysfunction with encephalomalacia. MRI scan in the early stage in this condition may provide evidences of brain impairment, and is beneficial for the early diagnosis, treatment and prognosis assessment. Crown Copyright © 2010. Published by Elsevier Ireland Ltd. All rights reserved.
Haase, Roland; Nagel, Frank; Hirsch, Wolfgang; Sitka, Uwe
Group B Streptococcus (GBS) is a well-known cause of neonatal pneumonia, sepsis and meningitis. Peripartal antibiotic prophylaxis for early-onset GBS infection is in routine use since the beginning of the last decade, but strategies for effective prevention of late-onset GBS infections are still lacking. Few hours after discharge from a non-local maternity ward a 3-week-old boy was admitted to our hospital because of GBS meningitis with necrotizing encephalomalacia. Maternal mastitis, not a disease of the baby, had led to the first admission. Case history and negative maternal swabs and cultures for GBS led to the hypothesis of nosocomial infection. Screening and risk based peripartal antibiotic prophylaxis, better monitoring and improved therapeutic modalities have reduced the incidence and mortality of early-onset GBS infections, but peripartal prophylaxis failed to influence late-onset GBS infections. Up to 40 % of infants with late-onset meningitis develop neurological sequelae. Maternal vaccination with multivalent conjugate vaccines against GBS is a new strategy which may lead to passive protection of the infant. Further studies to examine the efficacy of vaccines are in progress.
Morris, Winston E; Goldstein, Jorge; Redondo, Leandro M; Cangelosi, Adriana; Geoghegan, Patricia; Brocco, Marcela; Loidl, Fabián C; Fernandez-Miyakawa, Mariano E
Clostridium perfringens epsilon toxin (ETX), the most potent toxin produced by this bacteria, plays a key role in the pathogenesis of enterotoxaemia in ruminants, causing brain edema and encephalomalacia. Studies of animals suffering from ETX intoxication describe severe neurological disorders that are thought to be the result of vasogenic brain edemas and indirect neuronal toxicity, killing oligodendrocytes but not astrocytes, microglia, or neurons in vitro. In this study, by means of intravenous and intracerebroventricular delivery of sub-lethal concentrations of ETX, the histological and ultrastructural changes of the brain were studied in rats and mice. Histological analysis showed degenerative changes in neurons from the cortex, hippocampus, striatum and hypothalamus. Ultrastructurally, necrotic neurons and apoptotic cells were observed in these same areas, among axons with accumulation of neurofilaments and demyelination as well as synaptic stripping. Lesions observed in the brain after sub-lethal exposure to ETX, result in permanent behavioral changes in animals surviving ETX exposure, as observed individually in several animals and assessed in the Inclined Plane Test and the Wire Hang Test. Pharmacological studies showed that dexamethasone and reserpine but not ketamine or riluzole were able to reduce the brain lesions and the lethality of ETX. Cytotoxicity was not observed upon neuronal primary cultures in vitro. Therefore, we hypothesize that ETX can affect the brain of animals independently of death, producing changes on neurons or glia as the result of complex interactions, independently of ETX-BBB interactions. Copyright © 2017 Elsevier Ltd. All rights reserved.
Comitato, Raffaella; Ambra, Roberto
Vitamin E is a generic term frequently used to group together eight different molecules, namely: α-, β-, γ- and δ-tocopherol and the corresponding tocotrienols. The term tocopherol and eventually Vitamin E and its related activity was originally based on the capacity of countering foetal re-absorption in deficient rodents or the development of encephalomalacia in chickens. In humans, Vitamin E activity is generally considered to be solely related to the antioxidant properties of the tocolic chemical structure. In recent years, several reports have shown that specific activities exist for each different tocotrienol form. In this short review, tocotrienol ability to inhibit cancer cell growth and induce apoptosis thanks to specific mechanisms, not shared by tocopherols, such as the binding to Estrogen Receptor-β (ERβ) and the triggering of endoplasmic reticulum (EndoR) stress will be described. The neuroprotective activity will also be presented and discussed. We propose that available studies strongly indicate that specific forms of tocotrienols have a distinct mechanism and biological activity, significantly different from tocopherol and more specifically from α-tocopherol. We therefore suggest not pooling them together within the broad term “Vitamin E” on solely the basis of their putative antioxidant properties. This option implies obvious consequences in the assessment of dietary Vitamin E adequacy and, probably more importantly, on the possibility of evaluating a separate biological variable, determinant in the relationship between diet and health. PMID:29156559
Han, Sang-Beom; Song, Shi-Hun; Youm, Jin-Young; Koh, Hyeon-Song; Kim, Seon-Hwan; Kwon, Hyon-Jo
Objective Chronic subdural hematoma (CSDH) is relatively common in neurosurgical field. However not all patients develop CSDH after minor head trauma. In this study, we evaluate the risk factors of post-traumatic CSDH. Methods Two-hundred and seventy-seven patients were enrolled and analyzed in this study from January 2012 to December 2013. Of those, 20 participants had minor head trauma developed CSDH afterward. We also included 257 patients with minor head trauma who did not develop CSDH during the same follow-up period as the control group. We investigated the risk factors related to the development of CSDH after minor head trauma. Results Old age (p=0.014), preexisting diabetes mellitus (p=0.010), hypertension (p=0.026), history of cerebral infarction (p=0.035), antiplatelet agents (p=0.000), acute subdural hematoma in the convexity (p=0.000), encephalomalacia (p=0.029), and long distance between skull and brain parenchyma (p=0.000) were significantly correlated with the development of CSDH after trauma. Multivariate analysis revealed that only the maximum distance between the skull and the cerebral parenchyma was the independent risk factor for the occurrence of CSDH (hazard ratio 2.55, p=0.000). Conclusion We should consider the possibility of developing CSDH in the post-traumatic patients with the identified risk factors. PMID:27169043
Miller, L D; Davis, A J; Jenny, A L; Fekadu, M; Whitfield, S G
The appearance of bovine spongiform encephalopathy (BSE) as a new disease of cattle in 1985-1987 increased worldwide interest in various aspects of human and animal spongiform encephalopathies. In the United States, a part of the surveillance effort has been directed toward prospective examination of bovine brain specimens for lesions of BSE. One focus area has been to obtain specimens from cattle that (1) are two years of age or older, (2) have documented signs of neurologic disease, and (3) have received protein supplement as a substantial part of the i.v. ration. Another focus area has been to examine rabies-suspect cases that were rabies-negative. A third area has been to obtain the results of bovine neuropathology examinations being conducted at other state and regional laboratories. Specimens have been obtained by direct submission and by referral from other public health and veterinary diagnostic laboratories. Many of the cases have been classified as having (1) inflammatory lesions such as listeriosis, pseudorabies, brain abscesses and inflammation of undetermined cause, (2) degenerative lesions such as polio-encephalomalacia, lead poisoning, Wallerian degeneration, siderocalcinosis, and lipofuscinosis, (3) neoplastic lesions such as meningioma and Schwannoma, and (4) no significant findings. Other case results were reported as inflammation or no significant findings. Of the 459 cases reported here none has contained lesions with the characteristics and distribution typical of BSE.
Garzon, Eliana; Gupta, Ajay; Bingaman, William; Sakamoto, Americo C; Lüders, Hans
Describe an ictal EEG pattern of paradoxical lateralization in children with unilateral encephaloclastic hemispheric lesion acquired early in life. Of 68 children who underwent hemispherectomy during 2003-2005, scalp video-EEG and brain MRI of six children with an ictal scalp EEG pattern discordant to the clinical and imaging data were reanalyzed. Medical charts were reviewed for clinical findings and seizure outcome. Age of seizure onset was 1 day-4 years. The destructive MRI lesion was an ischemic stroke in 2, a post-infectious encephalomalacia in 2, and a perinatal trauma and hemiconvulsive-hemiplegic syndrome in one patient each. Ictal EEG pattern was characterized by prominent ictal rhythms with either 3-7 Hz spike and wave complexes or beta frequency sharp waves (paroxysmal fast) over the unaffected (contralesional) hemisphere. Scalp video-EEG was discordant, however, other findings of motor deficits (hemiparesis; five severe, one mild), seizure semiology (4/6), interictal EEG abnormalities (3/6), and unilateral burden of MRI lesion guided the decision for hemispherectomy. After 12-39 months of post-surgery follow up, five of six patients were seizure free and one has brief staring spells. We describe a paradoxical lateralization of the EEG to the "good" hemisphere in children with unihemispheric encephaloclastic lesions. This EEG pattern is compatible with seizure free outcome after surgery, provided other clinical findings and tests are concordant with origin from the abnormal hemisphere.
Keller, Krista A; Guzman, David Sanchez-Migallon; Muthuswamy, Anantharaman; Forrest, Lisa J; Steinberg, Howard; Sladky, Kurt; Petersen, Sophie
A 37-year-old female yellow-headed Amazon parrot (Amazona ochrocephala oratrix) was presented after a 4-month-period behavior change and intermittent episodes of obtunded mentation. Clinical findings on physical examination included ataxia, a weak grasp, and reluctance to move. Results of magnetic resonance imaging were consistent with severe hydrocephalus without evidence of cerebrospinal fluid obstruction. The bird was treated with tapering dosages of prednisolone over a 4-month period, during which time the episodes did not occur. Discontinuation of treatment was attempted several times but resulted in relapse. After 3.5 years of maintenance treatment with prednisolone, the bird was presented subsequent to a 5-hour episode of obtunded mentation and worsening neurologic signs. Despite increasing the dose of prednisolone and providing additional supportive care, the bird's condition worsened, and euthanasia was elected. Necropsy findings included severe hydrocephalus with significant loss of right cerebral parenchyma and no evidence of cerebrospinal fluid obstruction. Histologic examination of the remaining cerebral parenchyma revealed a moderate, multifocal, cellular infiltrate; encephalomalacia; fibrosis; and hemosiderosis in tissue adjacent to the distended ventricles. Other findings included hepatic vacuolar degeneration. Diagnostic imaging and postmortem findings were consistent with a diagnosis of hydrocephalus ex vacuo. To our knowledge, this is the first report of hydrocephalus in an Amazon parrot as well as the first report of hydrocephalus in any avian species associated with long-term follow-up and prolonged corticosteroid treatment.
Kim, Ji Hye; Jeon, Tae Yeon; Rha, Jung Ho; Eo, Hong; Yoo, So-Young; Shu, Chang Hae
Objective We analyzed the diffusion and perfusion characteristics of acute MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episode) lesions in a large series to investigate the controversial changes of the apparent diffusion coefficient (ADC) that were reported in prior studies. Materials and Methods We analyzed 44 newly appearing lesions during 28 stroke-like episodes in 13 patients with MELAS. We performed a visual assessment of the MR images including the ADC and perfusion maps, comparison of the ADC between the normal and abnormal areas, comparison of % ADC between the 44 MELAS lesions and the 30 acute ischemic infarcts. In addition, the patterns of evolution on follow-up MR images were analyzed. Results Decreased, increased, and normal ADCs were noted in 16 (36%), 16 (36%), and 12 (27%) lesions, respectively. The mean % ADC was 102 ± 40.9% in the MELAS and 64 ± 17.8% in the acute vascular infarcts (p < 0.001), while perfusion imaging demonstrated hyper-perfusion in six acute MELAS lesions. On follow-up images, resolution, progression, and tissue loss were noted in 10, 4, and 17 lesions, respectively. Conclusion The cytotoxic edema gradually evolves following an acute stroke-like episode in patients with MELAS, and this may overlap with hyper-perfusion and vasogenic edema. The edematous swelling may be reversible or it may evolve to encephalomalacia, suggesting irreversible damage. PMID:21228936
LaDouceur, E E B; Anderson, M; Ritchie, B W; Ciembor, P; Rimoldi, G; Piazza, M; Pesti, D; Clifford, D L; Giannitti, F
Aleutian disease virus (ADV, Amdovirus, Parvoviridae) primarily infects farmed mustelids (mink and ferrets) but also other fur-bearing animals and humans. Three Aleutian disease (AD) cases have been described in captive striped skunks; however, little is known about the relevance of AD in free-ranging carnivores. This work describes the pathological findings and temporospatial distribution in 7 cases of AD in free-ranging striped skunks. All cases showed neurologic disease and were found in a 46-month period (2010-2013) within a localized geographical region in California. Lesions included multisystemic plasmacytic and lymphocytic inflammation (ie, interstitial nephritis, myocarditis, hepatitis, meningoencephalitis, pneumonia, and splenitis), glomerulonephritis, arteritis with or without fibrinoid necrosis in several organs (ie, kidney, heart, brain, and spleen), splenomegaly, ascites/hydrothorax, and/or encephalomalacia with cerebral microangiopathy. ADV infection was confirmed in all cases by specific polymerase chain reaction and/or in situ hybridization. The results suggest that AD is an emerging disease in free-ranging striped skunks in California. © The Author(s) 2014.
Almeida, Cristina; Freitas, Cristina; Sales-Sanz, Marco; Ribeiro, Sara
We report a 48-year-old female patient who presented to the emergency room with right eyelid edema, with 3 days of evolution. She had suffered minor trauma to this eye one week before. She reported episodes of right eyelid swelling of spontaneous resolution since the occurrence of a traumatic brain injury 5 years ago. Ophthalmological examination showed a soft and painless eyelid edema of the right eye. Brain computed tomography showed an area of bone discontinuity of the orbital roof with brain herniation and a CSF leak into the eyelid (blepharocele). Magnetic resonance confirmed the result of TC and revealed an area of frontal encephalomalacia. Ibuprofen (800 mg/day) was prescribed, with complete resolution within 20 days. She was evaluated by Neurosurgery with no indication of surgery due to the resolution of the edema and absence of symptoms. Blepharocele is a rare entity that should be considered in the differential diagnosis of unilateral eyelid edema. It can be secondary to an orbital fracture or congenital lesion. PMID:28848682
Bide, R W; Schofield, L; Risk, D J
There have been numerous studies of the central nervous system (CNS) involvement in organophosphate (OP) poisoning showing status epilepticus and/or 'electrographic seizures'. Brain damage has been demonstrated as 'neuronal necrosis' primarily in the cortex, thalamus and hippocampus. To the authors' knowledge there have been no reports of partial/total paralysis following close upon OP exposure although delayed paralysis has been reported. This report summarizes the immediate, OP induced paralytic events recorded in guinea pigs during development of the Canadian reactive skin decontaminant lotion (RSDL). As part of the development work, supra-lethal cutaneous doses of OP were applied to large numbers of guinea pigs followed by decontamination with the RSDL or predecessor lotions and solvents. Soman (pinacolyl methylphosphonofluoridate; GD) challenges were applied to 1277 animals and S-(2-diisopropyl-aminoethyl) methylphosphorothiolate (VX) challenges to 108. The classic sequence of clinical signs--ptyalism, tremors, fasciculations, convulsions, apnea and flaccid paralysis before death--was seen in the 658 animals that died and in many of the survivors. Eighty-four of 688 survivors of GD and 4 of 39 survivors of VX showed random paralysis of various distal regions following recovery from an insult which produced convulsions and/or flaccid paralysis. Because the experiments were designed to assess the decontamination procedures, there were no apparent relationships between the amounts of OP applied and the sequellae recorded. The observations of paralysis were also incidental to the prime focus of the experiments. Because of this, only ten animals paralysed following GD exposure were examined for histological effects. The pathologist diagnosed 'encephalomalacia' and 'focal necrotic lesions' in the cerebral cortex and 'focal necrotic lesions' in one spinal cord. Of the 84 guinea pigs paralysed after GD challenge, one was not decontaminated and the decontaminants used
Robles, M; Choi, B H; Han, B; Santa Cruz, K; Kim, R C
Russian knapweed is a perennial weed found in many parts of the world, including southern California. Chronic ingestion of this plant by horses has been reported to cause equine nigropallidal encephalomalacia (ENE), which is associated with a movement disorder simulating Parkinson's disease (PD). Repin, a principal ingredient purified from Russian knapweed, is a sesquiterpene lactone containing an alpha-methylenebutyrolactone moiety and epoxides and is a highly reactive electrophile that can readily undergo conjugation with various biological nucleophiles, such as proteins, DNA, and glutathione (GSH). We show in this study that repin is highly toxic to C57BL/6J mice and Sprague-Dawley rats and acutely induces uncoordinated locomotion associated with postural tremors, hypothermia, and inability to respond to sonic and tactile stimuli. We also show that repin intoxication reduces striatal and hippocampal GSH and increases total striatal dopamine (DA) levels in mice. Striatal microdialysis in rats, however, has demonstrated a significant reduction of extracellular DA levels. These findings, coupled with the absence of any demonstrable change in striatal DOPAC levels, suggest that repin acts by inhibiting DA release, a hypothesis that is further supported by our demonstration that, in cultured PC12 cells, repin inhibits the release of DA without affecting its uptake. We believe, therefore, that inhibition of DA release represents one of the earliest pathogenetic events in ENE, leading eventually to striatal extracellular DA denervation, oxidative stress, and degeneration of nigrostriatal pathways. Since the neurotoxic effects of repin appear to be mediated via oxidative stress, and since repin is a natural product isolated from a plant in our environment that can cause a movement disorder associated with degeneration of nigrostriatal pathways, clarification of the mechanism of repin neurotoxicity may provide new insights into our understanding of the pathogenesis of PD
Jordan, Yusef J; Lightfoote, Johnson B; Jordan, John E
To evaluate the economic impact and diagnostic utility of computed tomography (CT) in the management of emergency department (ED) patients presenting with headache and nonfocal physical examinations. Computerized medical records from 2 major community hospitals were retrospectively reviewed of patients presenting with headache over a 2.5-year period (2003-2006). A model was developed to assess test outcomes, CT result costs, and average institutional costs of the ED visit. The binomial probabilistic distribution of expected maximum cases was also calculated. Of the 5510 patient records queried, 882 (16%) met the above criteria. Two hundred eighty-one patients demonstrated positive CT findings (31.8%), but only 9 (1.02%) demonstrated clinically significant results (requiring a change in management). Most positive studies were incidental, including old infarcts, chronic ischemic changes, encephalomalacia, and sinusitis. The average cost of the head CT exam and ED visit was $764 (2006 dollars). This was approximately 3 times the cost of a routine outpatient visit (plus CT) for headache ($253). The incremental cost per clinically significant case detected in the ED was $50078. The calculated expected maximum number of clinically significant positive cases was almost 50% lower than what was actually detected. Our results indicate that emergent CT imaging of nonfocal headache yields a low percentage of positive clinically significant results, and has limited cost efficacy. Since the use of CT for imaging patients with headache in the ED is widespread, the economic implications are considerable. Health policy reforms are indicated to better direct utilization in these patients.
Tellez-Zenteno, Jose F; Sadanand, Venkatraman; Riesberry, Martha; Robinson, Christopher A; Ogieglo, Lissa; Masiowski, Paul; Vrbancic, Mirna
Epilepsy surgery is increasingly well-supported as an effective treatment for patients with intractable epilepsy. It is most often performed on younger patients and the safety and efficacy of epilepsy surgery in elderly patients are not frequently described. We report a case of a 75-year-old right-handed man who underwent a left fronto-temporal craniotomy for resection of a suprasellar meningioma in 2002. Immediately following hospital discharge, he began to experience complex partial seizures. He continued to have frequent seizures despite treatment with multiple combinations of antiepileptic medications. He presented with status epilepticus every two or three months, and required long periods of hospitalization on each occasion for post-ictal confusion and aphasia. Scalp EEG showed continuous spikes and polyspikes and persistent slowing in the left temporal area, as well as spikes in the left frontal area. EEG telemetry recorded multiple seizures, all with a clear focus in the left temporal area. MRI scan showed an area of encephalomalacia in the left temporal lobe, as well as post-surgical changes in the left frontal area. Neuropsychological testing showed bilateral memory impairment with no significant cognitive decline expected after unilateral temporal lobe resection. A left anteromesial temporal lobectomy was performed with intraoperative electrocorticography. Since surgery, the patient was not seizure-free (Engel class II-b), but had no further episodes of status epilepticus in one year and two months of follow-up. This is one of the oldest patients reported in the literature with epilepsy surgery and supports the possibility of epilepsy surgery in elderly patients for particular cases. In addition, few cases with such a malignant evolution of temporal lobe epilepsy have been described in this age group.
Diehl, B; Lüders, H O
Temporal lobe epilepsy (TLE) is the most common type of medically intractable partial epilepsy amenable to surgery. In the majority of cases, the underlying pathology in temporal lobe epilepsy is mesial temporal sclerosis (MTS). Whereas historically invasive recordings were required for most epilepsy surgeries, indications have dramatically changed since the introduction of high-resolution MRI, which uncovers structural lesions in a high percentage of cases. No invasive recordings are required to perform a temporal lobectomy in patients with intractable epilepsy who have structural imaging suggesting unilateral MTS and concordant interictal and ictal surface EEG recordings, functional imaging, and clinical findings. Invasive testing is needed if there is evidence of bitemporal MTS on structural imaging and/or electrophysiologically, and additional information from functional imaging, neuropsychology, and the intracarotid amobarbital (Wada) test also does not help to lateralize the epileptogenic zone. Depth electrodes can be particularly helpful in this setting. However, no surgery is indicated, even without invasive recordings, if bitemporal-independent seizures are recorded by surface EEG and all additional testing is inconclusive. Other etiologies of TLE such as a tumor, vascular malformation, encephalomalacia, or congenital developmental abnormality account for about 30% of all patients who undergo epilepsy surgery. Epilepsy surgery is indicated after limited electrophysiologic investigations if neuroimaging and electrophysiology converge. However, approaches for resection in lesional temporal lobe epilepsy vary among centers. Completeness of resection is crucial and invasive recordings may be needed to guide the resection by mapping eloquent cortex and/or to determine the extent of the non-MRI-visible epileptogenic area. Specific approaches for the different pathologies are discussed because there is evidence that the relationship between the lesions visible on
Anis, Anis; AboElkhair, Mohammed; Ibrahim, Mahmoud
In 2016, the highly pathogenic avian influenza (HPAI) H5N8 virus was detected in wild birds for the first time in Egypt. In the present study, we identified the HPAI virus H5N8 of clade 220.127.116.11 from domestic waterfowl in Egypt, suggesting its transmission to the domestic poultry from the migratory birds. Based on partial haemagglutinin gene sequence, this virus has a close genetic relationship with subtype H5N8 viruses circulating in Asia and Europe. Pathologically, H5N8 virus in hybrid duck induced nervous signs accompanied by encephalomalacia, haemorrhages, nonsuppurative encephalitis and nonsuppurative vasculitis. The granular layer of cerebellum showed multifocal areas of hydropic degeneration and the Purkinje cell neurons were necrotized or lost. Additionally, the lung, kidney and spleen were congested, and necrotizing pancreatitis was also observed. The co-circulation of both HPAI H5N1 and H5N8 subtypes with the low pathogenic avian influenza H9N2 subtype complicate the control of avian influenza in Egypt with the possibility of emergence of new reassortant viruses. Therefore, continuous monitoring with implementation of strict control measures is required. Research highlights HPAI H5N8 virus clade 18.104.22.168 was detected in domestic ducks and geese in Egypt in 2017. Phylogenetically, the virus was closely related to HPAI H5N8 viruses identified in Asia and Europe Nonsuppurative encephalitis was widely observed in HPAI H5N8 virus-infected ducks. Degeneration of the cerebellar granular layer was found in most of the brain tissues examined.
Bao, Xuhui; Chandramohan, Vidyalakshmi; Reynolds, Randall P; Norton, John N; Wetsel, William C; Rodriguiz, Ramona M; Aryal, Dipendra K; McLendon, Roger E; Levin, Edward D; Petry, Neil A; Zalutsky, Michael R; Burnett, Bruce K; Kuan, Chien-Tsun; Pastan, Ira H; Bigner, Darell D
D2C7-(scdsFv)-PE38KDEL (D2C7-IT) is a novel immunotoxin that reacts with wild-type epidermal growth factor receptor (EGFRwt) and mutant EGFRvIII proteins overexpressed in glioblastomas. This study assessed the toxicity of intracerebral administration of D2C7-IT to support an initial Food and Drug Administration Investigational New Drug application. After the optimization of the formulation and administration, two cohorts (an acute and chronic cohort necropsied on study days 5 and 34) of Sprague-Dawley (SD) rats (four groups of 5 males and 5 females) were infused with the D2C7-IT formulation at total doses of 0, 0.05, 0.1, 0.4 μg (the acute cohort) and 0, 0.05, 0.1, 0.35 μg (the chronic cohort) for approximately 72 h by intracerebral convection-enhanced delivery using osmotic pumps. Mortality was observed in the 0.40 μg (5/10 rats) and 0.35 μg (4/10 rats) high-dose groups of each cohort. Body weight loss and abnormal behavior were only revealed in the rats treated with high doses of D2C7-IT. No dose-related effects were observed in clinical laboratory tests in either cohort. A gross pathologic examination of systemic tissues from the high-dose and control groups in both cohorts exhibited no dose-related or drug-related pathologic findings. Brain histopathology revealed the frequent occurrence of dose-related encephalomalacia, edema, and demyelination in the high-dose groups of both cohorts. In this study, the maximum tolerated dose of D2C7-IT was determined to be between 0.10 and 0.35 μg, and the no-observed-adverse-effect-level was 0.05 μg in SD rats. Both parameters were utilized to design the Phase I/II D2C7-IT clinical trial.
Liu, Kun; Ma, Yan-xu; Zhang, Cheng-bing; Chen, Yi-ping; Ye, Xin-jian; Bai, Guang-hui; Yu, Zhi-kang; Yan, Zhi-han
To explore the clinical and magnetic resonance imaging (MRI) characteristics and the follow-up outcomes of neurologic complications in children with enterovirus 71-infected hand-foot-mouth disease. The clinical and MRI manifestations and follow-up outcomes in 35 children, at Second Affiliated Hospital, Wenzhou Medical College from August 2008 to November 2010, hospitalized with neurologic complications of enterovirus 71-infected hand-foot-mouth disease were retrospectively analyzed. Six children with aseptic meningitis presented the clinical symptoms and signs of meningitis. Five of them showed subdural effusion and ventriculomegaly, or both on MRI. At follow-ups, neurologic sequel could not be found. Among 24 cases with brainstem encephalitis, there were myoclonic jerks and tremor, ataxia, or both (grade I disease, n = 12), myoclonus and cranial-nerve involvement (grade II disease, n = 4), and cardiopulmonary failure after brain-stem infection (grade III disease, n = 8). In patients with brainstem encephalitis, lesions were predominantly located at the posterior portions of medulla and pons with hypointensity on T1WI and hyperintensity on T2WI. Cerebellar dentate nucleus, caudate nucleus and lenticular nucleus could also be involved. At follow-ups, the patients with mild symptoms had no neurologic sequel and the lesions within brain stem became small or vanished in most cases. While in the majority of serious patients, neurologic sequel could be found and the lesions located at brain stem became encephalomalacia. Fourteen cases with acute flaccid paralysis presented acute limb myasthenia with tendon reflex and muscular tension decreased. On spinal MRI, the lesions predominantly involved anterior horn regions of spinal cord with hypointensity on T1WI and hyperintensity on T2WI. Most patients improved their muscle strength and most lesions of spinal cord became smaller or vanished during follow-ups. MRI is the most effective modality of diagnosis and follow-up for
Xue, Jiao; Yang, Zhixian; Wu, Ye; Xiong, Hui; Zhang, Yuehua; Liu, Xiaoyan
To analyze clinical characteristics, treatment and prognosis in a cohort of children with vitamin B6 responsive infantile spasms. Ten patients were diagnosed as vitamin B6 responsive infantile spasms in Peking University First Hospital between January 2012 and May 2015.The clinical manifestations, diagnosis and treatment process, video-electroencephalogram, magnetic resonance imaging (MRI), epilepsy related genes and prognosis were retrospectively analyzed. Of the 10 patients, 5 were male, and 5 were female. Eight of them were normal at birth, and the other 2 patients had intracranial hemorrhage or anoxia.The age of epilepsy onset was from 3.5 to 8.0 months.All patients presented spasms primarily.Interictal electroencephalogram (EEG) showed hypsarrhythmia at seizures onset. MRI showed normal in 8 patients, and subarachnoid hemorrhage or multiple encephalomalacia foci after hemorrhage respectively in the other 2 patients. The results of blood biochemical, cerebrospinal fluid examination and urinary metabolic screening were negative. Epilepsy related genes including ALDH7A1 gene analysis showed wild type in all patients. Two patients were classified as symptomatic and eight might be idiopathic or cryptogenic. The initial dose of vitamin B6 was 10.0 mg/(kg·d). The interval between seizures onset and taking vitamin B6 was 0 to 4.0 months. Seizures disappeared completely within a week after administration of vitamin B6 in 9 patients and in 1.5 months in one patient.Of the 8 patients whose seizures were controlled completely during the follow-up period, 7 patients' EEG recovered within 1.5 to 4.0 months and then continued to be normal. The EEG of the rest of a patient returned to normal, but showed abnormal discharges after stopping taking vitamin B6. Two patients' EEG continued abnormal and seizures recurred due to vitamin B6 withdrawal. At the last follow-up, seizures were controlled in all patients. Drug treatment in one case had stopped. Vitamin B6 was used in 9