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neurology case report

Sandeep Kandregula, Amey R Savardekar, B N Nandeesh, A Arivazhagan, Malla Bhaskar Rao
Giant hypothalamic hamartomas (GHH) are extremely rare lesions in infants and usually intrinsically epileptogenic. We present the case of a 10-month-old girl child presenting with drug-resistant seizures and a giant hypothalamic lesion that was confirmed as hamartoma on histopathology. Surgical decompression and disconnection from the hypothalamus was performed with the intent of controlling her seizures. Unfortunately, the patient developed right middle cerebral artery and posterior cerebral artery territory infarction, possibly due to vasospasm or thrombosis of the vessels...
October 26, 2016: Pediatric Neurosurgery
Miriam Friedemann
In November 2012, 23 cases of ciguatera with typical combinations of gastrointestinal and neurological symptoms occurred in Germany after consumption of imported tropical fish (Lutjanus spp.). A questionnaire was used to gather information on the disease course and fish consumption. All patients suffered from pathognomonic cold allodynia. Aside from two severe courses of illness, all other cases showed symptoms of moderate intensity. During a three-year follow-up, seven patients reported prolonged paresthesia for more than one year...
October 24, 2016: Bundesgesundheitsblatt, Gesundheitsforschung, Gesundheitsschutz
Farahnaz Golriz, Lane F Donnelly, Sridevi Devaraj, Raj Krishnamurthy
BACKGROUND: Until recently scurvy has been viewed in developed countries as a disease of the past. More recently there have been reports of case series of children with scurvy who have had a delayed diagnosis after an extensive diagnostic workup that included imaging. Most of these children have had underlying neurologic conditions such as autism. OBJECTIVE: To review the medical records of children diagnosed with vitamin C (ascorbic acid) deficiency based on serum ascorbic acid levels at a large pediatric health care system, to determine imaging findings and utility of imaging in management, and to identify at-risk pediatric populations...
October 24, 2016: Pediatric Radiology
Anna Nolte, Aimara Bello, Michaela Drögemüller, Tosso Leeb, Eva Brockhaus, Wolfgang Baumgärtner, Peter Wohlsein
A female, 5-year-old American Staffordshire Terrier with severe progressive neurological deficits, particularly in terms of ataxia and keeping balance, was examined pathomorphologically and a genetic analysis was performed. In neurons of various localizations of the central nervous system an accumulation of a finely granular pale eosinophilic or light brown material was found. In addition, the cerebellum revealed marked degeneration and loss of Purkinje and inner granule cells. The accumulated PAS-positive, argyrophilic, autofluorescent material showed ultrastructurally a lamellar appearance suggestive of lipofuscin...
October 25, 2016: Tierärztliche Praxis. Ausgabe K, Kleintiere/Heimtiere
Yasutaka Tajima, Mariko Matsumura, Hiroaki Yaguchi, Yasunori Mito
In rare instances, recipients of organ transplants from human T-lymphotropic virus type I- (HTLV-I-) positive donors reportedly developed neurologic symptoms due to HTLV-I-associated myelopathy (HAM). We present herein two cases of HAM associated with renal transplantation from HTLV-I seropositive living-donors. The first patient was a 42-year-old woman with chronic renal failure for twelve years and seronegative for HTLV-I. She underwent renal transplantation with her HTLV-I seropositive mother as the donor, and she developed HAM three years after the transplantation...
2016: Case Reports in Neurological Medicine
Emna Farhat, Mourad Zouari, Ines Ben Abdelaziz, Cyrine Drissi, Rahma Beyrouti, Mohamed Ben Hammouda, Fayçal Hentati
BACKGROUND: Cerebellar ataxia represents a rare and severe complication of Sjӧgren syndrome (SS), especially with a progressive onset and cerebellar atrophy on imaging. CASE PRESENTATION: We report the case of a 30-year-old woman, with a past history of dry eyes and mouth, who presented a severe cerebellar ataxia worsening over 4 years associated with tremor of the limbs and the head. Brain MRI showed bilateral hyperintensities on T2 and FLAIR sequences, affecting periventricular white matter, with marked cerebellar atrophy...
2016: Cerebellum & Ataxias
Bobby Yanagawa, Gosta B Pettersson, Gilbert Habib, Marc Ruel, Gustavo Saposnik, David A Latter, Subodh Verma
There has been an overall improvement in surgical mortality for patients with infective endocarditis (IE), presumably because of improved diagnosis and management, centered around a more aggressive early surgical approach. Surgery is currently performed in approximately half of all cases of IE. Improved survival in surgery-treated patients is correlated with a reduction in heart failure and the prevention of embolic sequelae. It is reported that between 20% and 40% of patients with IE present with stroke or other neurological conditions...
October 25, 2016: Circulation
Kuan-Pin Chen, Cheng-Chia Lee, Chia-Lin Liao, Tao-Chieh Yang, Tsung-Lang Chiu, Chain-Fa Su
In this report, we present the results of using upfront gamma knife surgery (GKS) in the management of giant central neurocytoma (volume > 50 ml) without the initial removal of the tumor mass. Two patients underwent GKS for histologically proven central neurocytoma (CNC). Clinical and imaging studies were performed to evaluate the response to treatment. GKS involved delivery doses of 12 or 13 Gy to the tumor margin at the isodose line of 50%. Tumor response to GKS appeared as early as 4-6 months post-GKS, at which point a dramatic reduction in volume was observed...
October 21, 2016: World Neurosurgery
Dorota Tassigny, Basel Abu-Serieh, Daniella Tsague Fofe, Jacques Born, Germain Milbouw
BACKGROUND AND IMPORTANCE: Syringomyelia is a progressive cerebrospinal fluid disorder which can lead to irreversible spinal cord injury. To date, the optimal management of syringomyelic cavities remains controversial. Multiple studies have emphasized the importance of the cranio-cervical decompression or shunting procedures. However, excepted for syrinx related to Arnold Chiari malformation, nearly half of patients need to be re-operated. The purpose of the present study was to describe a simple and efficient surgical technique and to report the long-term radio-clinical outcomes...
October 21, 2016: World Neurosurgery
Vanessa E C Pourier, Gert J de Borst
OBJECTIVE: This review summarizes the available evidence and analyzes the current trends on treatments for carotid in-stent restenosis (ISR) after carotid artery stenting (CAS). METHODS: An update of a 2010 review of the literature (which included 20 articles) was conducted using PubMed and Embase. All studies published from inception until January 2016 reporting original data on ISR treatments were included. Treatment trends before and after 2005 were compared...
November 2016: Journal of Vascular Surgery
Hirofumi Kato, Takuya Yamagishi, Tomoe Shimada, Tamano Matsui, Masayuki Shimojima, Masayuki Saijo, Kazunori Oishi
Although severe fever with thrombocytopenia syndrome (SFTS) was first reported from Japan in 2013, the precise clinical features and the risk factors for SFTS have not been fully investigated in Japan. Ninety-six cases of severe fever with thrombocytopenia syndrome (SFTS) were notified through the national surveillance system between April 2013 and September 2014 in Japan. All cases were from western Japan, and 82 cases (85%) had an onset between April and August. A retrospective observational study of the notified SFTS cases was conducted to identify the clinical features and laboratory findings during the same period...
2016: PloS One
Bella H V Nguyen, James Kuo, Anadian Budiman, Hayden Christie, Sayed Ali
Immune checkpoint inhibitors have changed the landscape of the treatment of multiple solid malignancies, and have been used increasingly in the recent years. Although usually well tolerated, given the relative inexperience of using immune checkpoint inhibitors, we are still learning of new side effects from the treatment. We report on two cases of ocular myasthenia gravis that occurred after treatment with pembrolizumab, an antiprogrammed-death (anti-PD1) monoclonal antibody for advanced melanoma in responding patients...
October 21, 2016: Melanoma Research
Haruki Tokida, Yuhei Kanaya, Yutaka Shimoe, Shigeru Yamori, Koichi Tagawa, Masaru Kuriyama
We report a case of a 55-year-old man who developed acute-onset narrowing of his visual field. He showed right homonymous hemianopsia without any other neurological symptoms and signs. Brain CT and MRI showed localized hemorrhage (about 1.6 ml) in the left lateral geniculate body (LGB). A cerebral angiography showed no vascular anomalies of cerebral vessels, and the left anterior choroidal artery and left lateral posterior choroidal artery could be visualized well. He had hypertension, polycythemia and dyslipidemia and was a habitual smoker and an alcoholic...
October 21, 2016: Rinshō Shinkeigaku, Clinical Neurology
Koichi Wakabayashi, Fumiaki Mori, Akiyoshi Kakita, Hitoshi Takahashi, Shinya Tanaka, Jun Utsumi, Hidenao Sasaki
MicroRNAs (miRNAs) are small noncoding RNAs that regulate gene expression. Recently, we have shown that informative miRNA data can be derived from archived formalin-fixed paraffin-embedded (FFPE) samples from postmortem cases of amyotrophic lateral sclerosis and normal controls. miRNA analysis has now been performed on FFPE samples from affected brain regions in patients with multiple system atrophy (MSA) and the same areas in neurologically normal controls. We evaluated 50 samples from patients with MSA (n=13) and controls (n=13)...
October 20, 2016: Neuroscience Letters
Aditi Vian Varma, Lori McBride, Michael Marble, Ann Tilton
Congenital insensitivity to pain and anhidrosis (CIPA) is one of the hereditary autonomic and sensory neuropathies. Typically presenting in infancy, it manifests as hyperpyrexia from defects in sweating (autonomic) and self-mutilating injuries from pain insensitivity (sensory). CIPA being rare in North America, diagnosis is often missed due to variable presentation. Subsequent management of its complications is therefore delayed. We report an unusual presentation in a 2-year-old girl with preexisting diagnosis of CIPA who was evaluated for bilateral upper extremity paresis of insidious onset...
November 15, 2016: Journal of the Neurological Sciences
Miao Wang, Meichen Zhang, Lei Wu, Zhao Dong, Shengyuan Yu
BACKGROUND: Leukoencephalopathy with calcifications and cysts (LCC) is a rare disease in which parenchymal cysts and calcifications within a widespread leukoencephalopathy can cause a broad spectrum of neurological symptoms. We present cases with adult LCC and discuss previously described entities in relevant literature. CASE PRESENTATION: Two cases of adult-onset LCC confirmed by clinical presentations, typical neuroimaging and neuropathological findings are reported...
November 15, 2016: Journal of the Neurological Sciences
J Lagarde, M Sarazin
Frontotemporal degeneration (FTD) in its behavioral variant (bvFTD) is probably one of the conditions that best illustrates the links between psychiatry and neurology. It is indeed admitted that between a third and half of patients with this condition, especially in early-onset forms, receive an initial diagnosis of psychiatric disorder (depression, schizophrenia, bipolar disorder) and are then referred to a psychiatric ward. BvFTD can thus be considered a neurological disorder with a psychiatric presentation...
October 20, 2016: L'Encéphale
Matthias Karrasch, Elisabeth Fischer, Martin Scholten, Andreas Sauerbrei, Andreas Henke, Diane M Renz, Hans-Joachim Mentzel, Klas Böer, Sindy Böttcher, Sabine Diedrich, Andi Krumbholz, Roland Zell
Infection by Enterovirus A71 (EV-A71) is an important cause of hand, foot, and mouth disease (HFMD). Outbreaks including severe cases with neurological and cardiopulmonary complications have been reported particularly from Southeast Asia. In Europe, the epidemiology of EV-A71 is not well understood. In summer 2015, a two-year-old girl from Thuringia, Germany, presented with rhombencephalitis/brainstem encephalitis associated with severe neurological and cardiopulmonary complications. EV-A71 was detected in stool and almost the entire viral genome was amplified and sequenced...
October 19, 2016: Journal of Clinical Virology: the Official Publication of the Pan American Society for Clinical Virology
Moushira E Zaki, Hala T El-Bassyouni, Angie M S Tosson, Eman Youness, Jihan Hussein
OBJECTIVE: Evidence of oxidative stress was reported in individuals with Down syndrome. There is a growing interest in the contribution of the immune system in Down syndrome. The aim of this study is to evaluate the coenzyme Q10 (CoQ10) and selected pro-inflammatory markers such as interleukin 6 (IL-6) and tumor necrosis factor α (TNFα) in children with Down syndrome. METHODS: Eighty-six children (5-8 years of age) were enrolled in this case-control study from two public institutions...
October 19, 2016: Jornal de Pediatria
Sofia Morfopoulou, Edward T Mee, Sarah M Connaughton, Julianne R Brown, Kimberly Gilmour, W K 'Kling' Chong, W Paul Duprex, Deborah Ferguson, Mike Hubank, Ciaran Hutchinson, Marios Kaliakatsos, Stephen McQuaid, Simon Paine, Vincent Plagnol, Christopher Ruis, Alex Virasami, Hong Zhan, Thomas S Jacques, Silke Schepelmann, Waseem Qasim, Judith Breuer
Routine childhood vaccination against measles, mumps and rubella has virtually abolished virus-related morbidity and mortality. Notwithstanding this, we describe here devastating neurological complications associated with the detection of live-attenuated mumps virus Jeryl Lynn (MuV(JL5)) in the brain of a child who had undergone successful allogeneic transplantation for severe combined immunodeficiency (SCID). This is the first confirmed report of MuV(JL5) associated with chronic encephalitis and highlights the need to exclude immunodeficient individuals from immunisation with live-attenuated vaccines...
October 21, 2016: Acta Neuropathologica
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