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Rasmussen's encephalitis

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https://www.readbyqxmd.com/read/28303446/expression-of-human-cytomegalovirus-components-in-the-brain-tissues-of-patients-with-rasmussen-s-encephalitis
#1
Yao Zhang, Yisong Wang, Sichang Chen, Shuai Chen, Yuguang Guan, Changqing Liu, Tianfu Li, Guoming Luan, Jing An
Rasmussen's encephalitis (RE) is a rare and severe progressive epileptic syndrome with unknown etiology. Infection by viruses, including human cytomegalovirus (HCMV), has been speculated to be a potential trigger for RE. However, no viral antigens have been detected in the brains of patients with RE; thus, a possible clinical linkage between viral infections and RE has not been firmly established. In this study, we evaluated the expression of HCMV pp65 antigen in brain sections from 26 patients with RE and 20 non-RE patients by immunohistochemistry and in situ hybridization, and assessed the associations between HCMV infection and clinical parameters...
March 2, 2017: Virologica Sinica
https://www.readbyqxmd.com/read/28256379/outcome-of-childhood-onset-epilepsy-from-adolescence-to-adulthood-transition-issues
#2
REVIEW
R Nabbout, D M Andrade, N Bahi-Buisson, H Cross, I Desquerre, O Dulac, T Granata, E Hirsch, V Navarro, L Ouss, P L Pearl, D Schmidt, E Thiele, P R Camfield, C S Camfield
This is the second of three papers that summarize the second symposium on Transition in Epilepsies held in Paris in June 2016. This paper addresses the outcome for some particularly challenging childhood-onset epileptic disorders with the goal of recommending the best approach to transition. We have grouped these disorders in five categories with a few examples for each. The first group includes disorders presenting in childhood that may have late- or adult-onset epilepsy (metabolic and mitochondrial disorders)...
February 27, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28245617/the-diverse-roles-of-microglia-in-the-neurodegenerative-aspects-of-central-nervous-system-cns-autoimmunity
#3
REVIEW
Kaitlyn K Thompson, Stella E Tsirka
Autoimmune diseases of the central nervous system (CNS) involve inflammatory components and result in neurodegenerative processes. Microglia, the resident macrophages of the CNS, are the first responders after insults to the CNS and comprise a major link between the inflammation and neurodegeneration. Here, we will focus on the roles of microglia in two autoimmune diseases: the prevalent condition of multiple sclerosis (MS) and the much rarer Rasmussen's encephalitis (RE). Although there is an abundance of evidence that microglia actively contribute to neuronal damage in pathological states such as MS and RE, there is also evidence of important reparative functions...
February 25, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28105459/mog-antibody-positive-benign-unilateral-cerebral-cortical-encephalitis-with-epilepsy
#4
Ryo Ogawa, Ichiro Nakashima, Toshiyuki Takahashi, Kimihiko Kaneko, Tetsuya Akaishi, Yoshiki Takai, Douglas Kazutoshi Sato, Shuhei Nishiyama, Tatsuro Misu, Hiroshi Kuroda, Masashi Aoki, Kazuo Fujihara
OBJECTIVE: To describe the features of adult patients with benign, unilateral cerebral cortical encephalitis positive for the myelin oligodendrocyte glycoprotein (MOG) antibody. METHODS: In this retrospective, cross-sectional study, after we encountered an index case of MOG antibody-positive unilateral cortical encephalitis with epileptic seizure, we tested for MOG antibody using our in-house, cell-based assay in a cohort of 24 consecutive adult patients with steroid-responsive encephalitis of unknown etiology seen at Tohoku University Hospital (2008-2014)...
March 2017: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/28056425/fcd-type-ii-and-mtor-pathway-evidence-for-different-mechanisms-involved-in-the-pathogenesis-of-dysmorphic-neurons
#5
Laura Rossini, Flavio Villani, Tiziana Granata, Laura Tassi, Giovanni Tringali, Francesco Cardinale, Eleonora Aronica, Roberto Spreafico, Rita Garbelli
Type II focal cortical dysplasia (FCD II) is a malformation of cortical development, frequently associated with intractable epilepsy, characterised by cortical dyslamination, dysmorphic neurons (DNs) and balloon cells (BCs). We investigated the expression of pS6 (downstream target) and pPDK1-pAkt (upstream targets) as evidence for mTOR pathway activation and their co-expression with Interleukin-1β in FCD II surgical specimens and compared the findings with control non-epileptic tissue, non-malformed epileptic tissue or acquired epilepsy-Rasmussen's Encephalitis (RE) occasionally presenting pS6 and Interleukin-1β positive abnormal neurons...
January 2017: Epilepsy Research
https://www.readbyqxmd.com/read/28042372/rasmussen-encephalitis
#6
G Abbas Kharal, Sashank Prasad
No abstract text is available yet for this article.
January 2017: Neurohospitalist
https://www.readbyqxmd.com/read/28029552/epilepsia-partialis-continua-a-review
#7
REVIEW
Rūta Mameniškienė, Peter Wolf
Epilepsia partialis contina (EPC) in a narrow definition is a variant of simple focal motor status epilepticus in which frequent repetitive muscle jerks, usually arrhythmic, continue over prolonged periods of time. In a broader definition (used in this review) it also includes non-motor manifestations otherwise known as aura continua. EPC may occur as a single episode, repetitive episodes, it may be chronic progressive or non-progressive. It appears as an unusual manifestation of epilepsy in which more typical paroxysmal events are partly or entirely replaced by the sustained repetition of seizure fragments in rapid succession...
January 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/27913085/anti-n-methyl-d-aspartate-receptor-encephalitis-and-rasmussen-like-syndrome-an-association
#8
Kevin Gurcharran, Shefali Karkare
BACKGROUND: N-methyl-D-aspartate (NMDA) receptor encephalitis is an immune-mediated condition that has a broad spectrum of manifestations, including seizures, coma, psychosis, and focal neurological deficits. Although usually a diffuse process, unihemispheric involvement mimicking early stages of Rasmussen encephalitis can occur. Rasmussen's encephalitis is a unique syndrome characterized by progressive hemiplegia, drug-resistant focal epilepsy, cognitive decline, and hemispheric brain atrophy contralateral to the hemiplegia...
January 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/27900773/systematic-review-of-immunoglobulin-use-in-paediatric-neurological-and-neurodevelopmental-disorders
#9
REVIEW
Jonathan Gadian, Emma Kirk, Kate Holliday, Ming Lim, Michael Absoud
AIM: A systematic literature review of intravenous immunoglobulin (IVIG) treatment of paediatric neurological conditions was performed to summarize the evidence, provide recommendations, and suggest future research. METHOD: A MEDLINE search for articles reporting on IVIG treatment of paediatric neuroinflammatory, neurodevelopmental, and neurodegenerative conditions published before September 2015, excluding single case reports and those not in English. Owing to heterogeneous outcome measures, meta-analysis was not possible...
February 2017: Developmental Medicine and Child Neurology
https://www.readbyqxmd.com/read/27899037/a-comparison-of-hmgb1-concentrations-between-cerebrospinal-fluid-and-blood-in-patients-with-neurological-disease
#10
Lauren Elizabeth Walker, Michael John Griffiths, Fiona McGill, Penelope Lewthwaite, Graeme John Sills, Andrea Jorgensen, Daniel James Antoine, Tom Solomon, Anthony Guy Marson, Munir Pirmohamed
AIMS: To determine whether a correlation exists between paired cerebrospinal fluid (CSF) and serum levels of a novel inflammatory biomarker, high-mobility group box 1 (HMGB1), in different neurological conditions. METHODS: HMGB1 was measured in the serum and CSF of 46 neurological patients (18 idiopathic intracranial hypertension [IIH], 18 neurological infection/inflammation [NII] and 10 Rasmussen's encephalitis [RE]). RESULTS: Mean serum (± SD) HMGB1 levels were 1...
December 8, 2016: Biomarkers: Biochemical Indicators of Exposure, Response, and Susceptibility to Chemicals
https://www.readbyqxmd.com/read/27771212/adult-onset-rasmussen-s-encephalitis-associated-with-reflex-language-induced-seizures-responsive-to-rituximab-therapy
#11
Salwa El Tawil, Robin Morris, Nandini Mullatti, Lina Nashef, Sanjeev Rajakulendran
No abstract text is available yet for this article.
November 2016: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/27728538/a-rare-presentation-of-rasmussen-s-encephalitis-in-an-adult-female
#12
Shaik Razia, Dilip M Rampure, I B Ganiger
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27618602/long-term-effect-of-rituximab-in-a-case-with-late-onset-rasmussen%C3%A2-s-encephalitis-with-anti-ganglioside-iggq1b-and-anti-gad-antibodies-positivity-case-report
#13
Gabriela Timarova, Iveta Lisa, Peter Kukumberg
Rasmussen's encephalitis is a rare autoimmune encephalitis usually involving one brain hemisphere, presenting with refractory epileptic seizures, and neurological and cognitive decline. Only 10% of cases start later in adolescence/adulthood. The only effective treatment for refractory seizures in childhood is hemispherectomy. For late-onset cases with mild neurological deficit the hemispherectomy is usually postponed because of its severe consequences. Immunotherapy shows some temporal effect for seizure control and slowing the brain atrophy, mainly in late onset Rasmussen's encephalitis...
July 2016: Neuro Endocrinology Letters
https://www.readbyqxmd.com/read/27609620/automated-mri-volumetric-analysis-in-patients-with-rasmussen-syndrome
#14
Z I Wang, B Krishnan, D W Shattuck, R M Leahy, A N V Moosa, E Wyllie, R C Burgess, N B Al-Sharif, A A Joshi, A V Alexopoulos, J C Mosher, U Udayasankar, S E Jones
BACKGROUND AND PURPOSE: Rasmussen syndrome, also known as Rasmussen encephalitis, is typically associated with volume loss of the affected hemisphere of the brain. Our aim was to apply automated quantitative volumetric MR imaging analyses to patients diagnosed with Rasmussen encephalitis, to determine the predictive value of lobar volumetric measures and to assess regional atrophy differences as well as monitor disease progression by using these measures. MATERIALS AND METHODS: Nineteen patients (42 scans) with diagnosed Rasmussen encephalitis were studied...
December 2016: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/27597560/teaching-neuroimages-radiographic-progression-in-late-onset-rasmussen-encephalitis
#15
Carlos Leiva-Salinas, Mark Quigg
No abstract text is available yet for this article.
September 6, 2016: Neurology
https://www.readbyqxmd.com/read/27490897/elevated-expression-of-human-papillomavirus-antigen-in-brain-tissue-of-patients-with-rasmussen-s-encephalitis
#16
Shuai Chen, Sichang Chen, Yuguang Guan, Yao Zhang, Xueling Qi, Jing An, Yisong Wang, Guoming Luan
OBJECTS: To investigate the expression of human papillomavirus (HPV)-specific antigen in the brain tissue of patients with Rasmussen's Encephalitis (RE) and its possible link to the clinical manifestation of RE. METHODS: The correlation between RE and HPV antigen expression in brain tissue sections was investigated using immunohistochemical (IHC) staining, pathological examination, MRI and clinical manifestations. RESULTS: HPV antigen expression was elevated in three out of four patients with RE, whereas there were no detectable HPV antigens in six control patients...
October 2016: Epilepsy Research
https://www.readbyqxmd.com/read/27475280/ketogenic-diet-treatment-for-pediatric-super-refractory-status-epilepticus
#17
Brian Appavu, Lisa Vanatta, John Condie, John F Kerrigan, Randa Jarrar
PURPOSE: We aimed to study whether ketogenic diet (KD) therapy leads to resolution of super-refractory status epilepticus in pediatric patients without significant harm. METHOD: A retrospective review was performed at Phoenix Children's Hospital on patients with super-refractory status epilepticus undergoing ketogenic diet therapy from 2011 to 2015. RESULTS: Ten children with super-refractory status epilepticus, ages 2-16 years, were identified...
October 2016: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/27397698/evidence-for-resident-memory-t-cells-in-rasmussen-encephalitis
#18
COMMENT
Amer Khojah, Marisa Klein-Gitelman
Investigators from University of California Los Angeles studied the presence of different T cell subset population in the brain tissue of 7 patients with Rasmussen encephalitis, a rare neuroinflammatory disorder characterized by intractable seizures and usually associated with progressive hemi cerebral atrophy, who underwent brain surgery and compared them to patients with focal cortical dysplasia.
March 2016: Pediatric neurology briefs
https://www.readbyqxmd.com/read/27296534/-hemispherectomy-in-the-treatment-of-pediatric-symptomatic-epilepsy-of-children
#19
A G Melikyan, Yu V Kushel', A N Vorob'ev, N A Arkhipova, V S Sorokin, N V Lemeneva, I A Savin, I N Pronin, A B Kozlova, O A Grinenko, S B Buklina, I A Nagorskaya
INTRODUCTION AND PURPOSE: Hemispherectomy is a recognized option in the treatment of symptomatic forms of intractable focal epilepsy in patients with developmental brain malformations and some acquired lesions of one the hemispheres. The prognosis for an outcome of the technique is important in terms of the indications for surgical treatment. MATERIAL AND METHODS: We described the hemispherectomy technique and its variants and analyzed our own experience of surgery in 40 children...
2016: Zhurnal Voprosy Neĭrokhirurgii Imeni N. N. Burdenko
https://www.readbyqxmd.com/read/27248684/neuronal-autoantibodies-in-patients-with-rasmussen-s-encephalitis
#20
Bedia Samanci, Pınar Tektürk, Erdem Tüzün, Ece Erdağ, Demet Kınay, Zuhal Yapıcı, Betül Baykan
Rasmussen's encephalitis (RE) is a rare disease with unknown pathophysiology. To disclose whether anti-neuronal autoimmunity participates in the aetiology of RE, various neuronal autoantibodies (NAAbs) were investigated in sera of patients with RE and controls. The study included five patients who fulfilled the RE diagnostic criteria (clinical, EEG, and MRI findings) as the patient group, and 50 multiple sclerosis patients and 50 healthy subjects as the control groups. Sera were evaluated for various NAAbs by radioimmunoassay or cell-based assays...
June 1, 2016: Epileptic Disorders: International Epilepsy Journal with Videotape
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