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Rasmussen's encephalitis

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https://www.readbyqxmd.com/read/27913085/anti-n-methyl-d-aspartate-receptor-encephalitis-and-rasmussen-like-syndrome-an-association
#1
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...
October 11, 2016: Pediatric Neurology
https://www.readbyqxmd.com/read/27900773/systematic-review-of-immunoglobulin-use-in-paediatric-neurological-and-neurodevelopmental-disorders
#2
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...
November 30, 2016: 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
#3
Lauren Elizabeth Walker, Michael 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, 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...
November 30, 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
#4
Salwa El Tawil, Robin Morris, Nandini Mullatti, Lina Nashef, Sanjeev Rajakulendran
No abstract text is available yet for this article.
September 28, 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
#5
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
#6
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 16, 2016: Neuro Endocrinology Letters
https://www.readbyqxmd.com/read/27609620/automated-mri-volumetric-analysis-in-patients-with-rasmussen-syndrome
#7
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...
September 8, 2016: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/27597560/teaching-neuroimages-radiographic-progression-in-late-onset-rasmussen-encephalitis
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
https://www.readbyqxmd.com/read/27242065/intravenous-immunoglobulin-in-paediatric-neurology-safety-adherence-to-guidelines-and-long-term-outcome
#14
Margherita Nosadini, Shekeeb S Mohammad, Agnese Suppiej, Stefano Sartori, Russell C Dale
AIM: Intravenous immunoglobulin (IVIG) is an expensive therapy used in immunodeficiency and autoimmune disorders. Increasing demands and consequent shortages result in a need for usage to conform to guidelines. METHOD: We retrospectively evaluated IVIG use for neuroimmunological indications and adherence to existing guidelines in a major Australian paediatric hospital between 2000 and 2014. RESULTS: One-hundred and ninety-six children (96 male, 100 female; mean age at disease onset 6y 5mo [range 3mo-15y 10mo], mean age at first IVIG dose 7y 2mo [range 3mo-16y 5mo]) received IVIG for neuroimmunological indications during the study period (28...
May 31, 2016: Developmental Medicine and Child Neurology
https://www.readbyqxmd.com/read/27108105/upregulation-of-hmgb1-toll-like-receptor-and-rage-in-human-rasmussen-s-encephalitis
#15
Guoming Luan, Qing Gao, Feng Zhai, Yin Chen, Tianfu Li
Rasmussen encephalitis (RE) is a rare neurological disorder of childhood characterized by uni-hemispheric inflammation, progressive neurological deficits and intractable focal epilepsy. The pathogenesis of RE is still enigmatic. Activation of endogenous high-mobility group box-1 (HMGB1) and Toll-like receptor (TLR) has been proved to be with pro-inflammatory as well as pro-convulsant effects. We hypothesized that the epileptogenic mechanisms underlying RE are related to activation of HMGB1/TLR signaling. Immunnohistochemistry approach was used to examine the expression of HMGB1, TLR2, TLR4, receptor for advanced glycation end products (RAGE) in surgically resected human epileptic cortical specimens from RE (n=12), and compared that with control cortical issue (n=6)...
July 2016: Epilepsy Research
https://www.readbyqxmd.com/read/27106864/anti-tumor-necrosis-factor-alpha-therapy-adalimumab-in-rasmussen-s-encephalitis-an-open-pilot-study
#16
Stanislas Lagarde, Nathalie Villeneuve, Agnès Trébuchon, Elsa Kaphan, Anne Lepine, Aileen McGonigal, Agathe Roubertie, Marie-Anne J Barthez, Valérie Trommsdorff, Jérémie Lefranc, Samer Wehbi, Vincent des Portes, Virginie Laguitton, Pierre Quartier, Didier Scavarda, Bernard Giusiano, Mathieu Milh, Christine Bulteau, Fabrice Bartolomei
OBJECTIVE: Rasmussen's encephalitis (RE) is a severe chronic inflammatory brain disease affecting one cerebral hemisphere and leading to drug-resistant epilepsy, progressive neurologic deficit, and unilateral brain atrophy. Hemispherotomy remains the gold standard treatment but causes permanent functional impairment. No standardized medical treatment protocol currently exists for patients prior to indication of hemispherotomy, although some immunotherapies have shown partial efficacy with functional preservation but poor antiseizure effect...
June 2016: Epilepsia
https://www.readbyqxmd.com/read/27040081/cd8-t-cell-pathogenicity-in-rasmussen-encephalitis-elucidated-by-large-scale-t-cell-receptor-sequencing
#17
Tilman Schneider-Hohendorf, Hema Mohan, Christian G Bien, Johanna Breuer, Albert Becker, Dennis Görlich, Tanja Kuhlmann, Guido Widman, Sebastian Herich, Christiane Elpers, Nico Melzer, Klaus Dornmair, Gerhard Kurlemann, Heinz Wiendl, Nicholas Schwab
Rasmussen encephalitis (RE) is a rare paediatric epilepsy with uni-hemispheric inflammation and progressive neurological deficits. To elucidate RE immunopathology, we applied T-cell receptor (TCR) sequencing to blood (n=23), cerebrospinal fluid (n=2) and brain biopsies (n=5) of RE patients, and paediatric controls. RE patients present with peripheral CD8(+) T-cell expansion and its strength correlates with disease severity. In addition, RE is the only paediatric epilepsy with prominent T-cell expansions in the CNS...
April 4, 2016: Nature Communications
https://www.readbyqxmd.com/read/27004940/rethinking-the-magnetic-resonance-imaging-findings-in%C3%A2-early%C3%A2-rasmussen-encephalitis-a-case-report-and-review-of%C3%A2-the%C3%A2-literature
#18
Megan Holec, Yasunori Nagahama, Christopher Kovach, Charuta Joshi
OBJECTIVE: We present a child with Rasmussen encephalitis and highlight the pitfalls of diagnosis when magnetic resonance imaging (MRI) is negative for atrophy. We review the literature regarding this issue, introduce the FreeSurfer software as a potential means of noninvasive diagnosis, and discuss methods for prompt and definitive treatment. METHODS: In addition to the patient description, we review the English language literature regarding pathologic diagnosis of Rasmussen encephalitis using the key words Rasmussen encephalitis, focal lesions, MRI, atrophy, epilepsia partialis continua and hemiparesis in PubMed...
June 2016: Pediatric Neurology
https://www.readbyqxmd.com/read/26941743/evidence-for-resident-memory-t-cells-in-rasmussen-encephalitis
#19
Geoffrey C Owens, Julia W Chang, My N Huynh, Thabiso Chirwa, Harry V Vinters, Gary W Mathern
Rasmussen encephalitis (RE) is a rare pediatric neuroinflammatory disease of unknown etiology characterized by intractable seizures, and progressive atrophy usually confined to one cerebral hemisphere. Surgical removal or disconnection of the affected cerebral hemisphere is currently the only intervention that effectively stops the seizures. Histopathological evaluation of resected brain tissue has shown that activated brain resident macrophages (microglia) and infiltrating T cells are involved in the inflammatory reaction...
2016: Frontiers in Immunology
https://www.readbyqxmd.com/read/26844739/autoimmune-epilepsy
#20
REVIEW
Nicolas Gaspard
PURPOSE OF REVIEW: This review presents recent developments in the clinical features, immunologic basis, and treatment options for autoimmune encephalitis, seizures, and epilepsy. RECENT FINDINGS: In addition to the expansion of our knowledge on classic paraneoplastic limbic encephalitis with onconeural antibodies, recent years have witnessed the development of the category of encephalitis associated with antibodies directed toward neuronal surface antigens. Antibodies against the voltage-gated potassium channel are, in fact, directed toward an array of targets within a large molecular complex...
February 2016: Continuum: Lifelong Learning in Neurology
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