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rasmussen encephalitis

Ananth P Abraham, Maya Mary Thomas, Vivek Mathew, Karthik Muthusamy, Sangeetha Yoganathan, G Edmond Jonathan, Krishna Prabhu, Roy Thomas Daniel, Ari G Chacko
OBJECTIVE: To determine whether preoperative non-lateralizing scalp electroencephalography (EEG) influences seizure outcome following peri-insular hemispherotomy (PIH) in pediatric hemispheric epilepsy. METHODS: Retrospective data was collected on all 45 pediatric patients who underwent PIH between 2005 and 2016. All underwent a basic pre-surgical evaluation consisting of detailed history and examination, neuropsychological assessment, MRI, and EEG. SPECT/PET, fRMI, or Wada testing were done in only eight patients...
January 30, 2019: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Anna R Tröscher, Isabella Wimmer, Lucía Quemada-Garrido, Ulrike Köck, Denise Gessl, Sanne G S Verberk, Bethany Martin, Hans Lassmann, Christian G Bien, Jan Bauer
Microglia nodule formation is a common feature in inflammatory brain diseases mediated by T lymphocytes such as viral and paraneoplastic encephalitis, multiple sclerosis, and Rasmussen encephalitis (RE). However, its role has not been fully understood yet. We hypothesized that, in RE, microglial nodules provide an environment for the initiation of the later dominating T-cell cytotoxicity. In RE stage 0, small primary microglia nodules could be identified in the absence of T cells. These primary nodules showed inflammasome activation and endosomal Toll-like receptor upregulation...
January 20, 2019: Acta Neuropathologica
Mitchell T Foster, Kumar Das, Paul May
BACKGROUND: The authors report a case with interesting clinical and radiological outcomes following complete anatomical hemispherectomy. METHODS: A seven-year-old female with medically refractory epilepsy secondary to Rasmussen's encephalitis was treated with a complete right-sided anatomical hemispherectomy. RESULTS: Surgical intervention provided seizure relief, and at eleven-years post-operatively she was independently mobile, with spasticity of the upper limb...
January 17, 2019: British Journal of Neurosurgery
Vasiliki Pantazou, Jan Novy, Andrea O Rossetti
INTRODUCTION: Status epilepticus (SE) represents a neurological emergency that leads to considerable morbidity and mortality. Following failure of first-line therapy, usually with benzodiazepines, there is no clear evidence to guide treatment of refractory SE, although a wide variety of approaches has been described anecdotally. OBJECTIVE: The aim of this study was to assess the clinical response to corticosteroids in adults with refractory and super-refractory SE, describing, to the best of our knowledge, the first adult SE cohort treated with corticosteroids...
January 9, 2019: CNS Drugs
Mu Zhang, Zhen-Li Tang, Li-Wen Wu, Li Yang, Xiao-Le Wang, Fei Yin, Jing Peng, Chen Chen
OBJECTIVE: To investigate the etiology and clinical features of epilepsia partialis continua (EPC) in children. METHODS: A retrospective analysis was performed for the clinical features, diagnosis and treatment of six children with EPC, and the clinical and laboratory features and prognosis were compared between the children with different etiologies. RESULTS: There were five girls and one boy, with an onset age ranging from one year and seven months to nine years...
December 2018: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
Roberta Morace, Sara Casciato, Pier Paolo Quarato, Addolorata Mascia, Alfredo D'Aniello, Liliana G Grammaldo, Marco De Risi, Giancarlo Di Gennaro, Vincenzo Esposito
PURPOSE: The purpose of this study was to report long-term seizure outcome in patients who underwent frontal lobe epilepsy (FLE) surgery. METHOD: This retrospective study included 44 consecutive subjects who underwent resective surgery for intractable FLE at IRCCS NEUROMED (period 2001-2014), followed up for at least 2 years (mean: 8.7 years). All patients underwent noninvasive presurgical evaluation and/or invasive electroencephalography (EEG) monitoring when nonconcordant data were obtained or epileptogenic zone was hypothesized to be close to the eloquent cortex...
January 2019: Epilepsy & Behavior: E&B
Dong Liu, Xin Wang, Yisong Wang, Peigang Wang, Dongying Fan, Sichang Chen, Yuguang Guan, Tianfu Li, Jing An, Guoming Luan
Rasmussen's encephalitis (RE) is a rare pediatric neurological disorder, and the exact etiology is not clear. Viral infection may be involved in the pathogenesis of RE, but conflicting results have reported. In this study, we evaluated the expression of both Epstein-Barr virus (EBV) and human herpes virus (HHV) 6 antigens in brain sections from 30 patients with RE and 16 control individuals by immunohistochemistry. In the RE group, EBV and HHV6 antigens were detected in 56.7% (17/30) and 50% (15/30) of individuals, respectively...
October 2018: Virologica Sinica
L Liang, K P Ma, H Leng, Y L Li, J Fu, X X Yao, Y L Liu, Q Liu
Objective: To investigate the clinicopathologic features of Rasmussen syndrome (RS) and to raise awareness of this rare disease. Methods: Clinicopathologic data of 4 cases of RS were retrospectively analyzed at Beijing Haidian Hospital from 2008 to 2016. Results: The clinical manifestations included epilepsia partialis continua and progressive neurologic deficits in all patients.MRI demonstrated unihemispheric focal cortical atrophy in all cases. The histopathologic changes included variable degrees of lymphocytic infiltrate within the cortex, subarachnoid space and perivascular cuffing...
September 8, 2018: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
Giovanni Di Liberto, Stanislav Pantelyushin, Mario Kreutzfeldt, Nicolas Page, Stefano Musardo, Roland Coras, Karin Steinbach, Ilena Vincenti, Bogna Klimek, Thomas Lingner, Gabriela Salinas, Nathalie Lin-Marq, Ori Staszewski, Marta Joana Costa Jordão, Ingrid Wagner, Kristof Egervari, Matthias Mack, Camilla Bellone, Ingmar Blümcke, Marco Prinz, Daniel D Pinschewer, Doron Merkler
Inflammatory disorders of the CNS are frequently accompanied by synaptic loss, which is thought to involve phagocytic microglia and complement components. However, the mechanisms accounting for aberrant synaptic connectivity in the context of CD8+ T cell-driven neuronal damage are poorly understood. Here, we profiled the neuronal translatome in a murine model of encephalitis caused by CD8+ T cells targeting antigenic neurons. Neuronal STAT1 signaling and downstream CCL2 expression were essential for apposition of phagocytes, ensuing synaptic loss and neurological disease...
October 4, 2018: Cell
Elizabeth H Ristagno, Sonam C Bhalla, Lindsey K Rasmussen
This article aims to describe a rare cause of severe encephalitis in 2 cases of infants with signs of intracranial hypertension and severe autonomic dysregulation. The authors conclude that human parechoviruses are becoming a more recognized cause of encephalitis because of the increasing use of rapid detection methods. With early recognition of this clinical entity, improved care can be administered.
October 2018: Journal of Child Neurology
Ai Peng Tan, Yen Ling Jocelyn Wong, Bingyuan Jeremy Lin, Hsiang Rong Clement Yong, Kshitij Mankad
INTRODUCTION: Cerebral hemiatrophy is an uncommon neuroimaging finding of diverse etiologies, conventionally classified into two broad categories: congenital and acquired. The authors propose an alternative pragmatic clinical approach to cerebral hemiatrophy, classifying its diverse etiologies into a single event insult such as an in utero stroke, or a progressive disorder from an inflammatory or neoplastic process, the latter of which needs urgent intervention and will be the focus of our review paper...
December 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Mar Carreño, Maria José Martí, Ibán Aldecoa, Celia Painous, Estefanía Conde, Francesc Valldeoriola, Josep Valls-Solé, Núria Bargalló, Francisco Gil, Isabel Manzanares, Xavier Setoain, Antonio Donaire, Esteban Muñoz, Pedro Roldán, Teresa Boget, Luis Pintor, Eva Bailles, Jordi Rumià
OBJECTIVE: To describe an adult patient with Rasmussen's disease with focal dystonia as the most disabling symptom and the good response to unilateral globus pallidus internus (GPi) deep brain stimulation (DBS). METHODS: Retrospective review of clinical records and diagnostic tests. RESULTS: The patient had displayedmild focal seizures with sensory and motor symptoms on the left arm and hemiface since the age of 22. Ten years later she experienced abrupt onset of focal left dystonia involving mainly the leg...
January 2019: Journal of Neurology, Neurosurgery, and Psychiatry
Joana Machado-Santos, Etsuji Saji, Anna R Tröscher, Manuela Paunovic, Roland Liblau, Galina Gabriely, Christian G Bien, Jan Bauer, Hans Lassmann
Multiple sclerosis is an inflammatory demyelinating disease in which active demyelination and neurodegeneration are associated with lymphocyte infiltrates in the brain. However, so far little is known regarding the phenotype and function of these infiltrating lymphocyte populations. In this study, we performed an in-depth phenotypic characterization of T and B cell infiltrates in a large set of multiple sclerosis cases with different disease and lesion stages and compared the findings with those seen in inflammatory, non-inflammatory and normal human controls...
July 1, 2018: Brain: a Journal of Neurology
Hania Kebir, Lionel Carmant, François Fontaine, Kathie Béland, Ciprian M Bosoi, Nathalie T Sanon, Jorge I Alvarez, Sébastien Desgent, Camille L Pittet, David Hébert, Marie-Josée Langlois, Rose-Marie Rébillard, Dang K Nguyen, Cécile Cieuta-Walti, Gregory L Holmes, Howard P Goodkin, John R Mytinger, Mary B Connolly, Alexandre Prat, Elie Haddad
Rasmussen's encephalitis (RE) is a chronic inflammatory brain disorder that causes frequent seizures and unilateral hemispheric atrophy with progressive neurological deficits. Hemispherectomy remains the only treatment that leads to seizure freedom for this refractory epileptic syndrome. The absence of an animal model of disease has been a major obstacle hampering the development of effective therapies. Here, we describe an experimental mouse model that shares several clinical and pathological features with the human disease...
May 1, 2018: Journal of Clinical Investigation
Lawrence Steinman
Rasmussen's encephalitis (RE) is a neuroinflammatory disease that typically affects only one hemisphere of the brain, resulting in severe seizures. Sixty years after the disease was first described, the preferred and best treatment option for RE is grotesque and involves removing a hemisphere of the brain (hemispherectomy); therefore, a better understanding of this seizure disorder may provide additional, less invasive therapeutic options. In this issue of the JCI, Carmant and colleagues have developed an animal model of this focal seizure disorder...
May 1, 2018: Journal of Clinical Investigation
Loulieta Nazerai, Amalie Skak Schøller, Peter Overbeck Sharma Rasmussen, Søren Buus, Anette Stryhn, Jan Pravsgaard Christensen, Allan Randrup Thomsen
The association between recent Zika virus (ZIKV) infection and neurological complications, microcephaly in the fetus, and Guillain-Barré syndrome in adults underscores the necessity for a protective vaccine. Rational vaccine development requires an in-depth understanding of the mechanisms which could protect against infection with this virus. However, so far, such an analysis has been hampered by the absence of a suitable small animal model. Unlike the situation in humans, ZIKV only replicates effectively in the peripheral organs of mice, if type I IFN signaling is interrupted...
2018: Frontiers in Immunology
Gabriela G Regner, Patrícia Pereira, Douglas T Leffa, Carla de Oliveira, Rafael Vercelino, Felipe Fregni, Iraci L S Torres
Epilepsy is a chronic brain syndrome characterized by recurrent seizures resulting from excessive neuronal discharges. Despite the development of various new antiepileptic drugs, many patients are refractory to treatment and report side effects. Non-invasive methods of brain stimulation, such as transcranial direct current stimulation (tDCS), have been tested as alternative approaches to directly modulate the excitability of epileptogenic neural circuits. Although some pilot and initial clinical studies have shown positive results, there is still uncertainty regarding the next steps of investigation in this field...
2018: Frontiers in Neuroscience
Elane N Magno
Rasmussen's encephalitis (RE) is a rare and devastating unilateral inflammatory brain disease that causes severe and intractable partial epilepsy. It has been shown that epilepsy and subsequent inflammation have deleterious influence on hippocampal cell survival and neurogenesis, but this still has not been systematically explored in human tissue. In this study, we investigated the correlation between inflammation and epilepsy as well as the rates of hippocampal gliogenesis and neurogenesis in a pediatric group of six patients with RE and six control cases...
May 2018: Epilepsy & Behavior: E&B
Maren Lindner, Luisa Klotz, Heinz Wiendl
It is widely accepted that development of autoimmunity in the central nervous system (CNS) is triggered by autoreactive T cells, that are activated in the periphery and gain the capacity to migrate through endothelial cells at the blood-brain barrier (BBB) into the CNS. Upon local reactivation, an inflammatory cascade is initiated, that subsequently leads to a recruitment of additional immune cells ultimately causing demyelination and axonal damage. Even though the interaction of immune cells with the BBB has been in the focus of research for many years, the exact mechanisms of how immune cells enter and exit the CNS remains poorly understood...
July 2018: Journal of Neurochemistry
O Fernández-Concepción, M López Jiménez, C Valencia-Calderón, A Calderón-Valdivieso, A Recasén-Linares, L Reyes-Haro, C Vásquez-Ham
INTRODUCTION: There is sufficient evidence on the usefulness of surgery as a therapeutic alternative for patients with drug-resistant epilepsy; however this treatment is underutilized, especially in developing countries. METHODS: We describe the outcomes of epilepsy surgery in 27 paediatric patients at Hospital Baca Ortiz in Quito, Ecuador. Our analysis considered the following variables: reduction in seizure frequency, surgery outcome according to the Engel classification, improvement in quality of life, and serious complications due to surgery...
March 7, 2018: Neurología: Publicación Oficial de la Sociedad Española de Neurología
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