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rasmussen encephalitis

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https://www.readbyqxmd.com/read/30374827/detection-of-ebv-and-hhv6-in-the-brain-tissue-of-patients-with-rasmussen-s-encephalitis
#1
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
https://www.readbyqxmd.com/read/30220120/-rasmussen-syndrome-a-clinicopathologic-study-of-four-cases
#2
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
https://www.readbyqxmd.com/read/30173917/neurons-under-t-cell-attack-coordinate-phagocyte-mediated-synaptic-stripping
#3
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
https://www.readbyqxmd.com/read/30105932/a-case-series-of-parechovirus-encephalopathy-apnea-and-autonomic-dysregulation-in-critically-ill-infants
#4
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
https://www.readbyqxmd.com/read/30083798/clinico-radiological-approach-to-cerebral-hemiatrophy
#5
REVIEW
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
https://www.readbyqxmd.com/read/29986904/unilateral-pallidal-stimulation-for-disabling-dystonia-due-to-rasmussen-s-disease
#6
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...
July 9, 2018: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/29873694/the-compartmentalized-inflammatory-response-in-the-multiple-sclerosis-brain-is-composed-of-tissue-resident-cd8-t-lymphocytes-and-b-cells
#7
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
https://www.readbyqxmd.com/read/29629902/humanized-mouse-model-of-rasmussen-s-encephalitis-supports-the-immune-mediated-hypothesis
#8
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
https://www.readbyqxmd.com/read/29629901/blocking-immune-intrusion-into-the-brain-suppresses-epilepsy-in-rasmussen-s-encephalitis-model
#9
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
https://www.readbyqxmd.com/read/29623081/a-new-in-vivo-model-to-study-protective-immunity-to-zika-virus-infection-in-mice-with-intact-type-i-interferon-signaling
#10
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
https://www.readbyqxmd.com/read/29623027/preclinical-to-clinical-translation-of-studies-of-transcranial-direct-current-stimulation-in-the-treatment-of-epilepsy-a-systematic-review
#11
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
https://www.readbyqxmd.com/read/29579552/proliferative-hippocampal-activity-in-a-group-of-patients-with-rasmussen-s-encephalitis-neuronal-glial-and-bdnf-tissue-expression-correlations
#12
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
https://www.readbyqxmd.com/read/29574788/mechanisms-underlying-lesion-development-and-lesion-distribution-in-cns-autoimmunity
#13
REVIEW
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
https://www.readbyqxmd.com/read/29525400/safety-and-effectiveness-of-surgery-for-epilepsy-in-children-experience-of-a-tertiary-hospital-in-ecuador
#14
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
https://www.readbyqxmd.com/read/29414563/genital-automatisms-reappraisal-of-a-remarkable-but-ignored-symptom-of-focal-seizures
#15
Hava Özlem Dede, Nerses Bebek, Candan Gürses, Leyla Baysal-Kıraç, Betül Baykan, Ayşen Gökyiğit
OBJECTIVES: Genital automatisms (GAs) are uncommon clinical phenomena of focal seizures. They are defined as repeated fondling, grabbing, or scratching of the genitals. The aim of this study was to determine the lateralizing and localizing value and associated clinical characteristics of GAs. METHODS: Three hundred thirteen consecutive patients with drug-resistant seizures who were referred to our tertiary center for presurgical evaluation between 2009 and 2016 were investigated...
March 2018: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/29331846/functional-neuroimaging-in-rasmussen-syndrome
#16
Ichiro Kuki, Kazumi Matsuda, Yuko Kubota, Tetsuhiro Fukuyama, Yukitoshi Takahashi, Yushi Inoue, Haruo Shintaku
PURPOSE: For a diagnosis of Rasmussen syndrome (RS), clinical course together with electroencephalography (EEG) and magnetic resonance imaging (MRI) findings are considered important, but there are few reports on functional neuroimaging. This study investigated cerebral blood flow (CBF)-single photon emission computed tomography (SPECT), central benzodiazepine receptor (BZR)-SPECT, and fluorine-18 fluorodeoxy glucose-positron emission tomography (FDG-PET) in RS patients, and correlated neuroimaging results with MRI and pathological findings...
February 2018: Epilepsy Research
https://www.readbyqxmd.com/read/29259996/phenotypic-and-functional-complexity-of-brain-infiltrating-t-cells-in-rasmussen-encephalitis
#17
Faiez Al Nimer, Ivan Jelcic, Christian Kempf, Tom Pieper, Herbert Budka, Mireia Sospedra, Roland Martin
Objective: To characterize the brain-infiltrating immune cell repertoire in Rasmussen encephalitis (RE) with special focus on the subsets, clonality, and their cytokine profile. Methods: The immune cell infiltrate of freshly isolated brain tissue from RE was phenotypically and functionally characterized using immunohistology, flow cytometry, and T-cell receptor (TCR) deep sequencing. Identification of clonally expanded T-cell clones (TCCs) was achieved by combining flow cytometry sorting of CD4+ and CD8+ T cells and high-throughput TCR Vβ-chain sequencing...
January 2018: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/29258971/adult-onset-rasmussen-encephalitis-associated-with-focal-cortical-dysplasia
#18
Katharina Hohenbichler, Julie Lelotte, Renaud Lhommel, Riëm El Tahry, Pascal Vrielynck, Susana Ferrao Santos
Rasmussen encephalitis is a rare, devastating condition, typically presenting in childhood. Cases of adult-onset Rasmussen have also been described, but the clinical picture is less defined, rendering final diagnosis difficult. We present a case of adult-onset Rasmussen encephalitis with dual pathology, associated with focal cortical dysplasia and encephalitis. We interpreted the Rasmussen encephalitis to be caused by severe and continuous epileptic activity due to focal cortical dysplasia. The best therapeutic approach for such cases remains unclear...
December 1, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/29118737/a-case-series-of-adult-onset-rasmussen-s-encephalitis-diagnostic-and-therapeutic-challenges
#19
James Francis Castellano, Jenny A Meyer, Fred Alexander Lado
Rasmussen's encephalitis (RE) is a rare neurologic disorder characterized by progressive cerebral hemiatrophy and medically refractory epilepsy. The majority of current literature on this topic is focused on the pediatric population. In this case series, we will review three cases of adult-onset RE, as defined by fulfillment of the 2005 Bien criteria. The diagnostic challenge of characterizing this rare disease will be highlighted by the extensive serum, CSF, and pathologic sampling in all three patients. MR imaging and EEG data will be examined over time to characterize hallmark findings as well as progression...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/29116594/elevated-expression-of-ebv-and-tlrs-in-the-brain-is-associated-with-rasmussen-s-encephalitis
#20
Xin Wang, Yisong Wang, Dong Liu, Peigang Wang, Dongying Fan, Yuguang Guan, Tianfu Li, Guoming Luan, Jing An
Rasmussen's encephalitis (RE) is a rare pediatric neurological disorder, the etiology of which remains unclear. It has been speculated that the immunopathogenesis of RE involves damage to neurons, which eventually leads to the occurrence of RE. Viral infection may be a critical factor in triggering RE immunopathogenesis. In this study, we analyzed the expression of Epstein-Barr virus (EBV) antigens as well as of Toll-like receptor 3 (TLR3), TLR9, and downstream adapter TIRdomain-containing adapter-inducing interferon-β (TRIF) in the brain tissues of 26 patients with RE and 16 control individuals using immunohistochemistry (IHC)...
October 2017: Virologica Sinica
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