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Rasmussen's encephalitis

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https://www.readbyqxmd.com/read/29629902/humanized-mouse-model-of-rasmussen-s-encephalitis-supports-the-immune-mediated-hypothesis
#1
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...
April 9, 2018: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/29629901/blocking-immune-intrusion-into-the-brain-suppresses-epilepsy-in-rasmussen-s-encephalitis-model
#2
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...
April 9, 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
#3
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
#4
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
#5
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...
March 23, 2018: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/29574788/mechanisms-underlying-lesion-development-and-lesion-distribution-in-cns-autoimmunity
#6
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...
March 25, 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
#7
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
#8
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...
February 1, 2018: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/29331846/functional-neuroimaging-in-rasmussen-syndrome
#9
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
#10
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
#11
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
#12
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
#13
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
https://www.readbyqxmd.com/read/29103423/autoimmune-epilepsies
#14
Anusha K Yeshokumar, Carlos A Pardo
Autoimmune epilepsies describe clinical syndromes wherein the immune system is suspected to be involved in the pathogenesis of seizures or as a mechanism for neuronal injury following seizures. These diseases typically affect otherwise healthy children and are characterized by explosive onset of focal seizures, encephalopathy, cognitive deterioration, or other focal neurological deficits, or all of these. Traditional neurological diagnostics lack sensitivity and specificity in the diagnosis of autoimmune epilepsies, and results must be considered in the clinical context...
August 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/29096685/clinical-spectrum-of-woolly-hair-indications-for-cerebral-involvement
#15
Piero Pavone, Raffaele Falsaperla, Massimo Barbagallo, Agata Polizzi, Andrea D Praticò, Martino Ruggieri
BACKGROUND: Woolly Hair is an uncommon congenital anomaly of the scalp hair presenting with strongly coiled hair involving a localized area of the scalp or covering the entire side and occurring in non-black people. Isolated or localized wooly hair is usually benign and is not related to other disorders and/or complications. On the contrary, the generalized type may be related to disorders and syndromes affecting heart, cutis, liver and gastrointestinal organs. Among the syndromes presenting with wooly hair, the most known are the Naxos syndrome, the Carvajal-Huerta syndrome, the wooly hair/hypotrichosis, the ectodermal dysplasia-skin fragility, the tricho-hepato-enteric syndrome...
November 2, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/29091250/functional-hemispherectomy-for-adult-rasmussen-encephalitis-a-case-report-and-literature-review
#16
Qun Wang, Zhanpeng Zhu, Guangming Wang, Lichao Sun, Jiqing Qiu
BACKGROUND: Rasmussen encephalitis (RE) is a rare and severe brain disorder associated with unilateral hemispheric atrophy, manifesting as severe refractory epilepsy, hemiplegia, defects of motor and speech functions, and cognitive impairment. Treatment of RE, especially in adult patients, is extremely challenging. Herein, we report the case of an adult patient with RE who was treated with a functional hemispherectomy and achieved a favorable prognosis. CASE REPORT: A 29-year-old woman presented with a 24-year history of epileptic seizures...
September 26, 2017: Turkish Neurosurgery
https://www.readbyqxmd.com/read/29054955/trapped-ipsilateral-lateral-ventricle-a-delayed-complication-of-hemispherotomy-for-rasmussen-s-encephalitis
#17
Rajesh Shankar Iyer, Ravi Mohan Rao, Karunakaran Muthukalathi, Praveen Kumar
Hemispherotomy is the currently preferred surgical treatment option for refractory unihemispheric epilepsies. The incidence of hydrocephalus is greatly reduced in this disconnective procedure when compared with the resective procedure of anatomical hemispherectomy. We describe the occurrence of ipsilateral trapped lateral ventricle months after hemispherotomy for Rasmussen's encephalitis. There is enough evidence to suggest that this rare and interesting complication is due to the local inflammatory changes associated with the surgical trauma...
October 19, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29053073/chronic-subthreshold-cortical-stimulation-for-adult-drug-resistant-focal-epilepsy-safety-feasibility-and-technique
#18
Panagiotis Kerezoudis, Sanjeet S Grewal, Matthew Stead, Brian Nils Lundstrom, Jeffrey W Britton, Cheolsu Shin, Gregory D Cascino, Benjamin H Brinkmann, Gregory A Worrell, Jamie J Van Gompel
OBJECTIVE Epilepsy surgery is effective for lesional epilepsy, but it can be associated with significant morbidity when seizures originate from eloquent cortex that is resected. Here, the objective was to describe chronic subthreshold cortical stimulation and evaluate its early surgical safety profile in adult patients with epilepsy originating from seizure foci in cortex that is not amenable to resection. METHODS Adult patients with focal drug-resistant epilepsy underwent intracranial electroencephalography monitoring for evaluation of resection...
October 20, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28987176/immune-mediated-disorders
#19
Romana Höftberger, Hans Lassmann
Paraneoplastic and autoimmune encephalitis comprise a group of immune-mediated disorders that are associated with different immune effector mechanisms. Classic paraneoplastic neurologic syndromes are triggered by an antitumor immune response. The disease is considered to result from a T-cell response; in addition, patients harbour high titers of autoantibodies against intracellular antigens that are considered as epiphenomenon but are useful diagnostic markers. Neuropathology consists of T-cell-dominated inflammation, marked neuronal loss, and microglial activation with upregulation of HLA-DR...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28987170/neuropathology-of-epilepsy
#20
Eleonora Aronica, Angelika Mühlebner
Epilepsy is one of the most common neurologic disorders, affecting about 50 million people worldwide. The disease is characterized by recurrent seizures, which are due to aberrant neuronal networks resulting in synchronous discharges. The term epilepsy encompasses a large spectrum of syndromes and diseases with different etiopathogenesis. The recent development of imaging and epilepsy surgery techniques is now enabling the identification of structural abnormalities that are part of the epileptic network, and the removal of these lesions may result in control of seizures...
2017: Handbook of Clinical Neurology
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