Autoantibodies to Glutamate Receptor GluR3 in Rasmussen's Encephalitis

Rogers, S.W.; Andrews, P. Ian; Lc, Gahring; Whisenand, Teri D.; Cauley, Keith A.; Crain, B J; Hughes, TE; Heinemann, Sabine; McNamara, J. O.

doi:10.1126/science.8036512

Cited by 627 publications

(406 citation statements)

References 15 publications

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“…All these data are interpreted in terms that epilepsy pathogenesis may represent in some way an inflammatory process in the brain (Aarli, 2000;Aronica and Crino, 2011), and so-called Rasmussen's encephalitis confirms this suggestion (Rasmussen and Anderman, 1989;Rogers et al, 1994).…”

Section: Introductionmentioning

confidence: 56%

The interrelationship between clinical and immunity variables in epilepsy

Kalinin¹,

Subbotin²,

Yermakova³

2016

Journal of Epileptology

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SUMMARYIntroduction. The role of cellular immunity in the pathogenesis of epilepsy, as an interaction between immunity and clinical and neurobiological variables is not properly understood. Aim. The aim of the current study was to investigate the possible relationship between epilepsy forms, gender, focus localization, lateralization, handedness and cellular immunity with seizures frequency, their severity and length of therapeutic remission in partial forms of epilepsy. Material and methods. Ninety two patients (38 men and 54 women) were included in the study. Symptomatic epilepsy was diagnosed in 40 patients and the cryptogenic form was diagnosed in 52 patients. The amount of different lymphocyte clusters were evaluated and they were transformed into nominal variables for MANOVA analysis. MANOVA was used for the analysis of the interrelationship between nominal fixed factors (epilepsy forms, gender, handedness, and focus laterality, and immunity variables) and dependent variables (remission and seizure frequency and their severity). Results. Simple partial seizure (SPS) and complex partial seizure (CPS) frequencies were under the influence of interaction between immune and neurobiological variables. SPS, and in particular sensory SPS, were associated with CD4/CD8 ratio, gender, left temporal focus and handedness. The highest frequencies of SPS were revealed in cases of low CD4/CD8 ratio combined with left temporal focus, female gender and left-handedness. The maximal CPS frequency was observed in patients with a left frontal focus combined with a high B-lymphocyte level. The more severe seizures were revealed in left-handers with low CD8 and high CD4/CD8 ratio and in frontal left focus and a high T-lymphocyte level. There was a correlation between CD4 cell level and length of remission.

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Section: Introductionmentioning

confidence: 56%

The interrelationship between clinical and immunity variables in epilepsy

Kalinin¹,

Subbotin²,

Yermakova³

2016

Journal of Epileptology

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“…The clinical presentation is that of refractory seizures with cognitive decline, hemispheric atrophy, and progressive hemiparesis; young children tend to progress much more rapidly than young adults. A serendipitous finding that rabbits immunized with the glutamate receptor GluR3 developed a syndrome similar to RE, which led to the identification of antibodies to GluR3 in the serum of many patients with RE [272]. Despite the presence of GluR3 antibodies in the serum, histology suggests that RE may be a T-cell mediated disease [273].…”

Section: Rasmussen's Encephalitismentioning

confidence: 99%

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

Geldern¹,

McPharlin

Becker

2012

Neurotherapeutics

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This chapter will review the spectrum of immunemediated diseases that affect the nervous system and may result in an admission to the neurological intensive care unit. Immunomodulatory strategies to treat acute exacerbations of neurological diseases caused by aberrant immune responses are discussed, but strategies for long-term immunosuppression are not presented. The recommendations for therapeutic intervention are based on a synthesis of the literature, and include recommendations by the Cochrane Collaborative, the American Academy of Neurology, and other key organizations. References from recent publications are provided for the disorders and therapies in which randomized clinical trials and large evidenced-based reviews do not exist. The chapter concludes with a brief review of the mechanisms of action, dosing, and side effects of commonly used immunosuppressive strategies in the neurocritical care unit.

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“…In children, EPC is frequently caused by Rasmussen encephalitis, a condition of unclear etiology that has been associated with antibodies against glutamic acid receptors and with cytomegalovirus infection. 8,9 The causes of EPC include metabolic and developmental errors, brain tumors, demyelinating disorders, trauma and strokes. Recently, Shavit and associates 10 described three adults with cancer and focal sensorimotor encephalitis presenting with EPC.…”

Section: Discussionmentioning

confidence: 99%

Rolandic encephalopathy and epilepsia partialis continua following bone marrow transplant

Antunes

Boulad

Prasad

et al. 2000

Bone Marrow Transplant

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Summary:Epilepsia partialis continua (EPC) is a condition defined by prolonged focal myoclonus. Often resistant to therapy, EPC in children is frequently present in Rasmussen encephalitis, a form of chronic encephalitis of uncertain etiology. We discuss a child who developed bilateral EPC 5 months after a bone marrow transplant. Neuroimaging studies showed signal abnormalities on both sensory-motor areas. An extensive search failed to reveal the etiology of the disorder, but treatment with a broad-spectrum anti-viral agent was associated with resolution of the process. An unidentified infectious agent may be responsible for an encephalitis of the motor strip in immunosuppressed patients. Bone Marrow Transplantation (2000) 26, 917-919. Keywords: epilepsia partialis continua; bone marrow transplant; encephalitis A type of myoclonus in which one group of muscles, usually the flexors of the hand and fingers, is continuously involved in rhythmic monophasic contractions is called epilepsia partialis continua (EPC). 1 In children, EPC is the characteristic manifestation of Rasmussen encephalitis, but can also be encountered in mitochondrial disorders or migrational defects. 2 A number of different disorders have been associated with EPC in the adult population. 3 Recently, an HIV-infected patient was reported with isolated chronic EPC. 4 The etiology was unclear, but since no other viral agent was identified, focal HIV 1 encephalitis was the presumed cause. We followed an HIV-negative child with acute myelogenous leukemia who developed a strikingly similar disorder after a bone marrow transplant. It seems plausible that an unidentified infectious agent may be responsible for an encephalitis of the motor area presenting as EPC in immunocompromised patients. Case reportA 6-year-old Peruvian boy was well until January 1998 when he noticed left ear discomfort followed by a left facial droop. A right facial palsy ensued, and 3 months later a complete blood cell count (CBC) revealed an elevated white blood cell count (WBC) with 15% atypical cells. He was referred to Memorial Sloan-Kettering Cancer Center (MSKCC) where a bone marrow aspirate revealed 36% blasts. The morphology, immunophenotype and cytogenetic studies were consistent with AML M2 FAB morphology subtype. The patient was treated with four courses of dexamethasone, daunorubicin, cytosine arabinoside (Ara-C), thioguanine and etoposide along with intrathecal cytosine arabinoside. Remission was achieved with clearing of the neurologic deficits. On 30 July 1998 he received a T-cell depleted bone marrow transplant (BMT) from his HLA-matched brother. Pre-transplant cytoreduction consisted of 1500 cGy total body irradiation preceded by 600 cGy whole brain irradiation, thiotepa and cyclophosphamide. He received 3 doses of Ara-C intrathecally at 2, 3 and 4 months post transplant for CNS prophylaxis. After successful engraftment the patient was discharged and returned to his country in the first week of December.The child remained asymptomatic until 30 December 1998 (5 mo...

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Autoantibodies to Glutamate Receptor GluR3 in Rasmussen's Encephalitis

Cited by 627 publications

References 15 publications

The interrelationship between clinical and immunity variables in epilepsy

The interrelationship between clinical and immunity variables in epilepsy

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

Rolandic encephalopathy and epilepsia partialis continua following bone marrow transplant

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