Infantile epileptic encephalopathy with hypsarrhythmia (infantile spasms/west syndrome) and immunity

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dc.contributor.authorMontelli, Terezinha de Cresci Braga [UNESP]
dc.contributor.authorPeraçoli, M. T S [UNESP]
dc.contributor.institutionUniversidade Estadual Paulista (Unesp)
dc.date.accessioned2014-05-27T11:23:33Z
dc.date.available2014-05-27T11:23:33Z
dc.date.issued2008-06-01
dc.description.abstractWest syndrome is a severe epilepsy, occurring in infancy, that comprises epileptic seizures known as spasms, in clusters, and a unique EEG pattern, hypsarrhythmia, with psychomotor regression. Maturation of the brain is a crucial component. The onset is within the first year of life, before 12 months of age. Patients are classified as cryptogenic (10 to 20%), when there are no known or diagnosed previous cerebral insults, and symptomatic (80 to 90%), when associated with pre-existing cerebral damages. The time interval from a brain insult to infantile spasms onset ranged from 6 weeks to 11 months. West syndrome has a time-limited natural evolutive course, usually disappearing by 3 or 4 years of age. In 62% of patients, there are transitions to another age-related epileptic encephalopathies, the Lennox-Gastaut Syndrome and severe epilepsy with multiple independent foci. Spontaneous remission and remission after viral infections may occur. Therapy with ACTH and corticosteroids are the most effective. Reports about intravenous immunoglobulins action deserve attention. There is also immune dysfunction, characterized mainly by anergy, impaired cell-mediated immunity, presence of immature thymocytes in peripheral blood, functional impairment of T lymphocytes induced by plasma inhibitory factors, and altered levels of immunoglobulins. Changes in B lymphocytes frequencies and increased levels of activated B cells have been reported. Sensitized lymphocytes to brain extract were also described. Infectious diseases are frequent and may, sometimes, cause fatal outcomes. Increase of pro-inflamatory cytokines in serum and cerebrospinal fluid of epileptic patients were reported. Association with specific HLA antigens was described by several authors (HLA-DR7, HLA-A7, HLA-DRw52, and HLA-DR5). Auto-antibodies to brain antigens, of several natures (N-methyl-d-aspartate glutamate receptor, gangliosides, brain tissue extract, synaptic membrane, and others), were described in epileptic patients and in epileptic syndromes. Experimental epilepsy studies with anti-brain antibodies demonstrated that epileptiform discharges can be obtained, producing hyperexcitability leading to epilepsy. We speculate that in genetically prone individuals, previous cerebral lesions may sensitize immune system and trigger an autoimmune disease. Antibody to brain antigens may be responsible for impairment of T cell function, due to plasma inhibitory effect and also cause epilepsy in immature brains. © 2008 Bentham Science Publishers Ltd.en
dc.description.affiliationBotucatu Medical School São Paulo State University, 18618-970, Botucatu, São Paulo
dc.description.affiliationInstitute of Biosciences São Paulo State University, 18618-970, Botucatu, São Paulo
dc.description.affiliation, Rua General Telles, 267, 18600-030 Botucatu - SP
dc.description.affiliationUnespBotucatu Medical School São Paulo State University, 18618-970, Botucatu, São Paulo
dc.description.affiliationUnespInstitute of Biosciences São Paulo State University, 18618-970, Botucatu, São Paulo
dc.format.extent92-99
dc.identifierhttp://dx.doi.org/10.2174/187152408784533941
dc.identifier.citationCentral Nervous System Agents in Medicinal Chemistry, v. 8, n. 2, p. 92-99, 2008.
dc.identifier.doi10.2174/187152408784533941
dc.identifier.issn1871-5249
dc.identifier.scopus2-s2.0-45749125562
dc.identifier.urihttp://hdl.handle.net/11449/70429
dc.language.isoeng
dc.relation.ispartofCentral Nervous System Agents in Medicinal Chemistry
dc.relation.ispartofsjr0,423
dc.rights.accessRightsAcesso restrito
dc.sourceScopus
dc.subjectAutoimmunity
dc.subjectCell-mediated immunity
dc.subjectHumoral immunity
dc.subjectLennox-Gastaut syndrome
dc.subjectMultifocal independent spikes
dc.subjectWest syndrome
dc.subjectalpha interferon
dc.subjectantinuclear antibody
dc.subjectautoantibody
dc.subjectbeta2 glycoprotein 1
dc.subjectbrain antibody
dc.subjectbrain antigen
dc.subjectcardiolipin
dc.subjectcorticosteroid
dc.subjectcorticotropin
dc.subjectcytokine
dc.subjectdouble stranded DNA
dc.subjectganglioside
dc.subjectglutamate decarboxylase
dc.subjectglutamate receptor
dc.subjectglycoprotein gp 130
dc.subjectHLA A antigen
dc.subjectHLA A7 antigen
dc.subjectHLA antigen
dc.subjectHLA DR5 antigen
dc.subjectHLA DR7 antigen
dc.subjectimmunoglobulin
dc.subjectinterleukin 10
dc.subjectinterleukin 1beta
dc.subjectinterleukin 2
dc.subjectinterleukin 6
dc.subjectinterleukin 6 receptor
dc.subjectn methyl dextro aspartic acid receptor
dc.subjectphospholipid antibody
dc.subjecttumor necrosis factor alpha
dc.subjectunindexed drug
dc.subjectautoimmune disease
dc.subjectB lymphocyte
dc.subjectcellular immunity
dc.subjectclonal anergy
dc.subjectcorticosteroid therapy
dc.subjectdisease classification
dc.subjectepileptic discharge
dc.subjecthormonal therapy
dc.subjecthuman
dc.subjecthypsarrhythmia
dc.subjectimmunotherapy
dc.subjectinfantile spasm
dc.subjectLennox Gastaut syndrome
dc.subjectnonhuman
dc.subjectonset age
dc.subjectremission
dc.subjectreview
dc.subjectT lymphocyte
dc.subjectvirus infection
dc.titleInfantile epileptic encephalopathy with hypsarrhythmia (infantile spasms/west syndrome) and immunityen
dc.typeResenha
dcterms.licensehttp://eurekaselect.com/209
unesp.campusUniversidade Estadual Paulista (Unesp), Instituto de Biociências, Botucatupt

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Artigos - Microbiologia e Imunologia - IBB