You are in the accessibility menu

Please use this identifier to cite or link to this item:
Full metadata record
DC FieldValueLanguage
dc.contributor.authorMontelli, Terezinha de Cresci Braga-
dc.contributor.authorPeraçoli, M. T S-
dc.identifier.citationCentral Nervous System Agents in Medicinal Chemistry, v. 8, n. 2, p. 92-99, 2008.-
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.subjectCell-mediated immunity-
dc.subjectHumoral immunity-
dc.subjectLennox-Gastaut syndrome-
dc.subjectMultifocal independent spikes-
dc.subjectWest syndrome-
dc.subjectalpha interferon-
dc.subjectantinuclear antibody-
dc.subjectbeta2 glycoprotein 1-
dc.subjectbrain antibody-
dc.subjectbrain antigen-
dc.subjectdouble stranded DNA-
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.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.subjectinfantile spasm-
dc.subjectLennox Gastaut syndrome-
dc.subjectonset age-
dc.subjectT lymphocyte-
dc.subjectvirus infection-
dc.titleInfantile epileptic encephalopathy with hypsarrhythmia (infantile spasms/west syndrome) and immunityen
dc.contributor.institutionUniversidade Estadual Paulista (UNESP)-
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.rights.accessRightsAcesso restrito-
dc.relation.ispartofCentral Nervous System Agents in Medicinal Chemistry-
Appears in Collections:Artigos, TCCs, Teses e Dissertações da Unesp

There are no files associated with this item.

Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.