A Dysbalanced Immune System in Cryptogenic Lennox‐Gastaut Syndrome

Engelen, B.G.M. van; Weemaes, C.M.R.; Renier, W.O.; Bakkeren, J. A. J. M.; Borm, George F.; Strengers, P. F. W.

doi:10.1111/j.1365-3083.1995.tb03555.x

Cited by 17 publications

(8 citation statements)

References 18 publications

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“…Our group previously reported immunological disturbances in patients with WS and LGS, consisting of impairment in T cell subclasses, T cell functional tests, in vivo skin sensitization tests and immunoglobulin levels and the demonstration of the presence of antibody to brain extract in the sera of WS and LGS children [13][14][15][16][17] . Other authors have described similar findings recently [18][19][20][21] .…”

supporting

confidence: 85%

“…Other alterations such as impaired natural killer cell activity, low CD4+ and high CD8+ lymphocytes, reduction in the CD4+/CD8+ ratio, and decreased C4 complement were also described in epilepsy 29,30 . Impairment in humoral immune response to antigens was reported in children with LGS 21 . All authors agree about the presence of a combined disorder of the immune and the nervous systems.…”

Section: Resultsmentioning

confidence: 99%

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Immunologic aspects of West syndrome and evidence of plasma inhibitory effects on T cell function

Montelli

Soares

Peraçoli

2003

Arq. Neuro-Psiquiatr.

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-Study objective: : :: : The purpose of this study was to assess the extent of immune dysfunction in a well-defined group of epileptic patients: children with diagnosis of West syndrome (WS) or with transitions to another age-related EEG patterns, the multifocal independent spikes (MIS), and the slow spike-wave complexes (Lennox-Gastaut syndrome -LGS). Thus, WS was studied at different points of the natural evolutive history of the disease. Method: A group of 50 patients (33 with WS, 10 with LGS and 7 with MIS) and 20 age-matched healthy controls were submitted to enumeration of T lymphocyte subsets: CD1, CD3, CD4, CD8, CD4/CD8 ratio and lymphocyte proliferation assay to phytohaemagglutinin (PHA), in the presence of autologous and AB, homologous plasma. Dinitrochlorobenzene (DNCB) skin test sensitization was performed only in patients. Determinations of IgG, IgA, and IgM serum levels were compared to standard values for Brazilian population in different age ranges. Results: Sensitization to DNCB showed absent or low skin reactions in 76% of the patients. High levels of IgG (45.7%) and IgM (61.4%), and lower levels of IgA (23.9%) were detected in the serum of the patients. Enumeration of lymphocyte subsets in peripheral blood showed: low CD3+ (p<0.05), low CD4+ (p<0.05), high CD8+ (p<0.01) and low CD4+ / CD8+ ratio (p<0.001). The proportion of CD1+ cells in the control group was less than 3%, while ranged between 6 and 11 % in 18% of the patients. The in vitro PHA-induced T cell proliferation showed significantly low blastogenic indices only when patients , cells were cultured in presence of their own plasma. No differences in blastogenic indices were observed when the cells of patients and controls were cultured with human AB plasma. Conclusion: The immunodeficiency in WS was mainly characterized by anergy, impaired cell-mediated immunity, altered levels of immunoglobulins, presence of immature thymocytes in peripheral blood and functional impairment of T lymphocytes induced by plasma inhibitory factors.KEY WORDS: dinitrochlorobenzene, West syndrome, Lennox-Gastaut syndrome, multifocal-independent spike syndrome, T-lymphocytes subsets, immunoglobulins, proliferation assay, T cells, CD1, CD3, CD4, CD8.Aspectos imunológicos da síndrome de W Aspectos imunológicos da síndrome de W Aspectos imunológicos da síndrome de W Aspectos imunológicos da síndrome de W Aspectos imunológicos da síndrome de West e evidência de efeitos inibitórios do plasma sobre a função est e evidência de efeitos inibitórios do plasma sobre a função est e evidência de efeitos inibitórios do plasma sobre a função est e evidência de efeitos inibitórios do plasma sobre a função est e evidência de efeitos inibitórios do plasma sobre a função de células T de células T de células T de células T de células T RESUMO -Objetivo: O objetivo deste estudo foi determinar o perfil da deficiência imune em um grupo bem definido de epilepsia: crianças com síndrome de West (SW) e seus padrões EEG de evolução, idade-dependentes, como os complexos onda-agud...

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supporting

confidence: 85%

Section: Resultsmentioning

confidence: 99%

Immunologic aspects of West syndrome and evidence of plasma inhibitory effects on T cell function

Montelli

Soares

Peraçoli

2003

Arq. Neuro-Psiquiatr.

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“…Van Engelen et al 26 found an increased serum concentration of IgG, but the humoral immune response to hemocyanin was impaired. Otherwise, no specific immunological abnormalities were detected.…”

Section: Other Childhood Epilepsiesmentioning

confidence: 98%

Epilepsy and the Immune System

Aarli¹

2000

Arch Neurol

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“…associada, em 21% dos casos, a baixos níveis de IgG 35 que, na forma criptogênica, mostra um envolvimento imunológico definido por resposta humoral, funcionalmente alterada, à um antígeno primário (hemocianina) e elevação na concentração de IgG 36 . Ou seja, em termos fisiopatogênicos, NCC e SLG possuem um substrato imunológico para suas manifestações clínicas -na NCC, através de mecanismos complexos, e na SLG, por mecanismos ainda obscuros.…”

Section: Fig 3 Evolução Dos Eegs (Ii) a -Atividade Epileptiforme Tunclassified

Neurocisticercose e síndrome de lennox-gastaut: relato de caso

Agapejev

Padula

Morales

et al. 2000

Arq. Neuro-Psiquiatr.

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Relata-se o caso de uma menina que, aos 2 anos de idade, apresentou a forma epiléptica, hidrocefálica e encefalítica da neurocisticercose, diagnosticada por exame do líquido cefalorraqueano e tomografia computadorizada de crânio, evolução com crises polimórficas, episódios de descompensação da hipertensão intracraniana por obstrução do sistema de derivação ventriculoperitoneal, retardo no desenvolvimento neuropsicomotor e cegueira até que, aos 10 anos de idade, foi diagnosticada síndrome de Lennox-Gastaut. Atualmente, a paciente tem 16 anos, apresenta sequelas neurológicas e crises parciais complexas com automatismos, parcialmente controladas com o uso de clobazan e oxcarbazepina. A primeira associação de neurocisticercose e síndrome de Lennox-Gastaut foi descrita em 1973, por Frochtengarten & Scarante, em uma menina com quadro clínico semelhante ao do caso relatado.

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A Dysbalanced Immune System in Cryptogenic Lennox‐Gastaut Syndrome

Cited by 17 publications

References 18 publications

Immunologic aspects of West syndrome and evidence of plasma inhibitory effects on T cell function

Immunologic aspects of West syndrome and evidence of plasma inhibitory effects on T cell function

Epilepsy and the Immune System

Neurocisticercose e síndrome de lennox-gastaut: relato de caso

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