Cell-mediated and humoral immunity in West syndrome

The immunological status of five children with West syndrome consequent to previous cerebral lesions was investigated. Three children had West syndrome and two were in transition from West to Lennox-Gastaut syndrome. All of them showed cellular immunological deficiencies in the following tests: sensitization to DNCB, intracutaneous reaction to PHA, inhibition of leukocyte migration, blastic transformation of lymphocytes, T and B lymphocytes in peripheral blood and levels of serum immunoglobulins. These immunological deficiencies, of different degrees of severity, were associated with frequent infections in these children. A possible association between the immunological deficiencies and autoimmunity is discussed.

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

West 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.

DNA vaccine containing the mycobacterial hsp65 gene prevented insulitis in MLD-STZ diabetes

Background: Our group previously demonstrated that a DNA plasmid encoding the mycobacterial 65-kDa heat shock protein (DNA-HSP65) displayed prophylactic and therapeutic effect in a mice model for tuberculosis. This protection was attributed to induction of a strong cellular immunity against HSP65. As specific immunity to HSP60 family has been detected in arthritis, multiple sclerosis and diabetes, the vaccination procedure with DNA-HSP65 could induce a cross-reactive immune response that could trigger or worsen these autoimmune diseases. Methods: In this investigation was evaluated the effect of a previous vaccination with DNA-HSP65 on diabetes development induced by Streptozotocin (STZ). C57BL/6 mice received three vaccine doses or the corresponding empty vector and were then injected with multiple low doses of STZ. Results: DNA-HSP65 vaccination protected mice from STZ induced insulitis and this was associated with higher production of IL-10 in spleen and also in the islets. This protective effect was also concomitant with the appearance of a regulatory cell population in the spleen and a decreased infiltration of the islets by T CD8+ lymphocytes. The vector (DNAv) also determined immunomodulation but its protective effect against insulitis was very discrete. Conclusion: The data presented in this study encourages a further investigation in the regulatory potential of the DNA-HSP65 construct. Our findings have important implications for the development of new immune therapy strategies to combat autoimmune diseases. © 2009 Santos et al; licensee BioMed Central Ltd.

Efeito da própolis sobre a produção de citocinas e expressão do receptor TLR-4 por camundongos submetidos a estresse

Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)

Estado nutricional e perfil bioquímico de zinco e selênio em pacientes com deficiência de UgA

Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)

Efeito modulador de estratégias vacinais para tuberculose na encefalite autoimune experimental (EAE)

Pós-graduação em Doenças Tropicais - FMB

Estudo da associação entre urticária crônica idiopática e autoimunidade tireóidea: aspectos clínicos, positividade do teste do soro autólogo da pele e expressão gênica do receptor de TSH

Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)

Análise de associações de polimosfismos HPA, indicadores de autoimunidade e manifestações reumatológicas na Hepatite C

Pós-graduação em Fisiopatologia em Clínica Médica - FMB

Estudo da inflamação e da autoimunidade na doença hepática gordurosa não alcoólica

Pós-graduação em Fisiopatologia em Clínica Médica - FMB

Trypanosoma cruzi and Its soluble antigens induce NET release by stimulating toll-like receptors

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)

Avaliação audiológica em pacientes com doenças reumáticas pediátricas

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)