Serologic HLA Typing in Infantile Spasms

Hrachovy, Richard A.; Frost, James D.; Pollack, Marilyn S.; Glaze, Daniel G.

doi:10.1111/j.1528-1157.1988.tb04239.x

Cited by 13 publications

(3 citation statements)

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“…The same pattern was observed when convulsive tonic–clonic seizures were present. The only two previous reports in which HLA antigens were analyzed were done with a very small number of patients, as already mentioned (29,30). The first one explored only class I antigens and claimed no association, and the second reported an association with HLA‐DR52.…”

Section: Discussionmentioning

confidence: 99%

“…A second study analyzed the distribution of DR antigens in a group of patients of mixed origins, and claimed that IS was associated with the supertypic DR52 antigen, which shares some amino acid sequences with DR3 (DR17 and DR18 subtypes), DR5 (DR11 and DR12 subtypes) and DR6 (DR13 and DR14 subtypes) specificities. The authors included a very small patient sample size and a heterogeneous population in terms of ethnic background (30).…”

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Contribution of the MHC Class II Antigens to the Etiology of Infantile Spasm in Mexican Mestizos

et al. 2001

Epilepsia

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Summary:Purpose: Infantile spasms (ISs) are age-dependant epileptic seizures, which may be flexor, extensor, lightning or nods, or mixed. The aim of this study was the analysis of genetic factors within the human leukocyte antigen (HLA) complex associated with ISs.Methods: Sixty-five patients diagnosed according to the established international criteria were compared with 229 healthy individuals; all of them were Mexican Mestizos. Five families were also analyzed (seven affected and five healthy sibs); HLA class I and class II antigens were typed using the standard microlymphocytotoxicity methods.Results: The findings showed female gender preference (2:1). Two thirds were symptomatic, and prevalent seizures were of mixed type (67%). A strong association with HLA-DR17 was detected in the IS group (pc < 0.01; OR ‫ס‬ 3.6; EF ‫ס‬ 0.20). DR17 was also found increased in the symptomatic patients (p ‫ס‬ 0.009; OR ‫ס‬ 3.16) and in those with other types of seizures (p ‫ס‬ 0.001; OR ‫ס‬ 2.0). Conversely, HLA-DQ6 was significantly decreased (pc < 0.002; PF ‫ס‬ 0.37) in the total and in the symptomatic groups (p < 0.01). Haplotype linkage was not confirmed in the families; however, those with more than one affected sib shared at least one haplotype.Conclusions: These findings suggest the contribution of DR locus to the susceptibility and the participation of DQ region in the resistance to IS. Severity seems also to be influenced by HLA-DR17, and therefore class II typing may be a helpful tool for disease prognosis.

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Section: Discussionmentioning

confidence: 99%

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Contribution of the MHC Class II Antigens to the Etiology of Infantile Spasm in Mexican Mestizos

et al. 2001

Epilepsia

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“…Reported laboratory investigations in favour of an immunological aspect in human epilepsy include: an impaired humoral immunity predominantly IgA and IgG2 deficiencies,9-"2 lower kappa/lambda ratios of serum IgG, and serum IgM,"3 an increased incidence of antibodies against the frontal cortex,'415 increased percentage of B-cells,'6 decreased percentage of T-cells,12 decreased CD4-CD8 T-cell ratios,'7 and differences in HIA class II antigen frequencies compared with the general population. [18][19][20][21] The IgA deficiency found in 5-10% of patients with epilepsy is usually drug-induced, but such patients can have subnormal IgA before treatment.8 Other indications in favour of an immunological involvement in epilepsy are the clinical experience of exacerbation but also of total remission of intractable childhood epilepsy after viral infections." '3 Indirect evidence for reciprocal interactions between the immune and nervous system in epilepsy are the immunological alterations induced by anticonvulsant drugs such as, phenytoin, and carbamazepine,8'4 and the anticonvulsant effect of immunosuppressive drugs such as ACTH and corticosteroids in otherwise intractable childhood epilepsy like West syndrome.…”

Section: ) Possible Efficacy Of Ivig In Intractable Epilepsymentioning

confidence: 99%

Immunoglobulin treatment in human and experimental epilepsy.

Engelen

Renier

Weemaes

1994

Journal of Neurology, Neurosurgery & Psychiatry

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The relationship between human immunoglobulin administration, and human and experimental epilepsy was investigated by reviewing the literature as well as the authors' clinical and experimental studies. The focus was: 1) the possible efficacy of IVIg; 2) the 1) Possible efficacy of IVIg in intractable epilepsyTo ascertain possible efficacy, we identified all articles on IVIg (for reasons of convenience the abbreviation IVIg will be used for immunoglobulin administered intravenously and intramuscularly) treatment in intractable epilepsy by performing an English, German, and French-language literature search using MEDLINE (1966-93), by reviewing meeting reports, and by extensive hand search of bibliographies. In 24 studies, none with a placebo controlled design, 368 patients with epilepsy who were receiving IVIg were identified.3 Patients' ages ranged from < 1 to 35 years, mean 7-3 years. Female/male ratio was 06. All patients had intractable epilepsy. On average the percentage of patients with an IgG, deficiency was 25%. The total dose of IVIg varied between 03 and 6-8 gm/kg for a period of 0-15 to 12 months. Whenever reported, adverse effects of IVIg were minimal. None of the studies reported the need to stop IVIg administration because of adverse effects. On average the mean percentage of clinical seizure reduction, and the mean percentage of electroencephalographic (EEG) improvement was 52% and 45%, respectively. The average percentage of patients with complete seizure remission, and of patients with behavioural improvement was 23% and 63%, respectively.Cumulative meta-analysis of the reports was not possible because of the lack of controlled studies, the heterogeneity of the available studies, and the possible publication bias of unpublished negative data.

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The use of immunoglobulins in the treatment of human epilepsy

Villani

Avanzini

2002

Neurological Sciences

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The use of immunoglobulin (IVIg) in intractable epilepsy is one of its oldest applications in medicine, starting from the empirical observation of its beneficial effect on seizures. Immune system dysfunction may play a role in epilepsy by triggering, maintaining or, unexpectedly, improving intractable seizures. Several laboratory and clinical investigations are in favor of an immunological basis for different forms of experimental and human epilepsies. A wide range of immune abnormalities have been reported, suggesting the existence of different subtypes of epileptic syndromes with different abnormalities of the immune system. In this view, IVIg with its broad immunomodulatory mechanism of action could be effective in different forms of immune-dysregulated intractable epilepsies. Non-immunological mechanisms of action have been also suggested, based either on human epilepsy data or on animal experimental data. The possible anticonvulsant properties and the ability of IVIg to interfere with the final common pathway of seizures at a cellular level, with a significant increase in seizure threshold, have been demonstrated in different experimental epilepsy model. Although IVIg may represent a valuable resource in some drug-refractory epilepsies and its effectiveness has important pathogenetic implications, controlled studies with the systematic monitoring of immunological markers are needed to define more precise indications and to optimize the administration protocols.

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Serologic HLA Typing in Infantile Spasms

Cited by 13 publications

References 11 publications

Contribution of the MHC Class II Antigens to the Etiology of Infantile Spasm in Mexican Mestizos

Contribution of the MHC Class II Antigens to the Etiology of Infantile Spasm in Mexican Mestizos

Immunoglobulin treatment in human and experimental epilepsy.

The use of immunoglobulins in the treatment of human epilepsy

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