Objective: Clinical and experimental data support the role of immune mechanisms in the pathogeny of childhood epilepsy. The purpose of this report is to study the immunological aspects in severe myoclonic epilepsy in infancy (SMEI).
Patients and methods: 12 patients selected according to the following criteria: no previous personal history of disease, frequent familial history of epilepsy, beginning in the first year of life with seizures usually febriles, onset of afebrile seizures between 18 months and 4 years, normal EEG and psychomotor development are at the onset, seizures resistant to antiepileptic drugs. The following immunological evaluation, has been carried out: granulocytes phagocytic activity, lymphocytics subpopulations, serum immunoglobulins and IgG subclasses, hemolytic capacity of complement, lymphocytic transformation test.
Results: In five cases the immunological study was normal; five cases had IgA deficiency, associated in two to high serum IgG1 levels and in two to high IgG1 and low IgG2 levels; one case shows an IgM increase associated to hemolytic capacity of complement deficiency (CH50); four cases have a reduced response to different mitogens.
Conclusions: The value of this results is doubtful. It is possible that genetical and environmental factors are involved in SMEI and that the immunological abnormalities found favor the hyperergic reactions in given circumstances.