Genetic predisposition to severe myoclonic epilepsy in infancy.
OBJECTIVE
To address genetic predisposition to febrile convulsions (FCs) and epilepsy as an etiologic background of severe myoclonic epilepsy in infancy (SMEI).


METHODS
Familial antecedents of epilepsy and FCs were analyzed in four groups of patients with SMEI (65 cases), FCs (57 cases), childhood absences (67 cases), and a control group of patients with no neurologic problems (64 cases).


RESULTS
Patients with SMEI and those with FCs had significantly increased incidence of FCs in their relatives compared with those with absence epilepsy and with the control group. The incidence of epilepsy in relatives of patients with SMEI and absence epilepsy was increased compared with that in the control group and reached statistical significance. Epilepsy in relatives with SMEI had the characteristics of idiopathic generalized epilepsy.


CONCLUSIONS
A genetic predisposition could determine three types of epileptic syndromes: FCs, idiopathic generalized epilepsy, and SMEI.
Neuropediatrics Department, Hôpital Saint Vincent de Paul, 82 Avenue Denfert Rocherau, 75674 Paris, France.
A
A
Benlounis
R
R
Nabbout
J
J
Feingold
A
A
Parmeggiani
R
R
Guerrini
A
A
Kaminska
O
O
Dulac
D000293:Adolescent; D000367:Age Factors; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D004831:Epilepsies, Myoclonic / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics*; D004832:Epilepsy, Absence / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics; D004829:Epilepsy, Generalized / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics; D005190:Family; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D015994:Incidence; D007223:Infant; D008297:Male; D012107:Research Design; D003294:Seizures, Febrile / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics*; D012720:Severity of Illness Index; D011795:Surveys and Questionnaires