The idiopathic form of West syndrome.
The clinical and electroencephalographic data of 31 children with cryptogenic West syndrome (WS), selected from a series of 103 WS patients, with a follow-up between 4 and 12 years, were studied retrospectively to verify whether this group included patients who fulfilled the criteria for an idiopathic etiology. The results identified a possible idiopathic etiology in 17 patients (55%), who had a family history of other forms of idiopathic epilepsy or febrile convulsions, or who developed, during the follow-up, an EEG genetic trait such as a photoconvulsive response or spike-and-wave discharges, or rolandic spikes. All 17 children had a favorable outcome and all had normal neuropsychological development. Four children (13%) fulfilled the criteria for a true cryptogenic etiology, a causative lesion being suspected, but never proved. At the end of the follow-up all four had seizures, or developmental delay or both, all signs that suggest an underlying cerebral lesion. The other 10 children, representing 32% of the cryptogenic cases, had a good prognosis, with early disappearance of spasms and hypsarrhythmia, and normal neurological development, but none had an EEG epileptic trait or family history of epilepsy or febrile convulsions; although they could have had an idiopathic WS, this was not proved. We conclude that among the children classified as having a cryptogenic WS, many--in our series at least 55%--fulfill the criteria for an idiopathic etiology.
Vigevano
F
F
Section of Neurophysiology, Bambino Gesù, Children's Hospital, Rome, Italy.
Fusco
L
L
Cusmai
R
R
Claps
D
D
Ricci
S
S
Milani
L
L
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D004827:Epilepsy / Q000145:classification / Q000175:diagnosis / Q000235:genetics; D005190:Family; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D003294:Seizures, Febrile / Q000175:diagnosis / Q000235:genetics; D013036:Spasms, Infantile / Q000145:classification / Q000175:diagnosis* / Q000235:genetics