Epidemiologic features of infantile spasms in Iceland.
We determined the average annual incidence of epilepsy characterized by infantile spasms (IS) in Iceland based on the 13 cases identified in a 10-year interval (1981-1990). The cumulative incidence was 3 in 10,000 live births. Males were more frequently affected than females. All patients responded completely (10) or partially (3) to ACTH or steroid therapy. At follow-up, all children with cryptogenic IS are seizure-free and have developed normally intellectually. One of the children with cryptogenic IS has mild spastic diplegia. All children in the symptomatic group are mentally retarded, and 5 of 7 continue to have unprovoked seizures. We detected no evidence for familial aggregation, and the incidence of seizures or epilepsy did not appear to be increased in first-degree relatives of cases. The proportion of cases with a favorable outcome was greater than that reported in most clinical series.
Lúthvígsson
P
P
Department of Pediatrics, National University Hospital (Landspítalinn) Reykjavik, Iceland.
Olafsson
E
E
Sigurthardóttir
S
S
Hauser
W A
WA
D000324:Adrenocorticotropic Hormone / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003431:Cross-Cultural Comparison; D004827:Epilepsy / Q000188:drug therapy / Q000453:epidemiology; D005260:Female; D005387:Finland / Q000453:epidemiology; D005500:Follow-Up Studies; D006801:Humans; D007055:Iceland / Q000453:epidemiology; D015994:Incidence; D007223:Infant; D008297:Male; D011239:Prednisolone / Q000627:therapeutic use; D012737:Sex Factors; D013036:Spasms, Infantile / Q000188:drug therapy / Q000453:epidemiology*; D014481:United States / Q000453:epidemiology
D000324:Adrenocorticotropic Hormone; D011239:Prednisolone