Seizures have not historically been considered a major component of Down sy
ndrome. We examined the prevalence of epileptic seizures in 350 children an
d adolescents with Down syndrome evaluated at a regional center between 198
5 and 1997. Results showed that 28 patients (8%) had epileptic seizures: 13
(47%) partial seizures; 9 (32%) infantile spasms, and 6 (21%) generalized
tonic-clonic seizures. In the infantile spasm group, there was no relations
hip between the initial electroencephalogram (EEG) pattern and response to
treatment or longterm seizure control, or between type of pharmacologic tre
atment (valproic acid, adrenocorticotropic hormone or both) and clinical re
mission, EEG normalization or long-term seizure control. Neurodevelopmental
outcome was poor despite good seizure control in the infantile spasm group
. This regional study reinforces the relative association of seizures and D
own syndrome. A prospective study including a national/international regist
ry with emphasis on developmental assessment and long-term follow up is war
ranted. (C) 2001 Elsevier Science B.V. All rights reserved.