Whether seizures are the direct cause of cognitive deterioration in epilept
ic children is undetermined. This retrospective study aimed to delineate a
subgroup of pediatric patients with cognitive deterioration and refractory
seizures in the absence of recognized causes for mental retardation. Of the
80 children identified as having mental retardation and refractory seizure
disorder, seven (8.7%) had normal cognitive development until at least 1 y
ear of age. Their metabolic status was normal. Five of them suffered repeat
ed frequent partial seizures with onset in the first year of life and two h
ad repeated episodes of status epilepticus. All seven had similar character
istics of early onset partial seizures, six of them had partial seizures se
condarily generalized and one had complex partial seizures. The time of pea
k cognitive deterioration correlated with increases in seizure frequency du
ring that period. Evaluation revealed a well-defined epileptic focus in the
absence of neuroimaging abnormality except for hippocampal atrophy in the
two children with complex partial seizures and a small vascular malformatio
n in one child. Uncontrolled partial seizures in the first months of life m
ay result in cognitive deterioration. (C) 2000 Elsevier Science B.V. All ri
ghts reserved.