Mental outcome in West syndrome: prognostic value of some clinical factors

Fifty-eight cases of West syndrome (eight idiopathic, 50 secondary) have been followed-up for a mean period of 5 years 5 months. The relationship has been studied between intellectual status at the final follow-up and the following variables: etiology, age of onset, neurological examination and developmental assessment at the onset, CT scan findings, short-term effect of ACTH treatment, and age of acquiring four developmental milestones (sitting unsupported, walking unsupported, uttering the first specific word and using a sentence of two words). Developmental and/or neurological abnormalities before the onset of the spasms, symptomatic etiology and abnormal CT findings are associated with a low IQ at the final follow-up. The age for sitting unsupported and for walking unsupported has proved to be not predictive of intellectual outcome, but the speech development data have shown a highly significant relationship with intellectual development. Early identification of the children with mental retardation has turned out to be superior to prediction of normal mental outcome.