A long-term follow-up study of West syndrome

Since the syndrome of infantile spasms, hypsarrhythmia and mental retardation was first delineated, there have been many publications about long-term prognosis of the West syndrome. This paper evaluates some of the early factors on which long-term prognosis may depend.

Patients with West syndrome were reviewed retrospectively according to the medical records as classification, response to initial medication regimen, prognosis and present neurological status. Seizure control, speech, psychomotor development, mentality, learning ability, behavior and motor handicap were followed over a mean period of 6.1 years.

Thirty patients with complete hospital records were included in this study. Fourteen cases (46.7%) belonged to the symptomatic group, with a mean age of 3.4 months at onset. Four patients (13.3%) were cryptogenic type and a mean age of onset at 9.3 months. There were twelve cases (40%) in the doubtful group, whose mean onset age was 6.4 months. Of the symptomatic group, 92.8% patients had moderate to severe psychomotor retardation as compared to 25% in the cryptogenic group and 67% in the doubtful group; 35.7% of the symptomatic group patients suffered from motor handicap (spastic quadriplegia and hemiplegia as predominated), but only 8.3% of the doubtful group had motor handicap.

The data collected showed that the following factors seemed to be related to better prognosis: later onset, normal to mild psychomotor delay upon diagnosis, and good seizure control.