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Long-term outcome of patients with West syndrome

      Abstract

      The long-term outcome of Finnish children with West syndrome was evaluated. Two hundred and fourteen patients were followed up for 20–35 years or until death. A third of the patients died before the age of 3 years. The most common cause of death was infection. Autopsy revealed brain anomalies in 25 of 38 (66%) autopsied patients. Intellectual outcome was normal or slightly impaired in a quarter of the patients. All of them completed their education at a normal school or in a school for the educationally impaired children. Another fourth were taught in special training schools. Specific cognitive deficits were seen in some patients with normal intelligence. Nine attended secondary schools and seven of them had a professional occupation. Ten were married and five had children. One third of the patients were seizure-free, another third had seizures daily or monthly, and the remaining patients had seizures less frequently. Factors associated with a good prognosis were cryptogenic etiology, normal development before the onset of the spasms, a short treatment lag, and a good response to adrenocorticotropic hormone; this was seen in both the symptomatic and the cryptogenic group, and there were no relapses. In this study, the late appearance of focal abnormalities in electroencephalography was not associated with an unfavorable outcome. Focal abnormalities in temporal region were often seen in patients with autism. The location of an abnormality may be of importance for the prognosis. In this study, all the patients (100%) could be followed, which may be due to the special circumstances characteristic of Finland. The outcome in children with West syndrome seems to be better than is generally believed.

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