A Case of Schizencephaly Type I Associated with West Syndrome that Responded to ACTH Treatment

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Although schizencephaly is known to be complicated by various types of epilepsy, complication by West syndrome is rare. We encountered a boy with West syndrome accompanied by schizencephaly. There was no abnormality in the birth history. After birth, the right upper and lower limb movements were less frequent and weaker than those on the left side. The neck was stabilized at 4 months of age. At 5 months of age, the boy developed infantile spasms, and hypsarrythmia was noted on EEG. Brain MRI detected schizencephaly fused with the cerebral wall in the left temporal to parietal region, and schizencephaly type I was diagnosed. From the clinical features of the seizures and EEG findings, he was diagnosed with symptomatic West syndrome. ACTH therapy was performed at 0.02 mg/kg day, and hypsarrythmia was improved on EEG on day 21 and the seizures disappeared. Seizures have been controlled to left facial partial seizures by anticonvulsive monotherapy with carbamazepine thereafter. Brain MRI after ACTH therapy detected cerebral cortical atrophy. On ^<99m>Tc ECD-SPECT performed at 7 months, a decreased cerebral blood flow and defect were noted in a consistent shape with the schizencephaly on the left side. Spastic paralysis of the right upper and lower limbs remains. The right ankle joint shows foot-drop, but the patient can walk with an orthosis attachment. The patient was enrolled in a general class in primary school, and mental retardation is mild. In the Discussion, the characteristics of epilepsy in schizencephaly and symptomatic West syndrome, and the cause of schizencephaly are described with a review of the literature.

A Case of Schizencephaly Type I Associated with West Syndrome that Responded to ACTH Treatment

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