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Neurologic outcome of Aicardi syndrome (AS) is generally poor. The majority of patients attain milestones no greater than the equivalent of 6 months of age. The extent to which the underlying cerebral abnormalities vs the infantile spasms (ISs) contribute most to the poor cognitive outcome is unclear. We present two children with AS who responded to vigabatrin and had subsequent good psychomotor development.
Case reports.
Patient 1.
This girl was born at full term after a normal pregnancy. She developed normally until 4 months of age, when she started to have IS. Investigation confirmed the diagnosis of AS with a partial agenesis of the corpus callosum, cystic cerebellar lesions, bilateral chorioretinal lacunae, and colobomas.
The initial EEG showed a hypsarrhythmia variant involving primarily the left hemisphere. Complete clinical and EEG responses were achieved with 50 mg/kg/d of vigabatrin. We tried to wean her after 6 months but …
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