The evoked potentials and cranial computed tomographic (CT) scan findings in a case of early infantile globoid cell leukodystrophy are presented. The brain stem auditory evoked responses (BAERs) and the flash visual evoked potentials (VEPs) were abnormal. Repeated cranial CT scans showed multiple areas of increased attenuation and progressive cerebral atrophy. These evoked potential and CT scan patterns occurring concomitantly in an infant are suggestive of globoid cell leukodystrophy. (J Child Neurol 1986;1:126-130)
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