2 POSSIBLE CASES OF ALEXANDER-DISEASE - MULTIMODAL EVOKED-POTENTIALS AND MRI

We describe two unrelated girls with slowly progressive leukodystrophy with macrocephaly. On MRI, both patients exhibited diffuse involvemen t of the cerebral white matter, excluding the internal capsule and opt ic radiation. Their visual-evoked potentials were normal. Short-latenc y somatosensory-evoked potentials exhibited the absence of early scalp components. Auditory brainstem responses in both patients showed intr amedullary conduction disturbance. These MRI and electrophysiological findings are similar to those in infantile Alexander disease.