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Adult metachromatic leukodystrophy

Neurophysiologic findings

From the Laboratory of Clinical Neurophysiology, Rigshospitalet, University Hospital of Copenhagen, Denmark.

The visual and somatosensory evoked potentials were delayed in two cases of the adult form of metachromatic leukodystrophy. Brainstem auditory evoked potentials were normal. The conduction velocity along peripheral nerves was 50% slowed in one case and near normal in the other. The findings are compatible with demyelination in the central and peripheral nervous systems. The diagnosis of metachromatic leukodystrophy should be considered in cases of early dementia, with or without psychosis or other neurologic deficits, in which evoked potentials are delayed and peripheral nerve conduction is slowed.

Address correspondence and reprint requests to Dr. Wulff, Department of Neurophysiology, University Hospital, DK 5000 Odense C, Denmark.

Accepted for publication March 7, 1985.

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