In 13 patients, a diagnosis of adult metachro matic leukodystrophy was made. The main symptoms were dementia, behavioral abnormalities, ataxia, and polyneuropathy. In 12 patients, a diagnosis of arylsul fatase A pseudodeficiency was made. No character istic clinical syndrom e could be detected in these patients, Conclusions: Adult metachromatic leukodystrophy is a progressive metabolic disease with symptoms of demyelination of the central and peripheral nervous sys tems. Diagnosis must be confirmed by determination of arylsulfatase A activity and accumulation of sulfatides. Pseudodeficiency for arylsulfatase A can be confirmed or excluded by means of DNA analysis.

In the fall of 1988 all five animals in a herd of muskoxen (Ovibos moschatus) developed clinical signs of posterior ataxia. Postmortem investigation revealed inflammatory lesions of the caudal part of the spinal cord, mainly as leptomeningitis. Nematodes were seen in close association with the lesions. Although not identified, the parasites were probably an Elaphostrongylus sp.