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Metachromatic leukodystrophy

Comparison of early-and late-onset forms

Department of Neurology, The Johns Hopkins University School of Medicine, Baltimore.

Comparative study of peripheral nerve biopsies and cultured skin fibroblasts from patients with late infantile and early adult-onset forms of metachromatic leukodystrophy revealed similar pathologic changes in the early stages of nerve degeneration. However, the peripheral nerves in the adult-onset cases eventually reached a more chronically demyelinated fibrotic state than did nerves in the infantile cases. Cultured skin fibroblasts from the adult-onset patients, although clearly abnormal, were able to catabolize sulfatide significantly more effectively than the cultured skin fibroblasts from late infantile patients.

Reprint requests should be addressed to Dr. Percy, Charles R. Drew Postgraduate Medical School, Los Angeles, CA 90059.

Dr. Kaback's address is Harbor General Hospital Campus, UCLA, Torrance, CA 90509.

Supported in part by USPHS grant No. NS 08997 (Dr. Hemdon) and a grant from the John A. Hartford Foundation.

Dr. Percy was recipent of Teacher-Investigator Award NS 11,016 USPHS.

Accepted for publication October 7, 1976.

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