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NEUROLOGY 1994;44:662
© 1994 American Academy of Neurology

Characteristics of the dementia in late-onset metachromatic leukodystrophy

Elsa G. Shapiro, PhD, Lawrence A. Lockman, MD, David Knopman, MD and William Krivit, MD, PhD

Departments of Neurology, University of Minnesota, Minneapolis, MN (Drs. Shapiro, Lockman, and Knopman)
Pediatrics, University of Minnesota, Minneapolis, MN (Drs. Shapiro, Lockman, and Krivit).

Patients with metachromatic leukodystrophy (MLD) of juvenile or adult onset present with behavioral abnormalities. In nine patients, diagnosed between ages 11 and 33 years, behavior and neuropsychological test results disclosed a pattern of dementia combining features associated with both frontal and white matter abnormalities. All the patients had been considered to have a psychiatric disorder prior to the diagnosis of MLD, even though none had any of the cardinal features of schizophrenia or other major psychosis. Early diagnosis of late-onset MLD is important to provide access to appropriate effective therapy.

Address correspondence and reprint requests to Dr. Elsa G. Shapiro, Division of Pediatric Neurology, University of Minnesota, Box 486 UMHC, 420 Delaware St. S.E., Minneapolis, MN 55455.

Supported in part by NINDS grant 1 RO1 NS29099-01.

Received June 4, 1993. Accepted for publication in final form September 23, 1993.




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