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Progressive familial leukodystrophy of late onset

From the Departments of Neurology and Pathology, University of Minnesota, Minneapolis, MN (Drs. Knopman and Sung), and the Department of Pathology, University of Kentucky, Lexington, KY (Dr. Davis).
Portions of this work were presented at the Fourth International Conference on Alzheimer's Disease, Minneapolis, MN, August 2, 1994.
Received January 27, 1995. Accepted in final form June 20, 1995.
Address correspondence to Dr. David Knopman, Department of Neurology, Box 295, University of Minnesota Hospitals, Minneapolis, MN 55455.

We report a family in which three siblings developed dementia between the ages of 40 and 70 years.Two of the siblings developed symptoms of depression, abnormal behavior, and an inability to function, progressing to severe dementia. The third sibling had a severe dementia, the clinical details of which are not available. In the two deceased siblings, neuropathologic examinations demonstrated severe demyelination, axon loss, and gliosis in cerebral white matter. Cerebellar and brainstem white matter were unaffected. Cerebral gray matter was negligibly affected. The disorder, histopathologically classified as a pigmented orthochromatic leukodystrophy, is extremely rare. Its etiology is unknown, but the pathology and familial occurrence imply that it represents a genetic defect in a function localized in the cerebral white matter.

NEUROLOGY 1996;46: 429-434

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