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Hereditary adult-onset Alexander's disease with palatal myoclonus, spastic paraparesis, and cerebellar ataxia

From the Veterans Administration Outpatient Clinic (Dr. Schwankhaus) and the Department of Neurology (Dr. Schwankhaus), Texas Tech University Health Sciences Center, Lubbock, TX; the Department of Laboratory Medicine and Pathology (Neuropathology) (Dr. Parisi), Mayo Clinic, Rochester, MN; Ft. Worth, TX (Drs. Gulledge and Chin); and the Department of Neurology (Dr. Currier), University of Mississippi Medical Center, Jackson, MS.
Received November 8, 1994. Accepted in final form April 4, 1995.
Address correspondence and reprint requests to Dr. John Schwankhaus, Southwest Neurosciences Center, Peter O. Thomas Medical Arts Building, Suite 305, 11321 Interstate 30, Little Rock, AR 72209.

We describe a progressive neurologic disorder in three sisters characterized clinically by palatal myoclonus, spastic weakness, hyperreflexia, mild cerebellar dysfunction, and ocular motor abnormalities.Postmortem examination of one patient demonstrated widespread Rosenthal fiber deposition associated with demyelination. The father previously was reported to have similar pathologic findings and carried a clinical diagnosis of multiple sclerosis. These clinical and pathologic findings describe a rare familial leukodystrophy that corresponds most closely to cases reported as adult Alexander's disease. Although similar pathologically to the well-characterized infantile variant of Alexander's disease, it is not known whether this adult variant represents the same disease process.

NEUROLOGY 1995;45: 2266-2271

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