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NEUROLOGY 1987;37:645
© 1987 American Academy of Neurology

Chorea-amyotrophy with chronic hemolytic anemia

A variant of chorea-amyotrophy with acanthocytosis

Selden E. Spencer, MD, Francis O. Walker, MD and Steven A. Moore, MD, PhD

Departments of Neurology (Dr. Spencer) and Pathology (Dr. Moore), University of Iowa College of Medicine and Veterans Administration Medical Center, Iowa City, IA; and the Department of Neurology (Dr. Walker), Wake Forest University, Bowman Gray School of Medicine, Winston-Salem, NC.

We studied two siblings with chorea and amyotrophy of adult onset and spherocytic hemolytic anemia. Autopsy revealed an atrophic striatum with iron deposition and spheroid bodies. Degeneration of the substantia nigra and spinal cord anterior horns was seen without iron deposition. The disorder seems to be a variant of the chorea-amyotrophy-acanthocytosis syndrome, and the pathology may account for evidence of parkinsonism and amyotrophy in that syndrome. Acanthocytosis may not be the only hematologic abnormality in this disorder.

Address correspondence and reprint requests to Dr. Spencer, Department of Neurology, Washington University, 660 S. Euclid, St. Louis, MO 63110.

Received May 27, 1986. Accepted for publication in final form August 12, 1986.




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