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NEUROLOGY 1991;41:1000
© 1991 American Academy of Neurology

Diagnostic tests for choreoacanthocytosis

T. E. Feinberg, MD, C. D. Cianci, MS, J. S. Morrow, MD, J. C. Pehta, MD, C. M. Redman, PhD, T. Huima and W. J. Koroshetz, MD

Departments of Neurology and Psychiatry (Dr. Feinberg), Beth Israel Medical Center and Mount Sinai School of Medicine, New York, NY; Department of Pathology (C.D. Cianci and Dr. Morrow), Yale University School of Medicine, New Haven, CT; the New York Blood Center (Drs. Pehta and Redman, and T. Huima), New York, NY; and Department of Neurology (Dr. Koroshetz), Massachusetts General Hospital, Boston, MA.

Two patients with striatal atrophy and a clinical syndrome consistent with choreoacanthocytosis had normal dried blood smears but their red cells demonstrated an abnormal sensitivity to various conditions known to promote discocyte-echinocyte transformation. Dilution in normal saline, in vitro aging, and contact with glass caused a great proportion of these patients' red cells to develop multiple spiny or rounded projections. Under identical conditions, such shape changes did not occur in normal patients or in those with Huntington's disease. Scanning electron microscopy showed that the age-induced increase in acanthocytic-appearing cells could be reversed with chlorpromazine. These data suggest that the red cells from these patients with striatal degeneration are deficient in their ability to preserve normal shape in the face of echinocytic stress and that this observation has diagnostic and, possibly, pathophysiologic significance.

Address correspondence and reprint requests to Dr. Todd E. Feinberg, Neurobehavior Center, Beth Israel Medical Center, 317 East 17th Street, New York, NY 10003, or to Dr. Walter J. Koroshetz, Huntington's Disease Center, Massachusetts General Hospital, 50 Blossom Street, Boston, MA 02114.

Supported in part by grant HL33841 from the National Institutes of Health and a grant from the Horace W. Goldsmith Foundation.

Presented in part at the 42nd annual meeting of the American Academy of Neurology, Miami Beach, FL, April 1990.

Received November 16, 1990. Accepted for publication in final form December 27, 1990.




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Early Clinical Heterogeneity in Choreoacanthocytosis
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[Abstract] [Full Text] [PDF]


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