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Cerebral involvement in McLeod syndrome

Neurologische Klinik (Drs. Danek and Witt, and I. Uttner) and Radiologische Klinik (Drs. Vogl and Tatsch), Ludwig-Maximilians-Universität Munich, Germany.

McLeod syndrome is an Xp21-linked Kell blood group variant due to lack of erythrocyte protein Kx with associated RBC membrane dysfunction such as acanthocytosis. A man with this syndrome developed chorea and slight neuropsychological impairment. He had caudate atrophy on cerebral imaging and reduced striatal dopamine D²-receptor binding on single-photon emission computed tomography. Since Xp21 was partly deleted in the patient, the missing gene product (possibly Kx) may be essential for the integrity of the striatum.

Address correspondence and reprint requests to Dr. A. Danek, Neurologische Klinik, Postfach 701260, D-81377 München, Germany.

Received May 10, 1993. Accepted for publication in final form June 15, 1993.

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