CEREBRAL INVOLVEMENT IN MCLEOD SYNDROME

McLeod syndrome is an Xp21-linked Kell blood group variant due to lack of erythrocyte protein Kx with associated RBC membrane dysfunction su ch as acanthocytosis. A man with this syndrome developed chorea and sl ight neuropsychological impairment. He had caudate atrophy on cerebral imaging and reduced striatal dopamine D-2-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 f or the integrity of the striatum.