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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 a single-photon emission computed tomography.Since Xp21 was partly deleted in the patient, the mixing gene product(possibly Kx)may be essential for the integrity of the striatum. |
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