Serial assessment of patient MT with McLeod syndrome (MLS) and a cross-sectional study in nine patients with genetically confirmed chorea-acanthocytosis (ChAc) are presented along with a review of more than 20 case reports each from the literature. The cognitive and neuropsychiatric findings may be summarized as a fronto-subcortical syndrome but are not unique to either condition since a similar syndrome is also shared by other basal ganglia degenerations, most notably Huntington's disease. Some distinction may be made on the basis of age of neuropsychiatric manifestations (MLS: fourth to fifth decade - ChAc: second to third decade) and the more pronounced features of ChAc. Correlation with the involvement of specific fronto-subcortical loops may be possible in the future.
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