The X-linked McLeod neuroacanthocytosis syndrome strongly resembles Huntington's 
disease and has been reported in various countries world-wide. Herein, we report 
two Chilean brothers with predominant psychiatric features at disease onset 
including schizophrenia-like psychosis and obsessive compulsive disorder. 
Molecular genetic analysis revealed a small deletion in the XK gene 
(938-942delCTCTA), which has been already described in a North American patient 
of Anglo-Saxon descent and a Japanese family, presenting with seizures, muscle 
atrophy or chorea yet absence of psychiatric features. These findings argue 
against a founder effect and indicate a profound phenotypic variability 
associated with the 938-942delCTCTA deletion. Our report supports the inclusion 
of McLeod syndrome in the differential diagnosis of Huntington's disease as well 
as acute psychosis in male subjects.