THE PATTERN OF COGNITIVE IMPAIRMENTS IN NEUROACANTHOCYTOSIS - A FRONTOSUBCORTICAL DEMENTIA

Objective: To identify the neuropsychological profiles of patients wit h neuroacanthocytosis. Patients: The medical files of 19 previously re ported cases of neuroacanthocytosis were reviewed. Ten of the patients involved had undergone comprehensive cognitive assessments, and their neuropsychological records formed the basis of the present study. Dat a Extraction: Data on discrepancies between estimated optimal and obta ined measures of general intelligence and scores on focal cognitive te sts of memory, language, visual perception, and frontal lobe executive skills were tabulated and interpreted. Results: Eight of the patients had evidence of general intellectual deterioration. Five patients pre sented with memory impairment, two of whom showed visuoperceptual defi cits. None of the patients showed any significant high-level language deficits. The most consistent findings across cases was evidence of im pairment in frontal lobe executive skills and psychiatric morbidity. C onclusion: The cognitive and psychiatric features of the patients sugg ests that neuroacanthocytosis is a frontosubcortical type of dementia.