The pattern of cognitive impairments in neuroacanthocytosis. A frontosubcortical dementia

L. D. Kartsounis and R. J. Hardie
Department of Clinical Neuropsychology, National Hospital for Neurology and Neurosurgery, London, England.

OBJECTIVE: To identify the neuropsychological profiles of patients with neuroacanthocytosis. PATIENTS: The medical files of 19 previously reported 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. DATA EXTRACTION: Data on discrepancies between estimated optimal and obtained measures of general intelligence and scores on focal cognitive tests 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 presented with memory impairment, two of whom showed visuoperceptual deficits. None of the patients showed any significant high-level language deficits. The most consistent findings across cases was evidence of impairment in frontal lobe executive skills and psychiatric morbidity. CONCLUSION: The cognitive and psychiatric features of the patients suggests that neuroacanthocytosis is a frontosubcortical type of dementia.