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.