[Neuropsychological deterioration in Huntington's disease]

Huntington's disease (HD) is a neurodegenerative illness, autosomal dominantly inherited, that produces characteristic involuntary muscle movements (chorea) with cognitive abnormalities and personality.

Neuropathological studies report premature cell death principally affecting the basal ganglia. The disease most commonly begins in the fourth or fifth decade of life, although in some cases, abnormalities have been documented in early childhood and as late as 50 years of age. Early cognitive deterioration in HD is not uniform, and patients with recently diagnosed HD may evidence selective deficits in learning and visuospatial/visuomotor ability, along with a relatively consistent pattern of impairment on subtests of the Wechsler Adult Intelligence Scale (WAIS). Neuroimaging reports of basal ganglia function have described striatal abnormality in HD and related with neuropsychological impairment.

This paper provides the clinical-phenomenologic definition of the neuropsychological impairment and its associated features related with its neuropathological significance and to review the subject in the light of present day reports.