The neuropsychology of Huntington's disease

The neuropsychological features of Huntington's disease (HD) include defects in concentration, egocentric spatial orientation, visuomotor integration, memory, manipulation of knowledge, and conceptual reasoning. The memory disorder of HD is prominent, but unlike that seen in amnesic syndromes. It is characterized by faulty retrieval strategies and possibly by deficits in skill learning. The qualitative features of the dementia of HD suggest that the caudate nucleus plays a major role in the spatial representation of information, the formation of plans of action, and the initiation of appropriate retrieval processes for searching working and long-term memory. As a group, individuals at risk for HD often display mild impairments similar to those affected, but these deficits have not yet been shown to be useful predictors of disease in individual cases.