Olfactory functions in asymptomatic carriers of the Huntington disease mutation.

Huntington's disease (HD) is a neurodegenerative disorder initially affecting the basal ganglia and especially the head of the caudate nucleus. Neuropsychological research has indicated that olfactory dysfunction may appear early in HD, prior to the onset of significant motor or cognitive dysfunction. The aim of this study was to examine whether asymptomatic carriers of the Huntington disease mutation also exhibit olfactory dysfunction. To address this issue we presented an extensive olfactory test battery comprising tasks assessing olfactory sensitivity, intensity discrimination, quality discrimination, episodic odor memory, and odor identification, to a group of gene carriers and nonmutation carriers of the disease. The results showed that gene carriers were selectively impaired in discriminating odor quality, although performance did not differ from noncarriers across the other tasks. The role played by striatum and then in particular the caudate nucleus for olfactory processing in general, and for odor quality discrimination in particular, is discussed.