ABSTRACT

The movement disorder of Huntington’s Disease (HD) consists of two components: involuntary movement and abnormal voluntary movements. Chorea, or choreoathetosis, is the movement abnormality most frequently associated with HD. HD patients occasionally develop classic obsessive-compulsive disorder, with typical symptoms such as fear of contamination or excessive handwashing. The availability of the HD gene test and consequent improved detection of late-onset HD will undoubtably lead to the ascertainment of an increasing number of cases in which HD is complicated by another disorder. The nature of the HD mutation now provides a molecular basis for understanding anticipation, the phenomenon of increasing disease severity or decreasing age-of-onset in successive generations. Atrophy of the cerebral cortex was one of the first observations made of pathological changes in HD, but was later somewhat neglected with the appreciation of the prominence of the atrophy of the striatum.