Summary

This is a Ph.D. dissertation. Huntington's Disease (HD) is a dominantly inherited late onset neurodegenerative disorder, initially affecting the basal ganglia and especially the head of the caudate nucleus. Eventually, atrophy is more diffuse extending to the cerebral cortex and other brain nuclei. A triad of progressive motor, cognitive, and emotional symptoms characterizes adult onset HD. Contents include: General Introduction and Aims of the Study, Cognitive Changes in Patients with HD and Asymptomatic Carriers of the HD Mutation, Recent Memory and Recognition Memory in Huntington's Disease, Habitual Learning in Huntington's and Parkinson's Disease, Effect of Oral Creatine Supplementation on Cognition in HD, General Summary and Perspectives.