ABSTRACT

References 737

Huntington’s disease (HD) is an autosomal-dominant progressive neurodegenerative disorder beginning classically in mid-life with the characteristic triad of clinical features (Folstein, 1989) – motor disorder, cognitive disorder and emotional disorder. Within this characteristic triad, there is variety in the clinical phenotype with respect to timing of onset, symptoms and progression. Death usually occurs 20-30 years after the onset of motor symptoms.