Huntington’s disease presenting as adult-onset tourettism

Abstract

Huntington’s disease (HD) is an autosomal dominant progressive neurodegenerative disease, caused by trinucleotide repeat expansion (CAG) in the Huntingtin gene (HTT) on chromosome 4. It is typically characterized by the combination of chorea with or without other extrapyramidal symptoms, oculomotor abnormalities, cognitive decline, and neuropsychiatric manifestations. However, HD consists of considerable phenotypic variability. Though chorea is the most common extrapyramidal manifestation, it is also associated with other movement disorders such as dystonia, myoclonus, tics, parkinsonism, and ataxia.