Parkinsonism in Huntington's disease.

Abstract

Huntington's disease (HD) is usually characterized by involuntary hyperkinetic movements, called chorea. The intensity of chorea exhibits a peak in middle stages of HD and then decreases as HD progresses. In contrast, Pakinsonian signs of HD are often less appreciated. They typically progress in a fairly linear pattern over time. In fact, bradykinesia is detectable early on in premanifest gene carriers up to two decades prior to the clinical manifestation of HD symptoms using quantitative motor (Q-Motor) assessments such as finger tapping (digitomotography). Other Parkinsonian symptoms besides bradykinesia are rigidity and postural instability. They typically results in falls and injuries in advanced stages of HD. A primarily Parkinsonian motor phenotype, often seen with little to no chorea, is characteristically observed in older, late manifesting patients and in pediatric HD subjects. Establishing a diagnosis of HD is difficult in these groups and patients are often misdiagnosed in early stages.