Dystonia-Predominant Adult-Onset Huntington Disease

Abstract

JUVENILE Huntington disease (HD), with onset before 20 years of age, is characterized by chorea that is less prominent than in the adult-onset form, as well as a variety of motor features that are more frequent in juvenile HD than in adult-onset HD, including dystonia, parkinsonism (rigidity and bradykinesia), and eye movement abnormalities.^1-9 In a study of dystonia in HD,¹⁰ several cases came to our attention of adults who, at their initial evaluation, had prominent dystonia with a relative paucity of chorea (ie, an adult-onset form of HD that resembles juvenile HD). Our goals are to estimate the prevalence of this condition in an HD clinic and to further study the association between the motor signs of HD and age of onset.