title: Oromandibular dyskinesia as the initial manifestation of late-onset huntington disease.
abstract: Huntington's disease (HD) is a neurodegenerative disorder characterized by a triad of choreoathetosis, dementia and dominant inheritance. The cause of HD is an expansion of CAG trinucleotide repeats in the HD gene. Typical age at onset of symptoms is in the 40s, but the disorder can manifest at any time. Late-onset (≥ 60 years) HD is clinically different from other adult or juvenile onset HD and characterized by mild motor problem as the initial symptoms, shorter disease duration, frequent lack of family history, and relatively low CAG repeats expansion. We report a case of an 80-year-old female with oromandibular dyskinesia as an initial manifestation of HD and 40 CAG repeats.
authors.affiliation: Department of Neurology, Chonnam National University Medical School, Gwangju, Korea.
authors.forename: Dong-Seok
authors.initials: DS
authors.lastname: Oh
authors.affiliation: Department of Neurology, Chonnam National University Medical School, Gwangju, Korea.
authors.forename: Eun-Seon
authors.initials: ES
authors.lastname: Park
authors.affiliation: Department of Neurology, Chonnam National University Medical School, Gwangju, Korea.
authors.forename: Seong-Min
authors.initials: SM
authors.lastname: Choi
authors.affiliation: Department of Neurology, Chonnam National University Medical School, Gwangju, Korea.
authors.forename: Byeong-Chae
authors.initials: BC
authors.lastname: Kim
authors.affiliation: Department of Neurology, Chonnam National University Medical School, Gwangju, Korea.
authors.forename: Myeong-Kyu
authors.initials: MK
authors.lastname: Kim
authors.affiliation: Department of Neurology, Chonnam National University Medical School, Gwangju, Korea.
authors.forename: Ki-Hyun
authors.initials: KH
authors.lastname: Cho
keywords: Intermediate CAG repeats; Late-onset Huntington disease; Oromandibular dyskinesia