Oromandibular Dyskinesia as the Initial Manifestation of Late-Onset Huntington Disease

• Main Authors: Dong-Seok Oh, Eun-Seon Park, Seong-Min Choi, Byeong-Chae Kim, Myeong-Kyu Kim, Ki-Hyun Cho
• Format: Article
• Language: English
• Published: Korean Movement Disorders Society 2011-10-01
• Series: Journal of Movement Disorders
• Subjects: Late-onset Huntington disease; Intermediate CAG repeats; Oromandibular dyskinesia
• Online Access: http://e-jmd.org/upload/jmd-4-2-75-6.pdf
• ISSN: 2005-940X; 2093-4939

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.