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1 "Intermediate CAG repeats"
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Case Report
Oromandibular Dyskinesia as the Initial Manifestation of Late-Onset Huntington Disease
Dong-Seok Oh, Eun-Seon Park, Seong-Min Choi, Byeong-Chae Kim, Myeong-Kyu Kim, Ki-Hyun Cho
J Mov Disord. 2011;4(2):75-77.
DOI: https://doi.org/10.14802/jmd.11016
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AbstractAbstract PDF

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.

Citations

Citations to this article as recorded by  
  • The oral manifestations of Huntington's disease: A systematic review of prevalence
    Luciana Munhoz, Ashjan Qasim Jabbar, William José e Silva Filho, Aline Yukari Nagai, Emiko Saito Arita
    Oral Diseases.2023; 29(1): 62.     CrossRef
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    Internal Medicine.2023; 62(1): 119.     CrossRef
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    Mohamed Z. Habib, Mariane G. Tadros, Hadwa A. Abd-Alkhalek, Magda I. Mohamad, Dalia M. Eid, Fatma E. Hassan, Hend Elhelaly, Yasser el Faramawy, Sawsan Aboul-Fotouh
    European Journal of Pharmacology.2022; 927: 175046.     CrossRef
  • Management of Traumatic Ulcerations of Lips in a Case of Huntington’s Disease: A Novel Application of Essix Retainer
    Mohamed Iqbal J
    Journal of Indian Orthodontic Society.2021; 55(4): 415.     CrossRef

JMD : Journal of Movement Disorders