Juvenile-onset Huntington's disease - Spectrum and evolution of presenting movement disorders.

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  • Additional Information
    • Source:
      Publisher: Wiley Periodicals, Inc on behalf of American Neurological Association Country of Publication: United States NLM ID: 101623278 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 2328-9503 (Electronic) Linking ISSN: 23289503 NLM ISO Abbreviation: Ann Clin Transl Neurol Subsets: MEDLINE
    • Publication Information:
      Original Publication: [Hoboken, NJ] : Wiley Periodicals, Inc on behalf of American Neurological Association, [2014]-
    • Subject Terms:
      Huntington Disease*/diagnosis ; Huntington Disease*/physiopathology ; Movement Disorders*/diagnosis ; Movement Disorders*/physiopathology ; Age of Onset*; Humans ; Male ; Female ; Adolescent ; Child ; Disease Progression ; Young Adult ; Dystonia/diagnosis ; Dystonia/physiopathology ; Dystonia/etiology ; Chorea/diagnosis ; Chorea/physiopathology ; Chorea/etiology
    • Abstract:
      Juvenile-onset Huntington's disease (HD) is a rare subset of HD with symptom-onset before the age of 18. In contrast to the adult population, children present early-on with behavioral, psychiatric, and cognitive symptoms, in addition to a diverse spectrum of movement disorders. This poses a distinct challenge in diagnosis and management. We here describe the spectrum of movement disorders, accompanied with detailed video recordings, in seven cases of juvenile-onset HD. Our findings highlight early cognitive and behavioral symptoms, preceding motor symptoms. The diverse movement disorder phenotypes included dystonia, Parkinsonism, myoclonus, and chorea, findings which underscore the heterogeneity of presenting symptoms.
      (© 2024 The Author(s). Annals of Clinical and Translational Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association.)
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    • Grant Information:
      K08 NS123552 United States NS NINDS NIH HHS; K08NS123552 National Institutes of Health/National Institute of Neurological Disorders and Stroke; International Parkinson and Movement Disorder Society
    • Publication Date:
      Date Created: 20240906 Date Completed: 20241028 Latest Revision: 20241106
    • Publication Date:
      20241107
    • Accession Number:
      PMC11514935
    • Accession Number:
      10.1002/acn3.52193
    • Accession Number:
      39239850