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    Managing juvenile Huntington’s disease

    Quarrell, O. and Nance, M.A. and Nopoulos, P. and Paulsen, J.S. and Smith, Jonathan A. and Squitieri, F. (2013) Managing juvenile Huntington’s disease. Neurodegenerative Disease Management 3 (3), pp. 267-276. ISSN 1758-2024.

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    Abstract

    Huntington’s disease (HD) is a well-recognized progressive neurodegenerative disorder that follows an autosomal dominant pattern of inheritance. Onset is insidious and can occur at almost any age, but most commonly the diagnosis is made between the ages of 35 and 55 years. Onset ≤20 years of age is classified as juvenile HD (JHD). This age-based definition is arbitrary but remains convenient. There is overlap between the clinical pathological and genetic features seen in JHD and more traditional adult-onset HD. Nonetheless, the frequent predominance of bradykinesia and dystonia early in the course of the illness, more frequent occurrence of epilepsy and myoclonus, more widespread pathology, and larger genetic lesion means that the distinction is still relevant. In addition, the relative rarity of JHD means that the clinician managing the patient is often doing so for the first time. Management is, at best, symptomatic and supportive with few or no evidence-based guidelines. In this article, the authors will review what is known of the condition and present some suggestions based on their experience.

    Metadata

    Item Type: Article
    School: School of Science > Psychological Sciences
    Depositing User: Administrator
    Date Deposited: 21 Jan 2014 14:37
    Last Modified: 07 Apr 2016 15:59
    URI: https://eprints.bbk.ac.uk/id/eprint/9078

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