Journal article
Managing juvenile Huntington's disease
Neurodegenerative disease management, Vol.3(3), pp.267-276
06/01/2013
DOI: 10.2217/NMT.13.18
PMCID: PMC3883192
PMID: 24416077
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 fdoing 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.
Details
- Title: Subtitle
- Managing juvenile Huntington's disease
- Creators
- Oliver W. J. Quarrell - Sheffield Childrens Hosp, Dept Clin Genet, Sheffield, S Yorkshire, EnglandMartha A. Nance - Struthers Parkinson's Center, 6701 Country Club Drive, Golden Valley, MN 55427, USA.Peggy Nopoulos - Univ Iowa, Carver Coll Med, Iowa City, IA 52242 USAJane S. Paulsen - University of IowaJonathan A. Smith - Birkbeck, University of LondonFerdinando Squitieri
- Resource Type
- Journal article
- Publication Details
- Neurodegenerative disease management, Vol.3(3), pp.267-276
- DOI
- 10.2217/NMT.13.18
- PMID
- 24416077
- PMCID
- PMC3883192
- NLM abbreviation
- Neurodegener Dis Manag
- ISSN
- 1758-2024
- eISSN
- 1758-2032
- Publisher
- Future Medicine Ltd
- Number of pages
- 10
- Grant note
- IMPax Pharmaceuticals Huntington Disease Society of America CHDI NIH; United States Department of Health & Human Services; National Institutes of Health (NIH) - USA TEVA Pharmaceuticals; Teva Pharmaceutical Industries NIH/National Institute of Neurological Disorders and Stroke (NINDS); United States Department of Health & Human Services; National Institutes of Health (NIH) - USA; NIH National Institute of Neurological Disorders & Stroke (NINDS) Prana Biotechnology Schering-Plough Research Institute; Merck & Company NS0040068 / NIH; United States Department of Health & Human Services; National Institutes of Health (NIH) - USA Biotie Therapies Inc. Neuromed R01NS055903 / NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE; United States Department of Health & Human Services; National Institutes of Health (NIH) - USA; NIH National Institute of Neurological Disorders & Stroke (NINDS) Neurosearch Associazione Italiana Corea di Huntington (AICH) EHDN National Center for Complimentary and Alternative Medicine (NCCAM); United States Department of Health & Human Services; National Institutes of Health (NIH) - USA; NIH National Center for Complementary & Alternative Medicine GGP12218 / Telethon; Fondazione Telethon National Parkinson Foundation Phytopharm Medivation/Pfizer; Pfizer
- Language
- English
- Date published
- 06/01/2013
- Academic Unit
- Neurology; Psychiatry; Stead Family Department of Pediatrics; Psychological and Brain Sciences; Iowa Neuroscience Institute
- Record Identifier
- 9984384319102771
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