期刊
NEURODEGENERATIVE DISEASE MANAGEMENT
卷 3, 期 3, 页码 267-276出版社
FUTURE MEDICINE LTD
DOI: 10.2217/NMT.13.18
关键词
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资金
- Huntington Disease Society of America
- National Parkinson Foundation
- IMPax Pharmaceuticals
- TEVA Pharmaceuticals
- Prana Biotechnology
- Schering-Plough Research Institute
- Phytopharm
- Biotie Therapies Inc.
- Neurosearch
- Medivation/Pfizer
- NIH/National Institute of Neurological Disorders and Stroke (NINDS)
- National Center for Complimentary and Alternative Medicine (NCCAM)
- CHDI
- NIH [NS0040068]
- EHDN
- Associazione Italiana Corea di Huntington (AICH)
- Neuromed
- Telethon [GGP12218]
- NIH
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.
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