Highlights & Basics
- Huntington disease is an autosomal dominant neurodegenerative disorder.
- Often presents in midlife but may appear at any age.
- Clinical manifestations include chorea, cognitive decline, loss of coordination, and personality change.
- Depression and suicide may be comorbid events.
- In the absence of available effective treatment, many at-risk individuals forgo predictive genetic testing.
Quick Reference
History & Exam
Key Factors
Other Factors
Diagnostics Tests
Treatment Options
Definition
Epidemiology
Etiology
Pathophysiology
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Citations
Walker FO. Huntington's disease. Lancet. 2007 Jan 20;369(9557):218-28.[Abstract]
Hayden MR. Huntington's chorea. New York, NY: Springer; 1981.
Phillips W, Shannon KM, Barker RA. The current clinical management of Huntington's disease. Mov Disord. 2008 Aug 15;23(11):1491-504.[Abstract]
Bonelli RM, Hofmann P. A systematic review of the treatment studies in Huntington's disease since 1990. Expert Opin Pharmacother. 2007 Feb;8(2):141-53.[Abstract]
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Fasano A, Bentivoglio AR. Tetrabenazine. Expert Opin Pharmacother. 2009 Dec;10(17):2883-96.[Abstract]
Quinn L, Kegelmeyer D, Kloos A, et al. Clinical recommendations to guide physical therapy practice for Huntington disease. Neurology. 2020 Feb 4;94(5):217-28.[Abstract][Full Text]
Bonelli RM, Wenning GK. Pharmacological management of Huntington's disease: an evidence-based review. Curr Pharm Des. 2006;12(21):2701-20.[Abstract]
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97. Quinn L, Kegelmeyer D, Kloos A, et al. Clinical recommendations to guide physical therapy practice for Huntington disease. Neurology. 2020 Feb 4;94(5):217-28.[Abstract][Full Text]
98. Cook C, Page K, Wagstaff A, et al; European Huntington's Disease Network. Occupational therapy for people with Huntington's disease: best practice guidelines. Jan 2012 [internet publication].[Full Text]
99. Quinn L, Busse M; European Huntington's Disease Network. Physiotherapy clinical guidelines for Huntington's disease. Neurodegen Dis Manage. 2012;2(1):21-31.[Full Text]
100. Hamilton A, Ferm U, Heemskerk AW, et al; European Huntington's Disease Network. Management of speech, language and communication difficulties in Huntington's disease. Neurodegen Dis Manage. 2012;2(1):67-77.[Full Text]
101. Brotherton A, Campos L, Rowell A, et al; Nutritional management of individuals with Huntington's disease: nutritional guidelines. Neurodegen Dis Manage. 2012;2(1):33-43.[Full Text]
102. Taylor LP, Besbris JM, Graf WD, et al. Clinical guidance in neuropalliative care: an AAN position statement. Neurology. 2022 Mar 8;98(10):409-16.[Abstract][Full Text]
103. Tarolli CG, Chesire AM, Biglan KM. Palliative care in Huntington disease: personal reflections and a review of the literature. Tremor Other Hyperkinet Mov (N Y). 2017;7:454.[Abstract][Full Text]
104. Khalil H, Quinn L, van Deursen R, et al. What effect does a structured home-based exercise programme have on people with Huntington's disease? A randomized, controlled pilot study. Clin Rehabil. 2013 Jul;27(7):646-58.[Abstract]
105. McGarry A, Auinger P, Kieburtz K, et al. Additional safety and exploratory efficacy data at 48 and 60 months from Open-HART, an open-label extension study of pridopidine in Huntington disease. J Huntingtons Dis. 2020;9(2):173-84.[Abstract]
106. McGarry A, Leinonen M, Kieburtz K, et al. Effects of pridopidine on functional capacity in early-stage participants from the PRIDE-HD study. J Huntingtons Dis. 2020;9(4):371-80.[Abstract][Full Text]
107. ClinicalTrials.gov. Pridopidine's outcome on function in Huntington disease, PROOF- HD. ClinicalTrails.gov indentifier: NCT04556656. Nov 2021 [internet publication].[Full Text]
108. Frank S, Stamler D, Sung V, et al. Deutetrabenazine effect on total motor score in patients with Huntington disease (First-HD) (S27.008). Neurology. 2016 Apr 5;86(16 Suppl):S27.008. [Full Text]
109. Claassen D, Frank S, Stamler D, et al. Rating swallowing function in patients with Huntington disease enrolled in the First-HD study (S25.006). Neurology. 2016 Apr 5;86(16 Suppl):S25.006.[Full Text]
110. Tabrizi SJ, Leavitt BR, Landwehrmeyer GB, et al. Targeting Huntingtin expression in patients with Huntington's disease. N Engl J Med. 2019 Jun 13;380(24):2307-16.[Abstract][Full Text]
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113. Huntington Study Group Reach2HD Investigators. Safety, tolerability, and efficacy of PBT2 in Huntington's disease: a phase 2, randomised, double-blind, placebo-controlled trial. Lancet Neurol. 2015 Jan;14(1):39-47.[Abstract]
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