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.
- The duration of disease is approximately 20 years from time of diagnosis to time of death.
- Pretest genetic counseling should be arranged prior to ordering genetic testing.
Quick Reference
History & Exam
Key Factors
Other Factors
Diagnostics Tests
Treatment Options
Definition
Epidemiology
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Citations
Bean L, Bayrak-Toydemir P; American College of Medical Genetics and Genomics. Standards and guidelines for clinical genetics laboratories, 2014 edition: technical standards and guidelines for Huntington disease. Genet Med. 2014 Dec;16(12):e2.[Abstract][Full Text]
Anderson KE, van Duijn E, Craufurd D, et al. Clinical management of neuropsychiatric symptoms of Huntington disease: expert-based consensus guidelines on agitation, anxiety, apathy, psychosis and sleep disorders. J Huntingtons Dis. 2018;7(3):355-66.[Abstract][Full Text]
Bachoud-Lévi AC, Ferreira J, Massart R, et al. International guidelines for the treatment of Huntington's disease. Front Neurol. 2019;10:710.[Abstract][Full Text]
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]
1. Walker FO. Huntington's disease. Lancet. 2007 Jan 20;369(9557):218-28.[Abstract]
2. Gonzalez-Alegre P, Afifi AK. Clinical characteristics of childhood-onset (juvenile) Huntington disease: report of 12 patients and review of the literature. J Child Neurol. 2006 Mar;21(3):223-9.[Abstract]
3. Medina A, Mahjoub Y, Shaver L, et al. Prevalence and incidence of Huntington's disease: an updated systematic review and meta-analysis. Mov Disord. 2022 Dec;37(12):2327-35.[Abstract][Full Text]
4. Bruzelius E, Scarpa J, Zhao Y, et al. Huntington's disease in the United States: variation by demographic and socioeconomic factors. Mov Disord. 2019 Jun;34(6):858-65.[Abstract][Full Text]
5. Rawlins M. Huntington's disease out of the closet? Lancet. 2010 Oct 23;376(9750):1372-3.[Abstract]
6. Roos RA. Huntington's disease: a clinical review. Orphanet J Rare Dis. 2010 Dec 20;5:40.[Abstract][Full Text]
7. Folstein S. Huntington's disease: a disorder of families. Baltimore, MD: Johns Hopkins University Press; 1989.
8. Pridmore SA. The large Huntington's disease family of Tasmania. Med J Aust. 1990 Nov 19;153(10):593-5.[Abstract]
9. Harper PS, Jones L. Huntington's disease: genetic and molecular studies. In: Bates G, Harper P, Jones L, eds. Huntington's disease. New York: Oxford University Press; 2002:113-58.
10. Wright HH, Still CN, Abramson RK. Huntington's disease in black kindreds in South Carolina. Arch Neurol. 1981 Jul;38(7):412-4.[Abstract]
11. Bean L, Bayrak-Toydemir P; American College of Medical Genetics and Genomics. Standards and guidelines for clinical genetics laboratories, 2014 edition: technical standards and guidelines for Huntington disease. Genet Med. 2014 Dec;16(12):e2.[Abstract][Full Text]
12. Wanker E, Dröge A. Structural biology of Huntington's disease. In: Bates G, Harper P, Jones L, eds. Huntington's disease. New York: Oxford University Press; 2002:327-47.
13. Marchut AJ, Hall CK. Effects of chain length on the aggregation of model polyglutamine peptides: molecular dynamics simulations. Proteins. 2007 Jan 1;66(1):96-109.[Abstract]
14. Oliveira JM, Jekabsons MB, Chen S, et al. Mitochondrial dysfunction in Huntington's disease: the bioenergetics of isolated and in situ mitochondria from transgenic mice. J Neurochem. 2007 Apr;101(1):241-9.[Abstract]
15. Stack EC, Smith KM, Ryu H, et al. Combination therapy using minocycline and coenzyme Q10 in R6/2 transgenic Huntington's disease mice. Biochim Biophys Acta. 2006 Mar;1762(3):373-80.[Abstract]
16. Graham RK, Deng Y, Slow EJ, et al. Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin. Cell. 2006 Jun 16;125(6):1179-91.[Abstract]
17. Peng Q, Masuda N, Jiang M, et al. The antidepressant sertraline improves the phenotype, promotes neurogenesis and increases BDNF levels in the R6/2 Huntington's disease mouse model. Exp Neurol. 2008 Mar;210(1):154-63.[Abstract]
18. Reiner A, Deng YP. Disrupted striatal neuron inputs and outputs in Huntington's disease. CNS Neurosci Ther. 2018 Apr;24(4):250-80.[Abstract][Full Text]
19. Stober T, Wussow W, Schimrigk K. Bicaudate diameter: the most specific and simple CT parameter in the diagnosis of Huntington's disease. Neuroradiology. 1984;26(1):25-8.[Abstract]
20. Rüb U, Seidel K, Heinsen H, et al. Huntington's disease (HD): the neuropathology of a multisystem neurodegenerative disorder of the human brain. Brain Pathol. 2016 Nov;26(6):726-40.[Abstract][Full Text]
21. Cepeda C, Wu N, André VM, et al. The corticostriatal pathway in Huntington's disease. Prog Neurobiol. 2007 Apr;81(5-6):253-71.[Abstract][Full Text]
22. Li JL, Hayden MR, Warby SC, et al. Genome-wide significance for a modifier of age at neurological onset in Huntington's disease at 6q23-24: the HD MAPS study. BMC Med Genet. 2006 Aug 17;7:71.[Abstract][Full Text]
23. Almqvist EW, Elterman DS, MacLeod PM, et al. High incidence rate and absent family histories in one quarter of patients newly diagnosed with Huntington disease in British Columbia. Clin Genet. 2001 Sep;60(3):198-205.[Abstract]
24. Ramos-Arroyo MA, Moreno S, Valiente A. Incidence and mutation rates of Huntington's disease in Spain: experience of 9 years of direct genetic testing. J Neurol Neurosurg Psychiatry. 2005 Mar;76(3):337-42.[Abstract][Full Text]
25. van Duijn E, Kingma EM, van der Mast RC. Psychopathology in verified Huntington's disease gene carriers. J Neuropsychiatry Clin Neurosci. 2007 Fall;19(4):441-8.[Abstract]
26. Walker FO. Huntington's disease. Semin Neurol. 2007 Apr;27(2):143-50.[Abstract]
27. Andrich J, Saft C, Ostholt N, et al. Assessment of simple movements and progression of Huntington's disease. J Neurol Neurosurg Psychiatry. 2007 Apr;78(4):405-7.[Abstract]
28. Marshall J, White K, Weaver M, et al. Specific psychiatric manifestations among preclinical Huntington disease mutation carriers. Arch Neurol. 2007 Jan;64(1):116-21.[Abstract][Full Text]
29. Craufurd D, Thompson JC, Snowden JS. Behavioral changes in Huntington Disease. Neuropsychiatry Neuropsychol Behav Neurol. 2001 Oct-Dec;14(4):219-26.[Abstract]
30. Paulsen JS, Nehl C, Hoth KF, et al. Depression and stages of Huntington's disease. J Neuropsychiatry Clin Neurosci. 2005 Fall;17(4):496-502.[Abstract]
31. Herzog-Krzywoszanska R, Krzywoszanski L. Sleep disorders in Huntington's disease. Front Psychiatry. 2019;10:221.[Abstract][Full Text]
32. Anderson KE, van Duijn E, Craufurd D, et al. Clinical management of neuropsychiatric symptoms of Huntington disease: expert-based consensus guidelines on agitation, anxiety, apathy, psychosis and sleep disorders. J Huntingtons Dis. 2018;7(3):355-66.[Abstract][Full Text]
33. Craufurd D, MacLeod R, Frontali M, et al. Diagnostic genetic testing for Huntington's disease. Pract Neurol. 2015 Feb;15(1):80-4.[Full Text]
34. MacLeod R, Tibben A, Frontali M, et al; Editorial Committee and Working Group 'Genetic Testing Counselling' of the European Huntington Disease Network. Recommendations for the predictive genetic test in Huntington's disease. Clin Genet. 2013 Mar;83(3):221-31.[Abstract]
35. Losekoot M, van Belzen MJ, Seneca S, et al; European Molecular Genetic Quality Network (EMQN). EMQN/CMGS best practice guidelines for the molecular genetic testing of Huntington disease. Eur J Hum Genet. 2013 May;21(5):480-6.[Abstract][Full Text]
36. Stoker TB, Mason SL, Greenland JC, et al. Huntington's disease: diagnosis and management. Pract Neurol. 2022 Feb;22(1):32-41.[Abstract]
37. Blekher T, Johnson SA, Marshall J, et al. Saccades in presymptomatic and early stages of Huntington disease. Neurology. 2006 Aug 8;67(3):394-9.[Abstract]
38. Gordon AM, Quinn L, Reilmann R, et al. Coordination of prehensile forces during precision grip in Huntington's disease. Exp Neurol. 2000 May;163(1):136-48.[Abstract]
39. Hoff JI, van Hilten BJ, Roos RA. A review of the assessment of dyskinesias. Mov Disord. 1999 Sep;14(5):737-43.[Abstract]
40. Burke JR, Wingfield MS, Lewis KE, et al. The Haw River syndrome: dentatorubropallidoluysian atrophy (DRPLA) in an African-American family. Nat Genet. 1994 Aug;7(4):521-4.[Abstract]
41. Dobson-Stone C, Velayos-Baeza A, Filippone LA, et al. Chorein detection for the diagnosis of chorea-acanthocytosis. Ann Neurol. 2004 Aug;56(2):299-302.[Abstract]
42. Breedveld GJ, Percy AK, MacDonald ME, et al. Clinical and genetic heterogeneity in benign hereditary chorea. Neurology. 2002 Aug 27;59(4):579-84.[Abstract]
43. Schrag A, Quinn NP, Bhatia KP, et al. Benign hereditary chorea - entity or syndrome? Mov Disord. 2000 Mar;15(2):280-8.[Abstract]
44. Hewer E, Danek A, Schoser BG, et al. McLeod myopathy revisited: more neurogenic and less benign. Brain. 2007 Dec;130(Pt 12):3285-96.[Abstract]
45. Stevanin G, Fujigasaki H, Lebre AS, et al. Huntington's disease-like phenotype due to trinucleotide repeat expansions in the TBP and JPH3 genes. Brain. 2003 Jul;126(Pt 7):1599-603.[Abstract][Full Text]
46. Schöls L, Bauer P, Schmidt T, et al. Autosomal dominant cerebellar ataxias: clinical features, genetics, and pathogenesis. Lancet Neurol. 2004 May;3(5):291-304.[Abstract]
47. Laplanche JL, Hachimi KH, Durieux I, et al. Prominent psychiatric features and early onset in an inherited prion disease with a new insertional mutation in the prion protein gene. Brain. 1999 Dec;122 ( Pt 12):2375-86.[Abstract][Full Text]
48. Al-Tahan AY, Divakaran MP, Kambouris M, et al. A novel autosomal recessive 'Huntington's disease-like' neurodegenerative disorder in a Saudi family. Saudi Med J. 1999 Jan;20(1):85-9.[Abstract]
49. Kambouris M, Bohlega S, Al-Tahan A, et al. Localization of the gene for a novel autosomal recessive neurodegenerative Huntington-like disorder to 4p15.3. Am J Hum Genet. 2000 Feb;66(2):445-52.[Abstract][Full Text]
50. Hartig MB, Hörtnagel K, Garavaglia B, et al. Genotypic and phenotypic spectrum of PANK2 mutations in patients with neurodegeneration with brain iron accumulation. Ann Neurol. 2006 Feb;59(2):248-56.[Abstract]
51. Chinnery PF, Crompton DE, Birchall D, et al. Clinical features and natural history of neuroferritinopathy caused by the FTL1 460InsA mutation. Brain. 2007 Jan;130(Pt 1):110-9.[Abstract][Full Text]
52. Isaacs JD, Rakshi J, Baker R, et al. Chorea associated with thyroxine replacement therapy. Mov Disord. 2005 Dec;20(12):1656-7.[Abstract]
53. Dike GL. Chorea gravidarum: a case report and review. Md Med J. 1997 Sep;46(8):436-9.[Abstract]
54. Cardoso F, Eduardo C, Silva AP, et al. Chorea in fifty consecutive patients with rheumatic fever. Mov Disord. 1997 Sep;12(5):701-3.[Abstract]
55. Font J, Cervera R, Espinosa G, et al. Systemic lupus erythematosus (SLE) in childhood: analysis of clinical and immunological findings in 34 patients and comparison with SLE characteristics in adults. Ann Rheum Dis. 1998 Aug;57(8):456-9.[Abstract][Full Text]
56. Cervera R, Asherson RA, Font J, et al. Chorea in the antiphospholipid syndrome. Clinical, radiologic, and immunologic characteristics of 50 patients from our clinics and the recent literature. Medicine (Baltimore). 1997 May;76(3):203-12.[Abstract]
57. Machado A, Chien HF, Deguti MM, et al. Neurological manifestations in Wilson's disease: report of 119 cases. Mov Disord. 2006 Dec;21(12):2192-6.[Abstract]
58. Shoulson I, Fahn S. Huntington disease: clinical care and evaluation. Neurology. 1979 Jan;29(1):1-3.[Abstract]
59. Huntington Study Group. Unified Huntington's Disease Rating Scale: reliability and consistency. Mov Disord. 1996 Mar;11(2):136-42.[Abstract]
60. Crozier S, Robertson N, Dale M. The psychological impact of predictive genetic testing for Huntington's disease: a systematic review of the literature. J Genet Couns. 2015 Feb;24(1):29-39.[Abstract][Full Text]
61. Ross CA, Reilmann R, Cardoso F, et al. Movement Disorder Society Task Force Viewpoint: Huntington's Disease Diagnostic Categories. Mov Disord Clin Pract. 2019 Sep;6(7):541-6.[Abstract][Full Text]
62. Mühlbӓck A, van Walsem M, Nance M, et al. What we don't need to prove but need to do in multidisciplinary treatment and care in Huntington's disease: a position paper. Orphanet J Rare Dis. 2023 Jan 30;18(1):19.[Abstract][Full Text]
63. Bachoud-Lévi AC, Ferreira J, Massart R, et al. International guidelines for the treatment of Huntington's disease. Front Neurol. 2019;10:710.[Abstract][Full Text]
64. Mestre T, Ferreira J, Coelho MM, et al. Therapeutic interventions for symptomatic treatment in Huntington's disease. Cochrane Database Syst Rev. 2009 Jul 8;(3):CD006456.[Abstract][Full Text]
65. Ferreira JJ, Rodrigues FB, Duarte GS, et al. An MDS evidence-based review on treatments for Huntington's disease. Mov Disord. 2022 Jan;37(1):25-35.[Abstract][Full Text]
66. Bonelli RM. Mirtazapine in suicidal Huntington's disease. Ann Pharmacother. 2003 Mar;37(3):452.[Abstract]
67. Ford MF. Treatment of depression in Huntington's disease with monoamine oxidase inhibitors. Br J Psychiatry. 1986 Nov;149:654-6.[Abstract]
68. Folstein S, Abbott MH, Chase GA, et al. The association of affective disorder with Huntington's disease in a case series and in families. Psychol Med. 1983 Aug;13(3):537-42.[Abstract]
69. Zenodo. Extended evidence-based guidance on psychological interventions for psychological difficulties in individuals with Huntington's disease, Parkinson's disease, motor neurone disease, and multiple sclerosis. Mar 2021 [internet publication].[Full Text]
70. British Psychological Society. Psychological interventions for people with Huntington's disease, Parkinson's disease, motor neurone disease, and multiple sclerosis. Jan 2021 [internet publication].[Full Text]
71. Cusin C, Franco FB, Fernandez-Robles C, et al. Rapid improvement of depression and psychotic symptoms in Huntington's disease: a retrospective chart review of seven patients treated with electroconvulsive therapy. Gen Hosp Psychiatry. 2013 Nov-Dec;35(6):678.e3-5.[Abstract]
72. Mowafi W, Millard J. Electroconvulsive therapy for severe depression, psychosis and chorea in a patient with Huntington's disease: case report and review of the literature. BJPsych Bull. 2021 Apr;45(2):97-104.[Abstract][Full Text]
73. Royuela Rico A, Gil-Verona JA, Macías Fernández JA. A case of obsessive symptoms in Huntington's disease [in Spanish]. Actas Esp Psiquiatr. 2003 Nov-Dec;31(6):367-70.[Abstract]
74. De Marchi N, Daniele F, Ragone MA. Fluoxetine in the treatment of Huntington's disease. Psychopharmacology (Berl). 2001 Jan 1;153(2):264-6.[Abstract]
75. Paleacu D, Anca M, Giladi N. Olanzapine in Huntington's disease. Acta Neurol Scand. 2002 Jun;105(6):441-4.[Abstract]
76. Squitieri F, Cannella M, Piorcellini A, et al. Short-term effects of olanzapine in Huntington disease. Neuropsychiatry Neuropsychol Behav Neurol. 2001 Jan;14(1):69-72.[Abstract]
77. Lipe H, Schultz A, Bird TD. Risk factors for suicide in Huntingtons disease: a retrospective case controlled study. Am J Med Genet. 1993 Dec 15;48(4):231-3.[Abstract]
78. Anderson KE, Marshall FJ. Behavioral symptoms associated with Huntington's disease. Adv Neurol. 2005;96:197-208.[Abstract]
79. Fisher CA, Sewell K, Brown A, et al. Aggression in Huntington's disease: a systematic review of rates of aggression and treatment methods. J Huntingtons Dis. 2014;3(4):319-32.[Abstract][Full Text]
80. Mahant N, McCusker EA, Byth K, et al. Huntington's disease: clinical correlates of disability and progression. Neurology. 2003 Oct 28;61(8):1085-92.[Abstract]
81. Reilmann R. Pharmacological treatment of chorea in Huntington's disease - good clinical practice versus evidence-based guideline. Mov Disord. 2013 Jul;28(8):1030-3.[Abstract][Full Text]
82. Fasano A, Bentivoglio AR. Tetrabenazine. Expert Opin Pharmacother. 2009 Dec;10(17):2883-96.[Abstract]
83. Chen JJ, Ondo WG, Dashtipour K, et al. Tetrabenazine for the treatment of hyperkinetic movement disorders: a review of the literature. Clin Ther. 2012 Jul;34(7):1487-504.[Abstract]
84. Huntington Study Group. Tetrabenazine as antichorea therapy in Huntington disease: a randomized controlled trial. Neurology. 2006 Feb 14;66(3):366-72.[Abstract]
85. Frank S. Tetrabenazine as anti-chorea therapy in Huntington disease: an open-label continuation study. Huntington Study Group/TETRA-HD Investigators. BMC Neurol. 2009 Dec 18;9:62.[Abstract][Full Text]
86. Coppen EM, Roos RA. Current pharmacological approaches to reduce chorea in Huntington's disease. Drugs. 2017 Jan;77(1):29-46.[Abstract][Full Text]
87. Lucetti C, Gambaccini G, Bernardini S, et al. Amantadine in Huntington's disease: open-label video-blinded study. Neurol Sci. 2002 Sep;23 Suppl 2:S83-4.[Abstract]
88. Lucetti C, Del Dotto P, Gambaccini G, et al. IV amantadine improves chorea in Huntington's disease: an acute randomized, controlled study. Neurology. 2003 Jun 24;60(12):1995-7.[Abstract]
89. Verhagen Metman L, Morris MJ, Farmer C, et al. Huntington's disease: a randomized, controlled trial using the NMDA-antagonist amantadine. Neurology. 2002 Sep 10;59(5):694-9.[Abstract]
90. Burgunder JM, Guttman M, Perlman S, et al. An International survey-based algorithm for the pharmacologic treatment of chorea in Huntington's disease. PLoS Curr. 2011 Aug 30;3:RRN1260.[Abstract][Full Text]
91. Reuter I, Hu MT, Andrews TC, et al. Late onset levodopa responsive Huntington's disease with minimal chorea masquerading as Parkinson plus syndrome. J Neurol Neurosurg Psychiatry. 2000 Feb;68(2):238-41.[Abstract][Full Text]
92. Racette BA, Perlmutter JS. Levodopa responsive parkinsonism in an adult with Huntington's disease. J Neurol Neurosurg Psychiatry. 1998 Oct;65(4):577-9.[Abstract][Full Text]
93. Bird MT, Paulson GW. The rigid form of Huntington's chorea. Neurology. 1971 Mar;21(3):271-6.[Abstract]
94. Li Y, Hai S, Zhou Y, et al. Cholinesterase inhibitors for rarer dementias associated with neurological conditions. Cochrane Database Syst Rev. 2015 Mar 3;(3):CD009444.[Abstract][Full Text]
95. Beglinger LJ, Adams WH, Langbehn D, et al. Results of the citalopram to enhance cognition in Huntington disease trial. Mov Disord. 2014 Mar;29(3):401-5.[Abstract]
96. 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]
97. 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]
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. Farag M, Salanio DM, Hearst C, et al. Advance care planning in Huntington's disease. J Huntingtons Dis. 2023;12(1):77-82.[Abstract]
103. 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]
104. 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]
105. 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]
106. 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]
107. Furr Stimming E, Claassen DO, Kayson E, et al. Safety and efficacy of valbenazine for the treatment of chorea associated with Huntington's disease (KINECT-HD): a phase 3, randomised, double-blind, placebo-controlled trial. Lancet Neurol. 2023 Jun;22(6):494-504.[Abstract]
108. 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]
109. Rodrigues FB, Quinn L, Wild EJ. Huntington's disease clinical trials corner: January 2019. J Huntingtons Dis. 2019;8(1):115-25.[Abstract][Full Text]
110. ClinicalTrials.gov. GENERATION HD2. A study to evaluate the safety, biomarkers, and efficacy of tominersen compared with placebo in participants with prodromal and early manifest Huntington's disease. Feb 2025 [internet publication].[Full Text]
111. PTC Therapeutics. Interim PIVOT-HD results demonstrate evidence of favorable CNS biomarker and clinical effects at month 12 in Huntington's disease patients. Jun 2024 [internet publication].[Full Text]
112. ClinicalTrials.gov. Safety and Proof-of-Concept (POC) study with AMT-130 in adults with early manifest Huntington disease. ClinicalTrials.gov identifier NCT04120493. 10 Dec 2021 [internet publication].[Full Text]
113. Verny C, Bachoud-Lévi AC, Durr A, et al. A randomized, double-blind, placebo-controlled trial evaluating cysteamine in Huntington's disease. Mov Disord. 2017 Jun;32(6):932-6.[Abstract]
114. Reilmann R, Squitieri F, Priller J, et al. Safety and tolerability of selisistat for the treatment of Huntington's disease: results from a randomized, double-blind, placebo-controlled phase II trial (S47.004). Neurology. 2014 Apr 8;82(10 Suppl):S47.004.[Full Text]
115. Niccolini F, Haider S, Reis Marques T, et al. Altered PDE10A expression detectable early before symptomatic onset in Huntington's disease. Brain. 2015 Oct;138(Pt 10):3016-29.[Abstract][Full Text]
116. Beaumont V, Zhong S, Lin H, et al. Phosphodiesterase 10A inhibition improves cortico-basal ganglia function in Huntington's disease models. Neuron. 2016 Dec 21;92(6):1220-37.[Abstract][Full Text]
117. Group HS. Brief report: Pfizer Amaryllis trial ends: negative results lead to termination of extension study. 2016 [internet publication].[Full Text]
118. Rodrigues FB, Abreu D, Damásio J, et al. Survival, mortality, causes and places of death in a European Huntington's Disease Prospective Cohort. Mov Disord Clin Pract. 2017 Sep-Oct;4(5):737-42.[Abstract][Full Text]
119. Hamilton JM, Wolfson T, Peavy GM, et al; Huntington Study Group. Rate and correlates of weight change in Huntington's disease. J Neurol Neurosurg Psychiatry. 2004 Feb;75(2):209-12.[Abstract][Full Text]
120. van der Burg JM, Björkqvist M, Brundin P. Beyond the brain: widespread pathology in Huntington's disease. Lancet Neurol. 2009 Aug;8(8):765-74.[Abstract]
121. Furby H, Siadimas A, Rutten-Jacobs L, et al. Natural history and burden of Huntington's disease in the UK: a population-based cohort study. Eur J Neurol. 2022 Aug;29(8):2249-57.[Abstract][Full Text]
122. Djoussé L, Knowlton B, Cupples LA, et al. Weight loss in early stage of Huntington's disease. Neurology. 2002 Nov 12;59(9):1325-30.[Abstract]
123. Hunt VP, Walker FO. Dysphagia in Huntington's disease. J Neurosci Nurs. 1989 Apr;21(2):92-5.[Abstract]
124. Wheelock VL, Tempkin T, Marder K, et al. Predictors of nursing home placement in Huntington disease. Neurology. 2003 Mar 25;60(6):998-1001.[Abstract]
125. Wesson M, Boileau NR, Perlmutter JS, et al. Suicidal ideation assessment in individuals with premanifest and manifest Huntington disease. J Huntingtons Dis. 2018;7(3):239-49.[Abstract][Full Text]
126. Favril L, Yu R, Uyar A, et al. Risk factors for suicide in adults: systematic review and meta-analysis of psychological autopsy studies. Evid Based Ment Health. 2022 Nov;25(4):148-55.[Abstract][Full Text]
127. Favril L, Yu R, Geddes JR, et al. Individual-level risk factors for suicide mortality in the general population: an umbrella review. Lancet Public Health. 2023 Nov;8(11):e868-77.[Abstract][Full Text]
128. Beghi M, Butera E, Cerri CG, et al. Suicidal behaviour in older age: a systematic review of risk factors associated to suicide attempts and completed suicides. Neurosci Biobehav Rev. 2021 Aug;127:193-211.[Abstract]
129. Shenassa ED, Rogers ML, Spalding KL, et al. Safer storage of firearms at home and risk of suicide: a study of protective factors in a nationally representative sample. J Epidemiol Community Health. 2004 Oct;58(10):841-8.[Abstract][Full Text]
130. Gorlyn M. Impulsivity in the prediction of suicidal behavior in adolescent populations. Int J Adolesc Med Health. 2005 Jul-Sep;17(3):205-9.[Abstract]
131. National Institute for Health and Care Excellence. Urinary incontinence in neurological disease: assessment and management. Oct 2023 [internet publication].[Full Text]
132. Bonelli RM, Wenning GK. Pharmacological management of Huntington's disease: an evidence-based review. Curr Pharm Des. 2006;12(21):2701-20.[Abstract]
133. Novak MJ, Tabrizi SJ. Huntington's disease. BMJ. 2010 Jun 30;340:c3109.[Abstract]
134. Shoesmith C, Abrahao A, Benstead T, et al. Canadian best practice recommendations for the management of amyotrophic lateral sclerosis. CMAJ. 2020 Nov 16;192(46):E1453-E1468.[Abstract][Full Text]
