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Huntington Disease

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Motion and Emotion

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Abstract

Huntington disease is a rare neurodegenerative movement disorder characterised by abrupt, irregular, and purposeless ‘dance-like’ movements. Huntington disease is a genetic condition inherited in an autosomal dominant fashion, with expansion of the trinucleotide CAG on the short arm of chromosome 4 identified as single genetic change. At a population level, age at onset of motor manifestations is inversely proportional to CAG repeat length, with higher numbers of repeats correlating with earlier development of symptoms. Higher CAG lengths (for instance, 55 and over) can lead to a juvenile form of Huntington disease (‘Westphal variant’), with more pronounced rigidity, akinesia, and cognitive changes, as well as psychiatric symptoms including depression, anxiety, impulsivity, and aggression. Overall, behavioural symptoms are common in Huntington disease. Specific changes in personality and behaviour are often more disabling than the motor features. Affective symptoms occur in about half of the patients and anxiety in about 20%. Irritability is also highly prevalent and usually occurs in the wider context of psychorigidity and deficits in social cognition. Dementia develops in all patients with Huntington disease later in the illness, whereas alterations in social cognition can occur in earlier stages of the illness.

The name ‘chorea’ is given to the disease on account of the dancing propensities of those who are affected by it, and it is a very appropriate designation

George Huntington, On chorea (1872)

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Suggested Reading

Books

  • Bates G, Tabrizi S, Jones L, editors. Huntington’s disease. 4th ed. Oxford: Oxford University Press; 2014.

    Google Scholar 

  • Naff CF. Huntington’s disease. New York: Greenhaven Press; 2012.

    Google Scholar 

  • Nguyen HHP. In: Cenci MA, editor. Behavioral neurobiology of Huntington’s disease and Parkinson’s disease. New York: Springer; 2015.

    Chapter  Google Scholar 

  • Quarrell O. Huntington’s disease: the facts. 2nd ed. Oxford: Oxford University Press; 2008.

    Google Scholar 

Articles

  • Anderson KE. Huntington’s disease. Handb Clin Neurol. 2011;100:15–24.

    Article  PubMed  Google Scholar 

  • Baig SS, Strong M, Quarrell OW. The global prevalence of Huntington’s disease: a systematic review and discussion. Neurodegener Dis Manag. 2016;6:331–43.

    Article  PubMed  Google Scholar 

  • Bates GP, Dorsey R, Gusella JF, Hayden MR, Kay C, Leavitt BR, Nance M, Ross CA, Scahill RI, Wetzel R, Wild EJ, Tabrizi SJ. Huntington disease. Nat Rev Dis Primers. 2015;1:15005.

    Article  PubMed  Google Scholar 

  • Bora E, Velakoulis D, Walterfang M. Social cognition in Huntington’s disease: a meta-analysis. Behav Brain Res. 2016;297:131–40.

    Article  PubMed  Google Scholar 

  • Carroll JB, Bates GP, Steffan J, Saft C, Tabrizi SJ. Treating the whole body in Huntington’s disease. Lancet Neurol. 2015;14:1135–42.

    Article  PubMed  Google Scholar 

  • Coppen EM, Roos RA. Current pharmacological approaches to reduce chorea in Huntington’s disease. Drugs. 2017;77:29–46.

    Article  CAS  PubMed  Google Scholar 

  • 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 Counsel. 2015;24:29–39.

    Article  CAS  Google Scholar 

  • Dale M, van Duijn E. Anxiety in Huntington’s disease. J Neuropsychiatry Clin Neurosci. 2015;27:262–71.

    Article  PubMed  Google Scholar 

  • Dayalu P, Albin RL. Huntington disease: pathogenesis and treatment. Neurol Clin. 2015;33:101–14.

    Article  PubMed  Google Scholar 

  • Eddy CM, Parkinson EG, Rickards EHG. Changes in mental state and behaviour in Huntington’s disease. Lancet Psychiatry. 2016;3:1079–86.

    Article  PubMed  Google Scholar 

  • Fisher CA, Sewell K, Brown A, Churchyard A. Aggression in Huntington’s disease: a systematic review of rates of aggression and treatment methods. J Huntington’s Dis. 2014;3:319–32.

    Google Scholar 

  • Genetic Modifiers of Huntington’s disease (GEM-HD) Consortium. Identification of genetic factors that modify clinical onset of Huntington’s disease. Cell. 2015;162:516–26.

    Article  CAS  Google Scholar 

  • Groves M, van Duijn E, Anderson K, Craufurd D, Edmondson MC, Goodman N, van Kammen D, Goodman L. An international survey-based algorithm for the pharmacological treatment of irritability in Huntington’s disease. PLoS Curr. 2011;3:RRN1259.

    Article  PubMed  PubMed Central  Google Scholar 

  • Hartmann CJ, Groiss SJ, Vesper J, Schnitzler A, Wojtecki L. Brain stimulation in Huntington’s disease. Neurodegener Dis Manag. 2016;6:223–36.

    Article  PubMed  Google Scholar 

  • Huntington G. On chorea. Med Surg Rep. 1872;26:317–21.

    Google Scholar 

  • Guidelines for the molecular genetics predictive test in Huntington’s disease. International Huntington Association (IHA) and World Federation of Neurology (WFN) Research Group on Huntington’s disease. Neurology. 1994;44:1533–66.

    Google Scholar 

  • Mason SL, Barker RA. Advancing pharmacotherapy for treating Huntington’s disease: a review of the existing literature. Expert Opin Pharmacother. 2016;17:41–52.

    Article  CAS  PubMed  Google Scholar 

  • McColgan P, Tabrizi SJ. Huntington’s disease: a clinical review. Eur J Neurol. 2018;25:24–34.

    Article  CAS  PubMed  Google Scholar 

  • Mestre TA, van Duijn E, Davis AM, Bachoud-Lévi AC, Busse M, Anderson KE, Ferreira JJ, Mahlknecht P, Tumas V, Sampaio C, Goetz CG, Cubo E, Stebbins GT, Martinez-Martin P, Members of the MDS Committee on Rating Scales Development. Rating scales for behavioral symptoms in Huntington’s disease: critique and recommendations. Mov Disord. 2016;31:1466–78.

    Article  PubMed  Google Scholar 

  • Mittal SK, Eddy C. The role of dopamine and glutamate modulation in Huntington disease. Behav Neurol. 2013;26:255–63.

    Article  PubMed  PubMed Central  Google Scholar 

  • Morreale MK. Huntington’s disease: looking beyond the movement disorder. Adv Psychosom Med. 2015;34:135–42.

    Article  PubMed  Google Scholar 

  • Moulton CD, Hopkins CW, Bevan-Jones WR. Systematic review of pharmacological treatments for depressive symptoms in Huntington’s disease. Mov Disord. 2014;29:1556–61.

    Article  CAS  PubMed  Google Scholar 

  • Nance MA. Genetic counseling and testing for Huntington’s disease: a historical review. Am J Med Genet Part B: Neuropsychiatric Genet. 2017;174:75–92.

    Article  Google Scholar 

  • Nopoulos PC. Huntington disease: a single-gene degenerative disorder of the striatum. Dialogues Clin Neurosci. 2016;18:91–8.

    PubMed  PubMed Central  Google Scholar 

  • Novak MJ, Tabrizi SJ. Huntington’s disease. Br Med J. 2010;340:c3109.

    Article  Google Scholar 

  • Paulsen JS. Cognitive impairment in Huntington disease: diagnosis and treatment. Curr Neurol Neurosci Rep. 2011;11:474–83.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Pidgeon C, Rickards H. The pathophysiology and pharmacological treatment of Huntington disease. Behav Neurol. 2013;26:245–53.

    Article  PubMed  PubMed Central  Google Scholar 

  • Pringsheim T, Wiltshire K, Day L, Dykeman J, Steeves T, Jette N. The incidence and prevalence of Huntington’s disease: a systematic review and meta-analysis. Mov Disord. 2012;27:1083–91.

    Article  PubMed  Google Scholar 

  • Quigley J. Juvenile Huntington’s disease: diagnostic and treatment considerations for the psychiatrist. Curr Psychiatry Rep. 2017;19:9.

    Article  PubMed  Google Scholar 

  • Rawlins MD, Wexler NS, Wexler AR, Tabrizi SJ, Douglas I, Evans SJ, Smeeth L. The prevalence of Huntington’s disease. Neuroepidemiology. 2016;46:144–53.

    Article  PubMed  Google Scholar 

  • Rosenblatt A. Neuropsychiatry of Huntington’s disease. Dialogues Clin Neurosci. 2007;9:191–7.

    PubMed  PubMed Central  Google Scholar 

  • Ross CA, Tabrizi SJ. Huntington’s disease: from molecular pathogenesis to clinical treatment. Lancet Neurol. 2011;10:83–98.

    Article  CAS  PubMed  Google Scholar 

  • Rüb U, Vonsattel JP, Heinsen H, Korf HW. The neuropathology of Huntington’s disease: classical findings, recent developments and correlation to functional neuroanatomy. Adv Anat Embryol Cell Biol. 2015;217:1–146.

    Article  PubMed  Google Scholar 

  • Scheuing L, Chiu CT, Liao HM, Linares GR, Chuang DM. Preclinical and clinical investigations of mood stabilizers for Huntington’s disease: what have we learned? Int J Biol Sci. 2014;10:1024–38.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Shannon KM, Fraint A. Therapeutic advances in Huntington’s disease. Mov Disord. 2015;30:1539–46.

    Article  PubMed  Google Scholar 

  • Sharma M, Deogaonkar M. Deep brain stimulation in Huntington’s disease: assessment of potential targets. J Clin Neurosci. 2015;22:812–7.

    Article  PubMed  Google Scholar 

  • Sitek EJ, Thompson JC, Craufurd D, Snowden JS. Unawareness of deficits in Huntington’s disease. J Huntington’s Dis. 2014;3:125–35.

    Google Scholar 

  • Tarolli CG, Chesire AM, Biglan KM. Palliative care in Huntington disease: personal reflections and a review of the literature. Tremor Other Hyperkinet Mov. 2017;7:454.

    Google Scholar 

  • Teixeira AL, de Souza LC, Rocha NP, Furr-Stimming E, Lauterbach EC. Revisiting the neuropsychiatry of Huntington’s disease. Dement Neuropsychol. 2016;10:261–6.

    Article  PubMed  PubMed Central  Google Scholar 

  • The Huntington’s Collaborative Research Group. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington’s disease chromosomes. Cell. 1993;72:971–83.

    Article  Google Scholar 

  • Unti E, Mazzucchi S, Palermo G, Bonuccelli U, Ceravolo R. Antipsychotic drugs in Huntington’s disease. Expert Rev Neurother. 2017;17:227–37.

    Article  CAS  PubMed  Google Scholar 

  • Vale TC, Cardoso F. Chorea: a journey through history. Tremor Other Hyperkinet Mov. 2015;5.

    Google Scholar 

  • Van Duijn E, Craufurd D, Hubers AM, Giltay EJ, Bonelli R, Rickards H, Anderson KE, van Walsem MR, van der Mast RC, Orth M, Landwehrmeyer GB, the European Huntington’s Disease Network Behavioural Phenotype Working group. Neuropsychiatric symptoms in a European Huntington’s disease cohort (REGISTRY). J Neurol Neurosurg Psychiatry. 2014;85:1411–8.

    Article  PubMed  Google Scholar 

  • Wexler NS, Collett L, Wexler AR, Rawlins M, Tabrizi SJ, Douglas I, Smeeth L, Evans SJ. Incidence of adult Huntington’s disease in the UK: a UK-based primary care study and systematic review. Br Med J Open. 2016;6:e009070.

    Google Scholar 

  • Wyant KJ, Ridder AJ, Dayalu P. Huntington’s disease: update on treatments. Curr Neurol Neurosci Rep. 2017;17:33.

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Dept of Neuropsychiatry, University of Birmingham, Birmingham, United Kingdom

    Andrea E. Cavanna

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  1. Andrea E. Cavanna
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Cavanna, A.E. (2018). Huntington Disease. In: Motion and Emotion. Springer, Cham. https://doi.org/10.1007/978-3-319-89330-3_8

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  • DOI: https://doi.org/10.1007/978-3-319-89330-3_8

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-89329-7

  • Online ISBN: 978-3-319-89330-3

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