Vol 54, No 4 (2020)
Review Article
Published online: 2020-04-03

open access

Page views 2240
Article views/downloads 996
Get Citation

Connect on Social Media

Connect on Social Media

Sexual dysfunction in Huntington’s Disease — a systematic review

Klaudia Szymuś1, Artur Bystrzyński1, Magdalena Kwaśniak-Butowska234, Agnieszka Konkel234, Agata Leśnicka5, Magdalena Nowacka6, Jarosław Sławek234
Pubmed: 32242915
Neurol Neurochir Pol 2020;54(4):305-311.


Introduction. Huntington’s Disease (HD) is a neurodegenerative disorder of which the main symptoms are motor, cognitive and behavioural problems sometimes including sexual dysfunction. Aim. To review the current knowledge on sexual dysfunction in HD. Methods. Databases of Pubmed and Scopus were searched. Only original studies performed after 1994 were included (from 1994 a genetic test = proven diagnosis). Results. 162 publications were found, but only nine met our established criteria. The majority of patients with HD suffer from sexual disorders. The most common are: hypoactive sexual disorder (53–83% of patients), hyperactive sexual disorder (6–30%), erectile (48–74%) and ejaculatory dysfunctions (30–65%), lubrication problems (53–83%), and orgasmic dysfunction (35–78%). Discussion. Results may be biased for several reasons e.g.: social taboos regarding sex lives, medications that affect sexual function, impaired self–awareness of patients, small study samples, a lack of standardised questionnaires, and a focus only on the presence of sexual problems without describing them. Conclusions. Sexual disorders in HD are common. This is a problem that is probably underestimated, both by patients/caregivers and physicians, who should focus more on these symptoms in order to improve patient quality of life.

Article available in PDF format

View PDF Download PDF file


  1. MACDONALD M. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell. 1993; 72(6): 971–983.
  2. Telenius H, Kremer B, Goldberg YP, et al. Somatic and gonadal mosaicism of the Huntington disease gene CAG repeat in brain and sperm. Nat Genet. 1994; 6(4): 409–414.
  3. Ross CA, Tabrizi SJ. Huntington's disease: from molecular pathogenesis to clinical treatment. Lancet Neurol. 2011; 10(1): 83–98.
  4. Huntington G. On Chorea. The Medical and Surgical Reporter: A Weekly Journal. 1872; 26(15): 317–21.
  5. Dunn KM, Croft PR, Hackett GI. Sexual problems: a study of the prevalence and need for health care in the general population. Fam Pract. 1998; 15(6): 519–524.
  6. American Psychiatric Association Diagnostic and Statistical Manual of Mental Disorders (DSM-IV). SpringerReference. .
  7. Fedoroff JP, Peyser C, Franz ML, et al. Sexual disorders in Huntington's disease. J Neuropsychiatry Clin Neurosci. 1994; 6(2): 147–153.
  8. Fava M, Rankin M. Sexual functioning and SSRIs. J Clin Psychiatry. 2002; 63 Suppl 5: 13–6; discussion 23.
  9. Clayton A, Ramamurthy S. The impact of physical illness on sexual dysfunction. Adv Psychosom Med. 2008; 29: 70–88.
  10. Craufurd D, Thompson JC, Snowden JS. Behavioral changes in Huntington Disease. Neuropsychiatry Neuropsychol Behav Neurol. 2001; 14(4): 219–226.
  11. Kolenc M, Kobal J, Podnar S. Male sexual function in presymptomatic gene carriers and patients with Huntington's disease. J Neurol Sci. 2015; 359(1-2): 312–317.
  12. Kolenc M, Kobal J, Podnar S. Female Sexual Dysfunction in Presymptomatic Mutation Carriers and Patients with Huntington’s Disease. Journal of Huntington's Disease. 2017; 6(2): 105–113.
  13. Aldaz T, Nigro P, Sánchez-Gómez A, et al. Non-motor symptoms in Huntington's disease: a comparative study with Parkinson's disease. J Neurol. 2019; 266(6): 1340–1350.
  14. Aziz NA, Anguelova GV, Marinus J, et al. Autonomic symptoms in patients and pre-manifest mutation carriers of Huntington's disease. Eur J Neurol. 2010; 17(8): 1068–1074.
  15. World Health Organization. ICD-10 Version:2016 [Internet]. 2016 [cited 2019 Dec 19]. Available from:. https://icd.who.int/browse10/2016/en.
  16. Kirkwood SC, Su JL, Conneally P, et al. Progression of symptoms in the early and middle stages of Huntington disease. Arch Neurol. 2001; 58(2): 273–278.
  17. Reininghaus E, Reininghaus B, Fitz W, et al. Sexual behavior, body image, and partnership in chronic illness: a comparison of Huntington's disease and multiple sclerosis. J Nerv Ment Dis. 2012; 200(8): 716–720.
  18. Simpson JA, Lovecky D, Kogan J, et al. Survey of the Huntington's Disease Patient and Caregiver Community Reveals Most Impactful Symptoms and Treatment Needs. J Huntingtons Dis. 2016; 5(4): 395–403.
  19. Dominguez JM, Hull EM. Dopamine, the medial preoptic area, and male sexual behavior. Physiol Behav. 2005; 86(3): 356–368.
  20. Argiolas A, Melis MR. Central control of penile erection: role of the paraventricular nucleus of the hypothalamus. Prog Neurobiol. 2005; 76(1): 1–21.
  21. Markianos M, Panas M, Kalfakis N, et al. Plasma testosterone in male patients with Huntington's disease: relations to severity of illness and dementia. Ann Neurol. 2005; 57(4): 520–525.
  22. Ho AK, Robbins AOG, Barker RA. Huntington's disease patients have selective problems with insight. Mov Disord. 2006; 21(3): 385–389.
  23. Hoth KF, Paulsen JS, Moser DJ, et al. Patients with Huntington's disease have impaired awareness of cognitive, emotional, and functional abilities. J Clin Exp Neuropsychol. 2007; 29(4): 365–376.
  24. Sitek EJ, Thompson JC, Craufurd D, et al. Unawareness of deficits in Huntington's disease. J Huntingtons Dis. 2014; 3(2): 125–135.
  25. Sitek EJ, Sołtan W, Wieczorek D, et al. Self-awareness of motor dysfunction in patients with Huntington's disease in comparison to Parkinson's disease and cervical dystonia. J Int Neuropsychol Soc. 2011; 17(5): 788–795.
  26. Mitchell KR, Jones KG, Wellings K, et al. Estimating the Prevalence of Sexual Function Problems: The Impact of Morbidity Criteria. J Sex Res. 2016; 53(8): 955–967.
  27. Schmidt EZ, Bonelli RM. Sexuality in Huntington's disease. Wien Med Wochenschr. 2008; 158(3-4): 78–83.
  28. Bachoud-Lévi AC, Ferreira J, Massart R, et al. International Guidelines for the Treatment of Huntington's Disease. Front Neurol. 2019; 10: 710.

Neurologia i Neurochirurgia Polska