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Psych TopicsConsultation-liaison psychiatry

Psych · Consultation-liaison psychiatry

Huntington disease psychiatry

Also known as Huntington's disease psychiatry · HD neuropsychiatry · Huntington disease depression · Huntington disease psychosis · Huntington irritability · Huntington suicide risk · Predictive testing Huntington psychiatry · VMAT2 Huntington psychiatry

Exam-exhaustive fellowship topic on the psychiatry of Huntington disease: premanifest and manifest neuropsychiatric syndromes, suicide risk, irritability and apathy, psychosis, expert-consensus psychopharmacology, VMAT2–mood interface, predictive testing ethics, and multidisciplinary CL–neurology care. FRANZCP-primary, globally tagged.

medium16 referencesUpdated 26 July 202612 min readVerification in progress

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FRANZCPMRCPsychABPNMD-DNBNEET-SS

Red flags

  • Suicide risk is elevated across premanifest and early–mid HD — assess at every contact, especially after genetic disclosure
  • New or worsening depression after starting a VMAT2 inhibitor (tetrabenazine, deutetrabenazine, valbenazine) — treat as drug-related dysphoria until proven otherwise
  • Covert predictive testing or casual testing of asymptomatic minors for adult-onset HD is unethical outside specialist frameworks
  • Severe irritability/aggression toward carers is a core HD neuropsychiatric target — not 'personality' alone
  • Apathy without dysphoria is not the same as major depression — different management emphasis
  • Acute behavioural change with fever, aspiration risk, or metabolic upset — delirium until proven otherwise
  • High-dose typical antipsychotics can cause severe rigidity, falls, and medical complications without stepwise review
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Saved on this device.

Practise this topic10 MCQs with explanations

Target exams

FRANZCPMRCPsychABPNMD-DNBNEET-SS

Red flags

  • Suicide risk is elevated across premanifest and early–mid HD — assess at every contact, especially after genetic disclosure
  • New or worsening depression after starting a VMAT2 inhibitor (tetrabenazine, deutetrabenazine, valbenazine) — treat as drug-related dysphoria until proven otherwise
  • Covert predictive testing or casual testing of asymptomatic minors for adult-onset HD is unethical outside specialist frameworks
  • Severe irritability/aggression toward carers is a core HD neuropsychiatric target — not 'personality' alone
  • Apathy without dysphoria is not the same as major depression — different management emphasis
  • Acute behavioural change with fever, aspiration risk, or metabolic upset — delirium until proven otherwise
  • High-dose typical antipsychotics can cause severe rigidity, falls, and medical complications without stepwise review
The one-line answer

Huntington disease psychiatry is core CL work, and it starts before the chorea does: expect depression, irritability, apathy, anxiety, psychosis and perseveration across premanifest and manifest stages; treat suicide risk as intrinsic to the disease, not incidental; use expert-consensus stepped psychopharmacology; and never start a VMAT2 chorea agent without a mood monitoring plan. Always co-manage with an HD or neurology service and genetics counselling.[1][5][10][12]

Meet the patient

A 41-year-old teacher is brought in by her husband. She has not been diagnosed with anything. Her father died in a nursing home at 58 "with a movement disorder nobody explained". Over two years she has become short-tempered, has been stood down after an outburst at work, sleeps poorly, and last week said the family would be better off without her. Her fingers move slightly when she rests her hands.[1][5][8]

Every registrar reaches for the depression scale. The examiner is waiting to see whether you take the pedigree, ask the suicide question properly, and notice that the psychiatry arrived before the chorea.[6][8][9]

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References16ShowHide
  1. [1]van Duijn E, Craufurd D, Hubers AA, et al. Neuropsychiatric symptoms in a European Huntington's disease cohort (REGISTRY) J Neurol Neurosurg Psychiatry, 2014.PMID 24828898
  2. [2]Craufurd D, Thompson JC, Snowden JS Behavioral changes in Huntington Disease Neuropsychiatry Neuropsychol Behav Neurol, 2001.PMID 11725215
  3. [3]Rosenblatt A Neuropsychiatry of Huntington's disease Dialogues Clin Neurosci, 2007.PMID 17726917
  4. [4]Paoli RA, Botturi A, Ciammola A, et al. Neuropsychiatric Burden in Huntington's Disease Brain Sci, 2017.PMID 28621715
  5. [5]Hubers AA, van Duijn E, Roos RA, et al. Suicidal ideation in a European Huntington's disease population J Affect Disord, 2013.PMID 23876196
  6. [6]van Duijn E, Fernandes AR, Abreu D, et al. Incidence of completed suicide and suicide attempts in a global prospective study of Huntington's disease BMJ Ment Health, 2021.PMID 34462049
  7. [7]Wetzel HH, Gehl CR, Dellefave-Castillo L, et al. Suicidal ideation in Huntington disease: the role of comorbidity J Neuropsychiatry Clin Neurosci, 2011.PMID 21605914
  8. [8]Epping EA, Mills JA, Beglinger LJ, et al. Characterization of depression in prodromal Huntington disease in the neurobiological predictors of HD (PREDICT-HD) study J Neuropsychiatry Clin Neurosci, 2013.PMID 23790259
  9. [9]Epping EA, Kim JI, Craufurd D, et al. Longitudinal Psychiatric Symptoms in Prodromal Huntington's Disease: A Decade of Data Am J Psychiatry, 2016.PMID 26472629
  10. [10]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.PMID 30040737
  11. [11]Groves M, van Duijn E, Anderson K, et al. An International Survey-based Algorithm for the Pharmacologic Treatment of Irritability in Huntington's Disease PLoS Curr, 2011.PMID 21975525
  12. [12]Huntington Study Group Tetrabenazine as antichorea therapy in Huntington disease: a randomized controlled trial Neurology, 2006.PMID 16476934
  13. [13]Frank S, Testa CM, Stamler D, et al. Effect of Deutetrabenazine on Chorea Among Patients With Huntington Disease: A Randomized Clinical Trial JAMA, 2016.PMID 27380342
  14. [14]Walker FO Huntington's disease Lancet, 2007.PMID 17240289
  15. [15]McColgan P, Tabrizi SJ Huntington's disease: a clinical review Eur J Neurol, 2018.PMID 28817209
  16. [16]van Duijn E Medical treatment of behavioral manifestations of Huntington disease Handb Clin Neurol, 2017.PMID 28947111

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