Huntington's disease
ICD-10 G10 · ICD-11 8A01.10

Treatment of Huntington's Disease When Chorea Causes Distress or Discomfort

In Huntington's disease, chorea — abnormal, involuntary, spontaneous, uncontrollable movements affecting the trunk, face, and limbs — does not always require pharmacological intervention. Drug treatment becomes the priority when these movements cause the patient clear distress or discomfort.

Clinical scenario

Chorea in Huntington's disease presents as irregular, intermittent, non-rhythmic, and aimless movements that can involve the trunk, face, and limbs. The threshold for initiating treatment is not the severity of the movements alone, but specifically whether those movements are causing the patient distress or discomfort.

Treatment approach (partial overview)

Established first-line pharmacological options are available for this clinical situation. The preferred agent depends on the patient's psychiatric profile — including any associated personality, behavioural, or psychotic features — as well as specific contraindications. Monotherapy is the recommended strategy.

The full selection criteria, alternatives, clinical considerations, and complete regimen are available in the structured protocol below ↓

Instant Access to Structured Evidence-Based Regimens

References

  1. Chorea is characterized by abnormal, involuntary, spontaneous, uncontrollable, irregular, intermittent, non-rhythmic and aimless movements affecting the trunk, the face, and the limbs.
  2. Drug treatment should be considered if chorea causes the patient distress or discomfort.
  3. Tetrabenazine is one of the first-line treatments for this symptom (Grade A) unless the patient suffers from not well-managed depression or suicidal thoughts.
  4. Second generation neuroleptics (Grade B) are first-line treatments for this symptom in particular when the patients have associated personality and/or behavioral or psychotic disorders.
  5. Monotherapy to treat chorea is preferred because combination therapy increases the risk of adverse effects and may complicate the management of non-motor symptoms.
  6. Deutetrabenazine may indeed be proposed as an alternative to tetrabenazine for the treatment of chorea in countries where the marketing authorization is already obtained, like in the USA.
DOI: 10.3389/fneur.2019.00710 View source ↗