management

Last reviewed 01/2018

Consult expert advice.

The chorea and dementia have a steady worsening progression. No treatment arrests the advance of the disease.

Symptomatic treatment may include:

• neuroleptic agents:
• psychotic features are seen in about 30% of patients with HD
• many suggest the use of 'modern' or 'atypical' antipsychotics because they are believed to have milder side effects on motor function (1)
• however, phenothiazines have been used to induce a Parkinsonian syndrome to reduce the chorea (2)

• tetrabenazine is used widely to suppress chorea (1,3):
• however functional improvement is often absent
• depression is a common side effect

• antidepressant:
• depression is common in patients with Huntington's disease

Chronic hospital or residential care may eventually be necessary due to the insurmountable stress placed upon relatives and carers. Often, the patient usually dies between 10 and 20 years after the onset of symptoms.

Notes:

• tetrabenazine works mainly as a vesicular monoamine transporter (VMAT) inhibitor, therefore promoting the early metabolic degradation of monoamines, especially the neurotransmitter dopamine

Reference:

1. Naarding H et al.Huntington's disease: a review of the literature on prevalence and treatment of neuropsychiatric phenomena. Eur Psychiatry. 2001 Dec;16(8):439-45
2. Craufurd D. Huntingdon's disease. Medicine International 1994;22(1):29-31.
3. Poon LH, Kang GA, Lee AJ. Role of tetrabenazine for Huntington's disease-associated chorea. Ann Pharmacother. 2010;44:1080-1089.