Affiliations: The Michael Trimble Neuropsychiatry Research Group,
Department of Neuropsychiatry, BSMHFT and University of Birmingham, Birmingham,
UK
Note: [] Corresponding author: Connie Pidgeon, Department of
Neuropsychiatry, The Barberry National Centre for Mental Health, Birmingham B15
2FG, UK. Tel.: +44 121 3012317; Fax: +44 121 3012291; E-mail:
[email protected]
Abstract: Introduction: Huntington disease (HD) is a progressive
neurodegenerative condition characterised by motor, cognitive and behavioural
dysfunction, and has an autosomal dominant mode of inheritance. As there is
currently no treatment to delay progression of the disease, pharmacological
intervention is aimed at symptomatic relief. Methods: We set out to assess the current evidence on the
pharmacological treatment of motor and non-motor symptoms in HD by carrying out
a systematic literature review across five large scientific databases. Results: The search generated 23 original studies meeting our search
criteria. Studies on the following drug classes were obtained: dopamine (DA)
depleting agents, neuroleptics, anti-glutamatergic agents, acetylcholinesterase
inhibitors, GABA agonists, cannabinoids, antidepressants and potential
neuroprotective agents. Tetrabenazine (TBZ), a DA depleting agent, was the only
pharmacotherapy shown to have a clinically meaningful, statistically
significant effect on chorea. The majority of the reviewed studies focussed on
the treatment of motor symptoms of HD. Discussion: Overall, the evidence base for the pharmacological
management of HD is poor. There is a clear need for future high quality
randomised controlled trials on the symptomatic treatment of HD, particularly
on the pharmacotherapy of non-motor symptoms of HD.
