Therapeutic interventions for symptomatic treatment in Huntington's disease
Editorial Group: Cochrane Movement Disorders Group
Published Online: 8 JUL 2009
Assessed as up-to-date: 22 DEC 2007
Copyright © 2009 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
How to Cite
Mestre T, Ferreira J, Coelho MM, Rosa M, Sampaio C. Therapeutic interventions for symptomatic treatment in Huntington's disease. Cochrane Database of Systematic Reviews 2009, Issue 3. Art. No.: CD006456. DOI: 10.1002/14651858.CD006456.pub2.
- Publication Status: New
- Published Online: 8 JUL 2009
Huntington's disease (HD) is an orphan autosomal dominant neurodegenerative disorder caused by the amplification of a nucleic acids triplet repeat. It is characterised by core symptoms of chorea, progressive dementia and psychiatric manifestations such as depression, irritability, apathy and psychosis. In current clinical practice, drugs exist that seem to improve symptoms for HD patients. However, their effectiveness has not been fully measured.
To evaluate the effectiveness of the available interventions for the symptomatic treatment of HD.
The search strategy developed for the Movement Disorders Group was undertaken. Cochrane Controlled Trials Register, Medline, EMBASE and Clinical Trials Database of the United States National Institute of Health were thoroughly searched up until December 2007.
All randomised, double-blinded, placebo-controlled clinical trials conducted on any symptomatic therapy used for HD with at least ten participants were included. Participants should have HD clinical features and a confirmatory genetic diagnosis or a compatible family history. All disease variants and ages of disease onset were included. Cross-over studies were included. All pharmacological and non-pharmacological interventions aimed at the control of signs and symptoms associated with HD were to be selected.
Data collection and analysis
Two reviewers independently assessed the identified trials for eligibility. In the selected trials, the assessment of their methodological quality was done according to the Cochrane Collaboration handbook, and eligible data were registered onto standardised forms. If possible, an intention-to-treat analysis was conducted. When data were not available in the original publication, the principal investigator of the trial was contacted. A meta-analysis was conducted when possible and otherwise the descriptive summary of the results was provided. The software Revman 5.0.15 was used for statistical analysis.
22 trials (1254 participants) were included. Nine trials had a cross-over design and 13 were conducted in parallel. Study duration ranged from 2 to 80 weeks. Various pharmacological interventions were studied, mostly, they were anti-dopaminergic drugs (n = 5), glutamate receptor antagonists (n = 5) and energy metabolites (n = 5). Only tetrabenazine showed a clear efficacy for the control of chorea. The remaining pharmacological interventions revealed no clear effectiveness.
No intervention proved to have a consistent symptomatic control in HD. Tetrabenazine is the anti-choreic drug with the best quality data available. Other symptomatic areas should be explored by well-designed randomised placebo-controlled studies.
Plain language summary
Interventions to control symptoms in Huntington's disease
Huntington's disease (HD) is an autosomal dominant neurodegenerative disease. No curative therapy is currently available. We proposed to assess the effectiveness of interventions aimed at controlling the symptoms of HD and to analyse the methodological quality of the corresponding clinical trials. 22 trials were identified. The review of these trials comprising 1254 HD patients revealed that no intervention produced a robust conclusive symptomatic effect. Nevertheless, tetrabenazine was the drug for which better data exists supporting a beneficial effect in the treatment of chorea. There were no available data for the specific treatment of other clinical relevant problems associated with HD such as depression, irritability, apathy, cognitive impairment or psychosis.
我們成立了動作障礙組的搜索策略。Cochrane Controlled Trials Register，Medline，EMBASE，和 Clinical Trials Database of the United States National Institute of Health皆被仔細得搜尋至2007年12月。
兩名評價員獨立評估以確認試驗的資格。在特定的試驗，評估其方法的品質是根據Cochrane協作手冊，並且將適合的資料註冊為標準形式。若可行的話，則進行意向性治療的分析。當資料在原稿中沒有提供，則連絡其試驗的主要研究者。我們賃可能做薈萃分析，不然就以說明性的摘要結果。對於統計分析我們用了software Rev Man 5.0.15。
包括22的試驗(1254參加者)。其中九個試驗有交叉研究和另外13個同時進行。研究時間從2到80週。我們研究了不同藥物的嘗試，包括抗多巴胺藥物(n = 5)，谷氨酸受體拮抗劑(n = 5)，和能量代謝物(n = 5)。只有tetrabenazine對於舞蹈症有明顯的效果。其他藥物並沒有產生明顯的效果。
此翻譯計畫由臺灣國家衛生研究院(National Health Research Institutes, Taiwan)統籌。