Therapeutic interventions for disease progression in Huntington's disease

There is no evidence of the efficacious interventions for modifying disease progression in Huntington's disease.

A Cochrane review included 8 studies with a total of 1366 subjects with confirmed Huntington’s disease. The median duration of studies was 52 weeks. The selected interventions were vitamin E, idebenone, baclofen, lamotrigine, creatine, coenzyme Q10 + remacemide, ethyl-eicosapentanoic acid and riluzole. The most frequently used primary outcome measure was a change in functional capacity using the Total Function Capacity (TFC)-UHDRS. No intervention produced positive results for the selected efficacy outcome measures. The selected interventions were found to be generally safe and well tolerated.

Comment: The quality of evidence is downgraded by study limitations (inadequate allocation concealment), inconsistency (heterogeneity in interventions and outcomes) and indirectness of evidence (differences between the population).

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1. Mestre T, Ferreira J, Coelho MM, Rosa M, Sampaio C. Therapeutic interventions for symptomatic treatment in Huntington's disease. Cochrane Database Syst Rev 2009 Jul 8;(3):CD006456.