Background Recent reviews of available drug trials and case reports conclude that the guidelines for the treatment of Huntington's disease (HD) symptoms are scarce.
Aims We wanted to better understand the use of neuroleptics and related drugs (NL-R) in HD by observing their use in clinical practice and their impact on disease progression.
Methods/techniques We explored and compared the profile and evolution of patients treated by NL-R in the cohort of the Huntington French Speaking Group Cohort. This cohort comparative study included 760 patients with available CAG repeats and medication information, followed for 22.4 (±23.3) months between 2002 and 2010.
Results/outcome 63% of patients were treated by a NL-R at least once during the follow up. The main NL-R used by the clinicians were (before and after 2006, respectively): olanzapine (38.8 vs 34.5%, NS), risperidone (15.2 vs 16.0%, NS), tiapride (9.4 vs 8.5%, NS) and tetrabenazine (0.8 vs 18.9%, p<0.001). Profiles differed by chorea (p<0.05), UHDRS Functional Assessment Scale (FAS), Total Functional Capacity (TFC) (p<0.05) and Independence Scale (IS) (p<0.01), with patients taking olanzapine being less disabled on the functional scales. Conversely, tiapride was provided to the most advanced patients. The risperidone group displayed the lowest chorea score (8.4±5.4). During treatment, effectiveness adjusted for baseline profile was statistically different between NL-R. Changes in clinical evolution differed for TFC and FAS (p<0.05) and for body mass index (BMI) (p<0.001). Patients under tiapride displayed the slower functional decline and patients with tetrabenazine were the only one where BMI decreased (3.4±5.7%/year).
Conclusion In our cohort study of 760 HD patients, the four main NL-R used in HD (olanzapine, risperidone, tiapride and tetrabenazine) seem to have different effectiveness on the progression of the disease.
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