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Experimental therapeutics: clinical
L03 Use and impact of neuroleptics in Huntington's disease: a prospective cohort study of the Huntington French speaking group
  1. G Dolbeau1,2,3,
  2. A Lombard3,
  3. K Youssov1,2,
  4. A Dürr2,4,
  5. P Charles2,4,
  6. C Verny5,
  7. J-P Azulay6,
  8. P Krystkowiak7,
  9. C Simonin8,
  10. C Tranchant9,
  11. C Goizet10,
  12. P Damier11,
  13. F Supiot12,
  14. E Broussolle13,
  15. J-F Démonet14,
  16. R-M Marie15,
  17. M Verin16,
  18. A-C Bachoud-Lévi1,2,*,
  19. P Maison1,2,3,*
  1. 1Inserm U955 E01, Creteil, France
  2. 2Centre de reference Maladie Rare, Maladie de Huntington, Creteil, France
  3. 3Pharmacologie Clinique Unite de recherche clinique CHU Henri Mondor, Creteil, France
  4. 4Hopital de la Pitie Salpêtrière Paris, Paris, France
  5. 5CHU d'Angers, Angers, France
  6. 6CHU La Timone, Marseille, France
  7. 7CHU Amiens, Amiens, France
  8. 8CHU Roger Salengro, Lille, France
  9. 9CHRU Schiltigheim, Strasbourg, France
  10. 10Groupe hospitalier Pellegrin, Bordeaux, France
  11. 11Hopital Laennec Bd J Monod, Nantes, France
  12. 12Hopital Erasme ULB, Bruxelles, Belgique
  13. 13Hopital Pierre Wertheimer, Lyon, France
  14. 14CHU Purpan, Place du Dr Baylac, Toulouse, France
  15. 15CHU Caen avenue de la cote de nacre, Caen, France
  16. 16CHU Pontchaillou, Rennes, France

Abstract

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.

  • Huntington
  • neuroleptic
  • effectiveness
  • cohort

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Footnotes

  • * These authors equally contributed to this project.

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