Effectiveness of anti-psychotics and related drugs in the Huntington French-speaking group cohort.
OBJECTIVE
Huntington's disease is a rare condition. Patients are commonly treated with antipsychotics and tetrabenazine. The evidence of their effect on disease progression is limited and no comparative study between these drugs has been conducted. We therefore compared the effectiveness of antipsychotics on disease progression.


METHODS
956 patients from the Huntington French Speaking Group were followed for up to 8 years between 2002 and 2010. The effectiveness of treatments was assessed using Unified Huntington's Disease Rating Scale (UHDRS) scores and then compared using a mixed model adjusted on a multiple propensity score.


RESULTS
63% of patients were treated with antipsychotics during the survey period. The most commonly prescribed medications were dibenzodiazepines (38%), risperidone (13%), tetrabenazine (12%) and benzamides (12%). There was no difference between treatments on the motor and behavioural declines observed, after taking the patient profiles at the start of the drug prescription into account. In contrast, the functional decline was lower in the dibenzodiazepine group than the other antipsychotic groups (Total Functional Capacity: 0.41 ± 0.17 units per year vs. risperidone and 0.54 ± 0.19 vs. tetrabenazine, both p<0.05). Benzamides were less effective than other antipsychotics on cognitive evolution (Stroop interference, Stroop color and Literal fluency: p<0.05).


CONCLUSIONS
Antipsychotics are widely used to treat patients with Huntington's disease. Although differences in motor or behavioural profiles between patients according to the antipsychotics used were small, there were differences in drug effectiveness on the evolution of functional and cognitive scores.
Equipe 01, U955, Inserm, Créteil, France ; Faculté de médecine, Université Paris Est, Créteil, France ; Service de Pharmacologie Clinique, Hôpital H. Mondor - A. Chenevier, AP-HP, Créteil, France ; Département d'Etudes Cognitives, Ecole Normale Supérieure, Paris, France.
Gaëlle
G
Désaméricq
Equipe 01, U955, Inserm, Créteil, France ; Faculté de médecine, Université Paris Est, Créteil, France ; Unité de recherche clinique, Hôpital H. Mondor - A. Chenevier, AP-HP, Créteil, France.
Guillaume
G
Dolbeau
Centre de référence des maladies neurogénétiques, service de neurologie, CHU d'Angers, Angers, France ; UMR CNRS 6214 - INSERM U1083, Angers, France.
Christophe
C
Verny
Centre de référence Maladie de Huntington, Hôpital H. Mondor - A. Chenevier, AP-HP, Créteil, France ; Département de génétique, Hôpital de la salpêtrière, AP-HP, Paris, France.
Perrine
P
Charles
Centre de référence Maladie de Huntington, Hôpital H. Mondor - A. Chenevier, AP-HP, Créteil, France.
Alexandra
A
Durr
Equipe 01, U955, Inserm, Créteil, France ; Faculté de médecine, Université Paris Est, Créteil, France ; Département d'Etudes Cognitives, Ecole Normale Supérieure, Paris, France ; Centre de référence Maladie de Huntington, Hôpital H. Mondor - A. Chenevier, AP-HP, Créteil, France.
Katia
K
Youssov
Departement of Neurology and Movement Disorders, CHRU Lille, Lille, France ; UMR837 INSERM - JPArc Team 6, Lille, France ; University Lille 2/Law & Health, Lille, France.
Clémence
C
Simonin
Service de Neurologie et pathologie du mouvement, Hôpital de la Timone, Marseille, France.
Jean-Philippe
JP
Azulay
Service de Neurologie, CHU Hautepierre, Strasbourg, France ; Université de Strasbourg, Strasbourg, France.
Christine
C
Tranchant
Université Bordeaux Segalen, Laboratoire Maladies Rares: Génétique et Métabolisme (MRGM), EA4576, CHU Bordeaux, Service de Génétique médicale, Bordeaux, France.
Cyril
C
Goizet
Centre d'Investigation Clinique 004, Inserm, Nantes, France.
Philippe
P
Damier
Faculté de Médecine et de Maïeutique Lyon Sud Charles Mérieux, Université Lyon I, Lyon, France ; Service de Neurologie C, Hôpital Neurologique Pierre Wertheimer, Hospices Civils de Lyon, Lyon, France ; Centre de Neurosciences Cognitives, UMR5229, CNRS, Bron, France.
Emmanuel
E
Broussolle
Centre Leenaards de la Mémoire, Département des Neurosciences Cliniques, CHUV, Lausanne, Switzerland.
Jean-François
JF
Demonet
Faculté de médecine, Université Paris Est, Créteil, France ; Centre d'Investigation Clinique 006, Inserm, Créteil, France ; Pôle Recherche clinique Santé Publique, Hôpital H. Mondor - A. Chenevier, AP-HP, Créteil, France.
Graca
G
Morgado
Equipe 01, U955, Inserm, Créteil, France ; Faculté de médecine, Université Paris Est, Créteil, France ; Département d'Etudes Cognitives, Ecole Normale Supérieure, Paris, France ; Centre de référence Maladie de Huntington, Hôpital H. Mondor - A. Chenevier, AP-HP, Créteil, France.
Laurent
L
Cleret de Langavant
Faculté de médecine, Université Paris Est, Créteil, France ; Service de Pharmacologie Clinique, Hôpital H. Mondor - A. Chenevier, AP-HP, Créteil, France.
Isabelle
I
Macquin-Mavier
Equipe 01, U955, Inserm, Créteil, France ; Faculté de médecine, Université Paris Est, Créteil, France ; Département d'Etudes Cognitives, Ecole Normale Supérieure, Paris, France ; Centre de référence Maladie de Huntington, Hôpital H. Mondor - A. Chenevier, AP-HP, Créteil, France.
Anne-Catherine
AC
Bachoud-Lévi
Equipe 01, U955, Inserm, Créteil, France ; Faculté de médecine, Université Paris Est, Créteil, France ; Département d'Etudes Cognitives, Ecole Normale Supérieure, Paris, France ; Centre de référence Maladie de Huntington, Hôpital H. Mondor - A. Chenevier, AP-HP, Créteil, France.
Patrick
P
Maison
D014150:Antipsychotic Agents / Q000627:therapeutic use*; D015331:Cohort Studies; D018450:Disease Progression; D005602:France; D006801:Humans; D006816:Huntington Disease / Q000188:drug therapy* / Q000503:physiopathology
D014150:Antipsychotic Agents