Bello Hamidou1,2, Benoit Marin1,2,3, Geraldine Lautrette4, Marie Nicol1,4, William Camu5, Philippe Corcia6, Marie-Christine Arnes-Bes7, Christine Tranchant8, Pierre Clavelou9, Didier Hannequin10, Giroud Maurice11, Katell Beauvais12, Jean-Christophe Antoine13, Véronique Danel-Brunaud14, Fausto Viader15, Pierre-Marie Preux1,2,3, Philippe Couratier1,2,4. 1. a INSERM UMR1094, Neuroépidémiologie Tropicale , Limoges , France. 2. b Université de Limoges, Faculté de Médicine, Institut d'Epidémiologie neurologique et Neurologie Tropicale, CNRS FR 3503 GEIST , Limoges , France. 3. c CHU Limoges, Centre d'Epidémiologie de Biostatistique et de Méthodologie de la Recherche , Limoges , France. 4. d CHU Limoges, Service de Neurologie, Centre SLA , Limoges , France. 5. e Centre SLA de Montpellier - Service de Neurologie, CHRU de Montpellier - Hôpital Gui de Chauliac , Montpellier , France. 6. f Centre SLA de Tours - Service de Neurologie, CHRU de Tours - Hôpital Bretonneau , Tours , France. 7. g Centre SLA de Toulouse - Service de neurologie et d'explorations fonctionnelles, Pôle Neurosciences, Hall B - 3e étage, CHU de Toulouse - Hôpital Pierre-Paul Riquet , Toulouse , France. 8. h Centre SLA de Strasbourg - Hôpital de jour - Neurologie Pôle tête-cou/CETD, CHU de Strasbourg - Hôpital de Hautepierre , Strasbourg , France. 9. i Centre SLA de Clermont-FD - Service de neurologie, CHU de Clermont-Ferrand - Hôpital Gabriel Montpied , Clermont-Ferrand , France. 10. j Centre SLA de Rouen - Centre national de référence pour les malades Alzheimer jeunes - Centre Mémoire de Ressource et Recherches, Département de neurologie - Unité de neuropsychologie, CHU de Rouen - Hôpital Charles Nicolle , Rouen , France. 11. k Centre SLA de Dijon - Neurologie Générale, Vasculaire et Dégénérative, CHU de Dijon Hôpital le BOCAGE , Limoges , France. 12. l Centre SLA de Dijon - Service de Neurophysiologie clinique, CHU Dijon Bourgogne - Hôpital François Mitterrand , Limoges , France. 13. m Centre SLA de Saint-Etienne - Service de Neurologie CHU de Saint-Etienne - Hôpital Nord , Saint-Etienne , France. 14. n Centre SLA de Lille - Service de neurologie A, Pôle Neurosciences et Appareil Locomoteur, CHRU de Lille - Hôpital Roger Salengro , Lille , France. 15. o Centre SLA de Caen - Service de neurologie, CHU de Caen - Hôpital de la Côte , Caen , France.
Abstract
Objectives were: i) to describe the phenotypic heterogeneity of incident amyotrophic lateral sclerosis (ALS) patients diagnosed in 2012 in French ALS centres; ii) to look at the associations between ALSFRS-R score and ALSFRS-R slope (ΔFS) at time of diagnosis with diagnosis delay, ALS phenotypes and Airlie House diagnosis criteria (AHDC); iii) to describe the rate of progression on ΔFS, according to diagnosis delay. METHODS: Incident ALS cases diagnosed in French ALS centres were included. The rate of progression was evaluated as follows: ΔFS = (48 - ALSFRS-R at time of diagnosis)/duration from onset to diagnosis (months). Fast and slow progressors were defined by ΔFS >1 and <0.5, respectively. RESULTS: At time of diagnosis, 476 patients were classified into eight phenotypes: bulbar (33.0%), spinal lumbar (28.2%), spinal cervical (23.1%), flail leg (4.4%), ALS/FTD (4.2%), possible flail arm (4.0%), respiratory (2.1%), dropped-head (1.0%). Median ΔFS (n = 358/476) was 1.0 [0.5-2.0]. ΔFS was associated with AHDC (p = 0.009), but not with clinical phenotype (p = 0.902). Stratification on diagnosis delay (<12 months or ≥18 months) allowed to differentiate fast progressors from slow progressors. CONCLUSION: At time of inclusion in therapeutic trial closed to diagnosis, ΔFS or diagnosis delay may discriminate the rate of progression.
Objectives were: i) to describe the phenotypic heterogeneity of incident amyotrophic lateral sclerosis (ALS) patients diagnosed in 2012 in French ALS centres; ii) to look at the associations between ALSFRS-R score and ALSFRS-R slope (ΔFS) at time of diagnosis with diagnosis delay, ALS phenotypes and Airlie House diagnosis criteria (AHDC); iii) to describe the rate of progression on ΔFS, according to diagnosis delay. METHODS: Incident ALS cases diagnosed in French ALS centres were included. The rate of progression was evaluated as follows: ΔFS = (48 - ALSFRS-R at time of diagnosis)/duration from onset to diagnosis (months). Fast and slow progressors were defined by ΔFS >1 and <0.5, respectively. RESULTS: At time of diagnosis, 476 patients were classified into eight phenotypes: bulbar (33.0%), spinal lumbar (28.2%), spinal cervical (23.1%), flail leg (4.4%), ALS/FTD (4.2%), possible flail arm (4.0%), respiratory (2.1%), dropped-head (1.0%). Median ΔFS (n = 358/476) was 1.0 [0.5-2.0]. ΔFS was associated with AHDC (p = 0.009), but not with clinical phenotype (p = 0.902). Stratification on diagnosis delay (<12 months or ≥18 months) allowed to differentiate fast progressors from slow progressors. CONCLUSION: At time of inclusion in therapeutic trial closed to diagnosis, ΔFS or diagnosis delay may discriminate the rate of progression.