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Exploring the diagnosis delay and ALS functional impairment at diagnosis as relevant criteria for clinical trial enrolment*

Bello Hamidou 1 Benoît Marin 1 Géraldine Lautrette 2, 3 Marie Nicol 2, 1, 3 William Camu 4, 5, 6 Philippe Corcia 7, 8 Marie-Christine Arnes-Bes 9 Christine Tranchant 10 Pierre Clavelou 11, 12 Didier Hannequin 13 Giroud Maurice 14 Katell Beauvais 15 Jean-Christophe Antoine 16, 17 Véronique Danel-Brunaud 18 Fausto Viader 19 Pierre-Marie Preux 1 Philippe Couratier 2, 1, 3
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.
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Submitted on : Tuesday, April 3, 2018 - 4:09:51 PM
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Bello Hamidou, Benoît Marin, Géraldine Lautrette, Marie Nicol, William Camu, et al.. Exploring the diagnosis delay and ALS functional impairment at diagnosis as relevant criteria for clinical trial enrolment*. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, Taylor & Francis, 2017, 18 (7-8), pp.519 - 527. ⟨10.1080/21678421.2017.1353098⟩. ⟨hal-01757319⟩



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