title: Evaluation of the application of the European guidelines for the diagnosis and clinical care of amyotrophic lateral sclerosis (ALS) patients in six French ALS centres.
abstract: OBJECTIVE
  Our objective was to evaluate the extent to which the 2005 recommendations of the European Federation of Neurological Sciences (EFNS) on the multidisciplinary management of amyotrophic lateral sclerosis (ALS) are followed in clinical practice.
  
  
  METHODS
  This was a multicentre observational study involving six French ALS referral centres receiving prevalent and incident cases. Recommendations were translated into ad hoc questions referring to key aspects of management, and their application was evaluated by a clinical research assistant who independently examined the medical charts (MCs). When necessary, an independent board-certified neurologist answered the questions based on examination of the MC and interview of the caring neurologist. Questions regarding diagnosis and communication were put to patients in a self-administered questionnaire.
  
  
  RESULTS
  In all, 376 patients [176 incident, 200 prevalent cases; median age at diagnosis 62.8 years (interquartile range 55.7-72.3); sex ratio 1.37; 27.3% bulbar onset] were included. All the topics covered in the recommendations were evaluated: diagnostic delay (e.g. mean 13.6 months, associated with age and onset); breaking the news (e.g. criteria for communication quality were satisfactory in more than 90%); multidisciplinary and sustained support (e.g. clinic visits were scheduled every 2-3 months in 90%). Also considered were whether riluzole had been offered, symptom management, genetic testing, ventilation, communication defects, enteral nutrition, palliative and end-of-life care. Characteristics associated with poor compliance with some guidelines (schedule of visits, delayed riluzole initiation) were also identified.
  
  
  CONCLUSIONS
  This is the first evaluation of the application of the EFNS recommendations for the management of ALS in a nationwide sample. The results allow us to highlight areas for improvement.
authors.affiliation: INSERM, U1094, Tropical Neuroepidemiology, Limoges, France.|Univ. Limoges, UMR_S 1094, Tropical Neuroepidemiology, Institute of Neuroepidemiology and Tropical Neurology, CNRS FR 3503 GEIST, F-87000, Limoges, France.|CHU Limoges, Centre d'Epidémiologie de Biostatistique et de Méthodologie de la Recherche, Limoges, France.
authors.forename: B
authors.initials: B
authors.lastname: Marin
authors.affiliation: Laboratorio di Malattie Neurologiche, Dipartimento di Neuroscienze, IRCCS Istituto di Ricerche Farmacologiche Mario Negri, Milano, Italy.
authors.forename: E
authors.initials: E
authors.lastname: Beghi
authors.affiliation: CHU Lyon Hôpital Neurologique P. Wertheimer, Electromyographie et pathologies neuromusculaires, Lyon, France.
authors.forename: C
authors.initials: C
authors.lastname: Vial
authors.affiliation: CHU Lyon Hôpital Neurologique P. Wertheimer, Electromyographie et pathologies neuromusculaires, Lyon, France.
authors.forename: E
authors.initials: E
authors.lastname: Bernard
authors.affiliation: CHU Limoges, Service de Neurologie, Centre Expert SLA, Limoges, France.
authors.forename: G
authors.initials: G
authors.lastname: Lautrette
authors.affiliation: CHU Clermont-Ferrand, Hopital Gabriel Montpied, Service de Neurologie, Centre Expert SLA, Clermont-Ferrand, France.
authors.forename: P
authors.initials: P
authors.lastname: Clavelou
authors.affiliation: CHU Clermont-Ferrand, Hopital Gabriel Montpied, Service de Neurologie, Centre Expert SLA, Clermont-Ferrand, France.
authors.forename: N
authors.initials: N
authors.lastname: Guy
authors.affiliation: CHU Pellegrin, Service de Neurologie, Centre Expert SLA, Bordeaux, France.
authors.forename: G
authors.initials: G
authors.lastname: Lemasson
authors.affiliation: CHU Pellegrin, Service de Neurologie, Centre Expert SLA, Bordeaux, France.
authors.forename: S
authors.initials: S
authors.lastname: Debruxelles
authors.affiliation: CHU Toulouse Purpan, Centre Expert SLA, Unité de neurophysiologie clinique, Toulouse, France.
authors.forename: P
authors.initials: P
authors.lastname: Cintas
authors.affiliation: CHU Saint-Étienne, Hôpital Nord, Service de Neurologie, Centre Expert SLA, Saint-Étienne, France.
authors.forename: J C
authors.initials: JC
authors.lastname: Antoine
authors.affiliation: CHU Saint-Étienne, Hôpital Nord, Service de Neurologie, Centre Expert SLA, Saint-Étienne, France.
authors.forename: J P
authors.initials: JP
authors.lastname: Camdessanche
authors.affiliation: Department of Basic Medical Sciences, Neuroscience and Sense Organs, University of Bari 'Aldo Moro', Bari, Italy.|Unit of Neurodegenerative Diseases, Department of Clinical Research in Neurology, University of Bari 'Aldo Moro', at Pia Fondazione Cardinale G. Panico, Tricase, Lecce, Italy.
authors.forename: G
authors.initials: G
authors.lastname: Logroscino
authors.affiliation: INSERM, U1094, Tropical Neuroepidemiology, Limoges, France.|Univ. Limoges, UMR_S 1094, Tropical Neuroepidemiology, Institute of Neuroepidemiology and Tropical Neurology, CNRS FR 3503 GEIST, F-87000, Limoges, France.|CHU Limoges, Centre d'Epidémiologie de Biostatistique et de Méthodologie de la Recherche, Limoges, France.
authors.forename: P M
authors.initials: PM
authors.lastname: Preux
authors.affiliation: INSERM, U1094, Tropical Neuroepidemiology, Limoges, France.|Univ. Limoges, UMR_S 1094, Tropical Neuroepidemiology, Institute of Neuroepidemiology and Tropical Neurology, CNRS FR 3503 GEIST, F-87000, Limoges, France.|CHU Limoges, Service de Neurologie, Centre Expert SLA, Limoges, France.
authors.forename: P
authors.initials: P
authors.lastname: Couratier
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis* / Q000628:therapy*; D005260:Female; D005602:France; D019983:Guideline Adherence / Q000592:standards*; D006801:Humans; D008297:Male; D008875:Middle Aged; D017410:Practice Guidelines as Topic
keywords: access and evaluation; amyotrophic lateral sclerosis; clinical audit; healthcare quality; practice guideline