Evaluation of the application of the European guidelines for diagnosis and clinical care of Amyotrophic Lateral Sclerosis.
Résumé
Objective: To evaluate the degree of application of the recommendations edited by the European Academy of Neurology (EAN) on the management of amyotrophic lateral sclerosis (ALS) in clinical practice. Background: The management of ALS is based on a multidisciplinary approach led by guidelines published by scientific communities. The EAN published guidelines in 2005 and 2012. Methods: Multicenter cross-sectional observational study involving six French ALS-referral centers and including prevalent and incident cases. Good practice points were translated into ad-hoc questions referring to key steps in the management. The application was evaluated by an independent clinical research assistant who examined the medical charts (MCs). When needed, an independent board-certified neurologist was in charge of answering the questions based on the examination of MCs and interview of the caring neurologist. Questions regarding diagnosis and communication were asked to patients through a self-administered questionnaire. Results: We included 376 ALS patients (176 incident, 200 prevalent cases). Median age at diagnosis was 62.8 years (IQR 55.7-72.3) and the sex-ratio was 1.37. A bulbar onset was present in 27.3[percnt] of cases. We evaluated all the topics covered in the EAN guidelines: diagnosis delay (e.g.mean 13.6 months, associated with age and onset), breaking the news (e.g.in more than 90[percnt], satisfaction of criteria for communication quality), multidisciplinary and sustained support (e.g.in 90[percnt], clinic visits scheduled at every 2-3 months), riluzole (e.g.offered and safety monitored in all patients), symptom management, genetic testing, ventilation, troubles of communication, enteral nutrition, and palliative and end-of-care. We also identified characteristics associated with poor compliance to guidelines. Conclusion: This is the first time for ALS clinicians to evaluate the application of the EAN recommendations for the management of ALS. This work will also allow us to propose (i) eventual modifications of impractical recommendations and (ii) sources of improvement for caring neurologists.