EFNS guidelines on the Clinical Management of Amyotrophic Lateral Sclerosis (MALS) - revised report of an EFNS task force
Abstract
Background: The evidence base for the diagnosis and management of amyotrophic lateral sclerosis (ALS) is weak. Objectives: To provide evidence‐based or expert recommendations for the diagnosis and management of ALS based on a literature search and the consensus of an expert panel. Methods: All available medical reference systems were searched, and original papers, meta‐analyses, review papers, book chapters and guidelines recommendations were reviewed. The final literature search was performed in February 2011. Recommendations were reached by consensus. Recommendations: Patients with symptoms suggestive of ALS should be assessed as soon as possible by an experienced neurologist. Early diagnosis should be pursued, and investigations, including neurophysiology, performed with a high priority. The patient should be informed of the diagnosis by a consultant with a good knowledge of the patient and the disease. Following diagnosis, the patient and relatives/carers should receive regular support from a multidisciplinary care team. Medication with riluzole should be initiated as early as possible. Control of symptoms such as sialorrhoea, thick mucus, emotional lability, cramps, spasticity and pain should be attempted. Percutaneous endoscopic gastrostomy feeding improves nutrition and quality of life, and gastrostomy tubes should be placed before respiratory insufficiency develops. Non‐invasive positive‐pressure ventilation also improves survival and quality of life. Maintaining the patient’s ability to communicate is essential. During the entire course of the disease, every effort should be made to maintain patient autonomy. Advance directives for palliative end‐of‐life care should be discussed early with the patient and carers, respecting the patient’s social and cultural background.
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Dysphagia is associated with many negative clinical short- and long-term outcomes, such as pneumonia, malnutrition, dehydration, and reduced quality of life.
“Dysphagia is associated with many negative clinical short- and long-term outcomes, such as”
From ESPEN guideline on hospital nutrition (2021) · cites this paper as [188]
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- Authors
- The EFNS Task Force on Diagnosis and Management of Amyotrophic Lateral Sclerosis:, Andersen, P. M., Abrahams, S., Borasio, G. D., de Carvalho, M., Chio, A., Van Damme, P., Hardiman, O., Kollewe, K., Morrison, K. E., Petri, S., Pradat, P., Silani, V., Tomik, B., Wasner, M., & Weber, M.
- Affiliations
- Umeå University (SE)
- Centre Hospitalier Universitaire Vaudois (CH)
- Trinity College Dublin (IE)
- Queen Elizabeth Hospital Birmingham (GB)
- Medizinische Hochschule Hannover (DE)
- Jagiellonian University (PL)
- Kantonsspital St. Gallen (CH)
- Corresponding author
- Andersen, Peter M.
- ORCID
- Venue
- European Journal of Neurology
- Publisher
- Wiley
- Published
- 2011-09-14
- Volume
- 19
- Issue
- 3
- Pages
- 360-375
- ISSN
- 1351-5101 (ISSN-L)
- 1468-1331
- OpenAlex
- W2140248514
- Cited by
- 1,117
- References
- 2
- Citations by year
- 2026: 36 · 2025: 60 · 2024: 88 · 2023: 96 · 2022: 106 · 2021: 111 · 2020: 117 · 2019: 86 · 2018: 71 · 2017: 91 · 2016: 85 · 2015: 59 · 2014: 48 · 2013: 40 · 2012: 21
- FWCI
- 20.95
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- European Journal of Neurology (publishedVersion)
- PubMed (publishedVersion)
- ePrints Soton (University of Southampton) (submittedVersion)
- IRIS (submittedVersion)
- Institutional Research Information System University of Turin (University of Turin) (submittedVersion)
- University of Birmingham Research Portal (University of Birmingham) (submittedVersion)
- Research Portal (Queen's University Belfast) (submittedVersion)
- SERVAL (Université de Lausanne) (submittedVersion)
- Lenus, The Irish Health Repository (Dr Steevens Hospital Library) (submittedVersion)
- Topics
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Health Sciences › Medicine › Genetics › Neurogenetic and Muscular Disorders Research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Amyloidosis: Diagnosis, Treatment, Outcomes
- Keywords
- amyotrophic lateral sclerosis, clinical management, early diagnosis, riluzole, multidisciplinary care, symptom management, percutaneous endoscopic gastrostomy, noninvasive positive pressure ventilation, advance directives, patient autonomy
- MeSH terms
- Amyotrophic Lateral Sclerosis, Humans, Evidence-Based Medicine, Advisory Committees
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