EFNS guidelines on the Clinical Management of Amyotrophic Lateral Sclerosis (MALS) - revised report of an EFNS task force.

Publication

EFNS guidelines on the Clinical Management of Amyotrophic Lateral Sclerosis (MALS) - revised report of an EFNS task force.

Departments

Umea University, Umea, Sweden University of Edinburgh, Edinburgh, UK Centre, Hospitalier Universitaire Vaudois, University of Lausanne, Lausanne, Switzerland , Hospital de Santa Maria, Lisbon, Portugal University of Turin and San Giovanni, Hospital, Turin, Italy University of Leuven and VIB, Leuven, Belgium Trinity, College and Beaumont Hospital, Dublin, Ireland Medizinische Hochschule Hannover, , Germany School of Clinical and Experimental Medicine, University of Birmingham, and Queen Elizabeth Hospital, Birmingham, UK Hopital de la Salpetriere, Paris,, France University of Milan Medical School, Milan, Italy Jagiellonian University, Medical College, Krakow, Poland Munich University Hospital, Munich, Germany, Kantonsspital St Gallen and University Hospital Basel, Basel, Switzerland.

Date

2012-02-01T10:05:22Z

URI

http://hdl.handle.net/10147/207346

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.

Language

ENG

Citation

Eur J Neurol. 2011 Sep 14. doi: 10.1111/j.1468-1331.2011.03501.x.

ISSN

1468-1331 (Electronic)
1351-5101 (Linking)

DOI

10.1111/j.1468-1331.2011.03501.x

PMID

21914052

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