EFNS guidelines on the Clinical Management of Amyotrophic Lateral Sclerosis (MALS) – revised report of an EFNS task force
Article first published online: 14 SEP 2011
© 2011 The Author(s). European Journal of Neurology © 2011 EFNS
European Journal of Neurology
Volume 19, Issue 3, pages 360–375, March 2012
How to Cite
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.-F., Silani, V., Tomik, B., Wasner, M. and Weber, M. (2012), EFNS guidelines on the Clinical Management of Amyotrophic Lateral Sclerosis (MALS) – revised report of an EFNS task force. European Journal of Neurology, 19: 360–375. doi: 10.1111/j.1468-1331.2011.03501.x
- Issue published online: 14 FEB 2012
- Article first published online: 14 SEP 2011
- Received 16 November 2010 Accepted 12 July 2011
- breaking the diagnosis;
- bronchial secretions;
- cognitive dysfunction;
- Evidence-based medicine;
- genetic counselling;
- palliative care;
- terminal care;
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.