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 patientÕs 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 patientÕs social and cultural background. ObjectivesThis systematic review is an objective appraisal of the evidence regarding the diagnosis and clinical management of patients with amyotrophic lateral sclerosis (ALS). Advances in the knowledge and care of ALS warrant an updating of the 2005 EFNS guidelines [1] with the primary aim of establishing evidence-based and patient-and carer-centred guidelines for diagnosing and managing patients with ALS for clinicians, with the secondary aim of identifying areas where further research is needed.

Accepting or declining non-invasive ventilation or gastrostomy in amyotrophic lateral sclerosis: patients’ perspectives

“…Current interventions are limited to slowing disease progression or palliating symptoms such as dysphagia, weight loss, and respiratory muscle weakness [3,4] and the recommended interventions, such as gastrostomy and noninvasive ventilation (NIV), tend to be required by people with ALS (pwALS) in later stages of the disease [3,5]. Gastrostomy has been shown to improve nutrition, and NIV can help relieve the symptoms experienced due to diaphragmatic weakness, interventions which have improved prognosis and quality of life [6][7][8][9] without increasing caregiver burden [7].…”

A multicentre evaluation of oropharyngeal secretion management practices in amyotrophic lateral sclerosis

“…Side effects were reported in 54% of such patients, a rate similar to that seen overall with anticholinergics. The switching and combination of anticholinergics was common place in this study, but is not well discussed in the literature and deserves further exploration [3,6,19]. It would also be useful to identify the common factors in patients who do not respond well or are intolerant to anticholinergics.…”

“…Despite remaining relatively unaddressed in many reviews and guideline articles [3,6,5,19], the use of conservative measures was often reported as part of the management for both thick and thin secretions. These are largely simple interventions which can be considered at the early stages of a secretion problem.…”

“…In the absence of a cure for ALS, an important aspect of management is to control symptoms in order to maximise quality of life [6,23]. The objective of this study was to explore the current management of saliva related problems by experienced clinicians at ALS centres in the UK.…”

“…These thick secretions may develop or be exacerbated following treatments for excessive thin saliva [6].…”