EAN guidelines on the management of ALS

New guidelines on the management of amyotrophic lateral sclerosis (ALS) were presented at the EAN 2023 and are projected to be published before the end of 2023. The guidelines are a joint effort of the EAN in collaboration with ERN Euro-NMD. Three new drugs soon to be evaluated may necessitate another update of these guidelines shortly.

The new ALS guidelines were presented by Prof. Philip van Damme (University Hospital Gasthuisberg, Belgium) [1]. This version replaces the previous guidelines dating back to 2012 [2]. Prof. Van Damme explained that 26 research questions were identified using the PICO framework. Of these, 8 were recommendations adapted from the British NICE guidelines (NICE guideline NG42: Motor neurone disease) [3]; the other 16 were de novo recommendations.

A cornerstone of ALS management is multidisciplinary care; the evidence is based mostly on observational studies. A team ideally consists of an ALS neurologist, a respiratory specialist, a nursing professional, a mental health professional, a social worker, a speech/language pathologist, a dietician, and a physical therapist.

The main recommendations concerning disease-modifying therapies are the following:

• Riluzole should be offered as a lifelong treatment option to all ALS patients at diagnosis. “A single daily dose of 50 mg can already be effective,” added Prof. Van Damme.
• Cell-based therapies are not recommended outside the context of clinical trials until positive phase 3 trial data is available.
• Edaravone and AMX0035 both receive temporary recommendations; the guideline committee wants to await the phase 3 trial results before issuing a final recommendation.
• Tofersen should be offered as first-line treatment in patients with progressive ALS caused by mutations in the superoxide dismutase 1 (SOD1) gene. The possibility of serious adverse events should be discussed with the patient.

There are ongoing RCTs for nutritional interventions. Despite there currently being no RCTs for gastrostomy, it should be discussed at an early stage with the patient. Furthermore, healthcare professionals should also explain the benefits of early placement and the possible risks of a late gastrostomy.

In conclusion, Prof. Van Damme expressed his hope that an update of these guidelines will be necessary shortly, as 3 additional drugs are likely to be evaluated by the EMA in the coming year.

1. Van Damme P, et al. EAN guideline on the management of amyotrophic lateral sclerosis in collaboration with ERN Euro-NMD. EAN 2023 Annual Meeting, 1-4 July, Budapest, Hungary.
2. Andersen PM, et al. Eur J Neurol. 2012;19(3):360–75.
3. https://www.nice.org.uk/guidance/ng42

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Posted on 7 September, 202322 February, 2024