Amyotrophic Lateral Sclerosis Guidelines

Updated: Aug 18, 2023
  • Author: Carmel Armon, MD, MSc, MHS; Chief Editor: Nicholas Lorenzo, MD, CPE, MHCM, FAAPL  more...
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Guidelines Summary

In October 2009, the American Academy of Neurology (AAN) published a 2-part, evidence-based Practice Parameter update about the care of patients with ALS. [2, 3]  These publications updated the 1999 evidence-based practice parameter. [173]

NIV, PEG, and riluzole

The chief findings of the practice parameter are that for extending life or slowing disease progression, the evidence is best for use of noninvasive ventilation (NIV), percutaneous endoscopic gastrostomy (PEG), and riluzole. The authors comment that these treatments are often underutilized and recommend that they should be offered to patients, in the case of riluzole, or considered by the physician, in the case of NIV and PEG.

The median extension of life by riluzole in placebo-controlled studies was 2-3 months. The median extension of life by NIV or PEG may be approximately 6 months, provided the treatments are applied early and adhered to. The efficacy of NIV is supported by a randomized, controlled trial. [187]

Multidisciplinary care

The authors also recommend that referral to a multidisciplinary clinic should be considered for managing patients with ALS. Such clinics can optimize healthcare delivery, prolong survival, and enhance quality of life.

Botulinum toxin

In patients with sialorrhea that is refractory to treatment with standard medications, botulinum toxin B injection of the salivary glands should be considered. Alternatively, low-dose radiation therapy to the salivary glands may be considered.

Botulinum toxin for ALS carries a manufacturer's warning regarding possible spread of the toxin to ventilatory muscles (leading to exacerbation of ventilatory failure) or bulbar muscles (exacerbating their weakness). The risk of these adverse effects tilts the balance in terms of safety toward salivary gland irradiation, except in patients with exceptional ventilatory and bulbar reserves.

Dextromethorphan and quinidine

Finally, the guideline recommends that dextromethorphan and quinidine be considered for pseudobulbar affect. A combination of dextromethorphan and quinidine (Nuedexta) has shown efficacy in ameliorating involuntary laughter and crying (the expression of pseudobulbar affect). [186, 187]  It has FDA approval for this indication.

Clinician and patient summaries of the AAN guidelines are available on the AAN website: