What are the current guidelines for the management of ALS, including pharmacological and non-pharmacological interventions?

Pharmacological interventions for amyotrophic lateral sclerosis (ALS):

• Riluzole is recommended as a disease-modifying treatment option for ALS to potentially slow disease progression (NG42, 1.9.1) ¹.
• For muscle cramps, quinine is considered first-line treatment; if ineffective or contraindicated, baclofen is second-line, followed by tizanidine, dantrolene, or gabapentin as alternatives (off-label use) ¹.
• Muscle stiffness and spasticity may be treated with baclofen, tizanidine, dantrolene, or gabapentin; referral to specialist services is advised if these are ineffective or not tolerated ¹.
• For sialorrhoea (drooling), antimuscarinic medicines are first-line pharmacological treatments; glycopyrronium bromide is preferred in those with cognitive impairment due to fewer CNS side effects ¹.
• Botulinum toxin A (e.g., Xeomin) can be considered as first- or second-line treatment for sialorrhoea, with specialist referral recommended ¹.
• Thick, tenacious saliva may be managed with humidification, nebulisers, and carbocisteine alongside supportive measures ¹.

Non-pharmacological interventions:

• Multidisciplinary team care is essential, involving healthcare and social care professionals with MND expertise, providing coordinated assessments and management every 2–3 months or as needed ¹.
• Exercise programmes tailored to the person’s function and preferences are recommended to maintain joint range of movement, prevent contractures, reduce stiffness, and optimise quality of life; family/carer involvement and safe manual handling advice are important ¹.
• Equipment and adaptations (e.g., orthoses, wheelchairs, assistive technology) should be provided promptly to support mobility and activities of daily living, with regular reassessment as disease progresses ¹.
• Swallowing, diet, posture, positioning, oral care, and suctioning advice are important for managing saliva problems and maintaining nutrition ¹.
• Advance care planning and end-of-life discussions should be offered sensitively at key points, considering communication ability and cognitive status, with support for anticipatory medicines and palliative care as needed ¹.