Treatment

There is no cure for motor neurone disease. Professional counselling and support are important.

Requires specialist advice.

Treatment against the disease process:

• the National Institute of Clinical Excellence (NICE) has recommended that riluzole is used in the treatment of the amyotrophic lateral sclerosis form of MND (1,2)

Symptomatic treatment includes:

• dysarthria - speech assessment and communication aids
• dysphagia - feeding gastrostomy; cricopharyngeal myotomy; pay caution to food consistency - that of porridge is usually swallowed better than solids or liquids
• dysphonia - speech therapists can give expert advice on speech and swallowing difficulties
• saliva problems (4)
  • consider a trial of antimuscarinic medicine as the first-line treatment for sialorrhoea in people with MND
  • consider glycopyrrolate as the first-line treatment for sialorrhoea in people with MND who have cognitive impairment, because it has fewer central nervous system side effects
  • if first-line treatment for sialorrhoea is not effective, not tolerated or contraindicated, consider referral to a specialist service for Botulinum toxin A
  • if a person with MND has thick, tenacious saliva:
    • review all current medicines, especially any treatments for sialorrhoea
    • provide advice on swallowing, diet, posture, positioning, oral care, suctioning and hydration
    • consider treatment with humidification, nebulisers and carbocisteine
• muscle weakness - physiotherapy, walking aids, splints
• muscle cramps
  • consider quinine as first-line treatment for muscle cramps in people with MND. If quinine is not effective, not tolerated or contraindicated, consider baclofen instead as second-line treatment. If baclofen is not effective, not tolerated or contraindicated, consider tizanidine, dantrolene or gabapentin (4)
• muscle stiffness, spasticity or increased tone
  • consider baclofen, tizanidine, dantrolene or gabapentin to treat muscle stiffness, spasticity or increased tone in people with MND. If these treatments are not effective, not tolerated or contraindicated, consider referral to a specialist service for the treatment of severe spasticity (4)
• respiratory function
  • offer non-invasive ventilation as treatment for people with respiratory impairment
  • consider urgent introduction of non-invasive ventilation for people with MND who develop worsening respiratory impairment and are not already using non-invasive ventilation
    • consider opioids as an option to relieve symptoms of breathlessness
    • consider benzodiazepines to manage breathlessness that is exacerbated by anxiety
  • respiratory failure - requires specialist advice
• mood - The Motor Neurone Disease Association gives valuable help to patients and relatives

It is important to consider the patient's quality of life in all decision making.

Reference:

1. NICE (January 2001). Guidance on the Use of Riluzole (Rilutek) for the treatment of Motor Neurone Disease.
2. NICE (April 2006). Review decision on the use of Riluzole for the treatment of motor neurone disease.
3. Orrell, R. & deBelleroche, JS. (1994). Superoxide dismutase and ALS. Lancet, 344, 1651-2.
4. NICE (February 2016).Motor neurone disease