Table 2

Symptomatic management in patients with motor neuron disease

Hypersalivation and droolingMostly patients with bulbar onset (approx 30%)Thin secretions: Hyoscine patches, amitriptyline, glycopyrronium, botulinum toxin. Thick secretions: pineapple or papaya juice, nebulised saline, β blockers
Spasticity, cramps and fasciculationOften a transient feature which recedes with disease progression. Not necessary to treat in most.Exercise and physiotherapy, baclofen or tizanidine, gabapentin, botulinum toxin, especially once the patient can no longer walk
Nocturnal sleep fragmentation and daytime somnolenceSymptomatic in about 40-50% of ALS patientsNasal intermittent positive pressure ventilation by mask
Dyspnoea at the end of lifeA minorityOramorph, with consent of patient, acknowledging secondary respiratory compromise effects of drug treatment
EmotionalityApproximately 10% of patients, often a transient featureAmitriptyline, selective serotonin reuptake inhibitors
Nutritional insufficiencyInevitable but affects people at different stagesAbout 60-70% of patients require enteral feeding (PEG or RIG)
Neck weakness, head drop10-20%Specialist collars such as the “Headmaster”
  • ALS, amyotrophic lateral sclerosis; PEG, percutaneous endoscopic gastrostomy; RIG, radiologically inserted gastrostomy.