ABSTRACT
The motor neurone diseases encompass a number of conditions that affect the motor neurones. These include amyotrophic lateral sclerosis (ALS), progressive bulbar palsy, primary muscular atrophy and primary lateral sclerosis, in addition to spinal muscular atrophy and Kennedy’s disease. The incidence is 1.5–2 per 100 000 per year with a slight male predominance with a male:female ratio of 1.7:1, higher in younger onset cases. Riluzole, a glutamate receptor antagonist, is the only drug licensed in the UK for the treatment of ALS. A number of trials have been undertaken to assess its effectiveness. Bulbar symptoms may be particularly distressing for patients, resulting in a range of difficulties including dysarthria, poor saliva control causing either constant drooling or pooling of secretions at the back of the mouth, dysphagia, laryngospasm and choking episodes. Involvement of palliative care services should be encouraged, and is increasingly being offered in the earlier stages of the disease course, prior to the patient entering the terminal phase.
