The two common names for this condition – amyotrophic lateral sclerosis (ALS) in the USA, motor neurone disease (MND) in the UK – are an established difficulty. For this discussion ALS will be preferred. The management of the symptoms of ALS, and particularly of the terminal stages, has received relatively little attention in many neurology texts. It was, however, the first major non-cancer diagnosis to receive the attention of palliative care units. An early text published by palliative care pioneer Dame Cicely Saunders devoted a major chapter to the care of ALS. Saunders recognized that the relatively predictable and inexorable course of ALS, and the steadily declining function of the patients made a palliative approach to symptom management highly appropriate. It also needed the same emotional support of patient and carers, and the same concern for the spiritual dimensions of suffering that she had recognized among cancer patients.

THE PALLIATIVE APPROACH

The availability of the glutamate antagonist riluzole promises no more than a small delay in the progress of ALS, but it will usually be gladly accepted and may seem useful for several months:

• riluzole 50 mg tab. 50 mg bd taken away from meal times.

Beyond that medication there is no reliable way to modify the disease, and there is a temptation for clinicians to adopt a therapeutic nihilism.