ABSTRACT
The precise cause(s) of the selective neurodegenerative process in motor neurone disease (MND) remain unknown. For individuals afflicted with the disease, there is progressive unremitting paralysis, leading to death usually within 3-5 years. Several aspects relating to the clinical course of MND can create difficulties both for families coming to terms with the disease and for their medical attendants. First, the progression often occurs over a rapid time course, so that the patient can no sooner have adapted to one level of disability when a new set of problems has to be faced. Second, because of the progression of disability, the optimum period for the provision of aids and appliances can be relatively short, and this aspect of patient care needs to be finely judged and organised quickly. Third, the majority of patients afflicted by MND retain normal cognitive function and full conscious appreciation of their worsening disability, and ultimately of their impending death. Feeling powerless to influence this distressing and tragic disease, the doctor might hesitate to discuss the diagnosis with the patient and to offer continuing medical care and support. However, even though there is no curative treatment for MND, and as yet no treatment that has a major effect on clinical and pathological progression, there is much that can be done to alleviate distress and maximise independence and quality of life for patients and their carers during the course of the disease. This provides a significant challenge to the physician, who must combine diagnostic skills, compassion, and the ability to manage a rehabilitative programme with skill in providing information and counselling to families appropriate for the stage of the disease. This chapter outlines the practical measures to be taken by doctors and other health-care professionals to help patients to achieve the highest possible quality of life during the course of MND.
