ABSTRACT
Amyotrophic lateral sclerosis (ALS) is a progressive fatal disorder with an incidence of about 2 in 100 000 per year, and a prevalence of about 6 in 100 000.1 It is characterised by increasing weakness of the limb, trunk, ventilatory and bulbar muscles, usually without impairment of the sphincters, or of intellectual faculties. As a result, there is increasing dependency on the patient's family and other carers.2 In the longer term, this will lead to a state of physical dependency and immobility. Despite the substantial impact of ALS/motor neurone disease (MND) upon patients, it is only very recently that work has been undertaken to assess systematically the impact in terms of subjective health status and quality of life. This chapter will outline the use of generic health status measures in the assessment of amyotrophic lateral sclerosis/motor neurone disease,3 and will outline the development and validation of the first measure designed specifically for patients diagnosed with these conditions.
