• quality of life
• motor neurone disease
• amyotrophic lateral sclerosis

The optimal approach to the evaluation of quality of life (QoL) in people with motor neurone disease (MND)/amyotrophic lateral sclerosis (ALS) is still unclear. The paper by Jenkinson and others (JNNP February issue, 242–245) is an important contribution to this debate.¹

The ideal QoL measure in MND needs to fulfil a range of requirements. It must not be an unacceptable burden on sufferers and carers. MND patients tire easily; if the tool is too long, fatigue will ensue and the responses will become increasingly unreliable. The nature of the questions may also colour the responses obtained. The Sickness Impact Profile (SIP) can come over very negatively to subjects. The process of completing this instrument can exacerbate feelings of despair, already often a major issue in MND patients.

Should it be generic or disease specific? Although generic scales such as the SIP and SF-36 have the advantage of enabling comparisons with other disorders, they do not reflect on specific issues affecting perception of QoL in MND.

The use of instruments such as the Schedule for the Evaluation of Individual QoL,² which evaluate patient selected QoL relevant issues serially during the course of the illness, do, however, allow for inclusion of items of specific concern for individual patients. MND patients often yield low scores on generic scales. Such “floor” effects can make evaluation of trends of QoL in advanced disease difficult. Also, it may not be possible to measure QoL in advanced disease, because of the effort required to complete the instrument.

The Amyotrophic Lateral Sclerosis Assessment Questionnaire (ALSAQ-40) has been a very significant step towards a disease specific QoL instrument for MND and asks the subject to give one of five possible answers to a series of 40 questions.³ These responses give an overall score with subscores in five domains (physical mobility, activities of daily living, eating and drinking, communication, and emotional functioning). Completion takes about 15 min in our department. One criticism of the ALSAQ-40 is that it concludes with an assessment of emotional functioning, which can be upsetting to patients, focusing as it does on burden, embarrassment, and hopelessness. The ALSAQ-5, which forms the basis of this paper by Jenkinson et al, attempts to achieve a comparable evaluation to the ALSAQ-40 by using a subset of only five questions from the ALSAQ-40. If the ALSAQ-5 was shown to have comparable validity to the ALSAQ-40 this would be a major advance. We would have a disease specific instrument capable of rapid and “painless” administration that could also be used in patients with advanced MND.

Although QoL issues are important for regulatory and other bodies, including the National Institute for Clinical Excellence, QoL data have been conspicuously absent from many major studies in MND. No prospective QoL data were collected in the trials that lead to the licensing of riluzole.^4–5 If validated in long term use, the ALSAQ-5 will be an important advance and facilitate the wider application of QoL measures in MND related studies to the benefit of health care planners, patients, and carers.