Journal of Neurology

, Volume 246, Supplement 3, pp III16–III21 | Cite as

Development and validation of a short measure of health status for individuals with amyotrophic lateral sclerosis/ motor neurone disease: the ALSAQ-40

  • Crispin JenkinsonEmail author
  • Cathy Brennan
  • Ray Fitzpatrick
  • Mark Bromberg
  • Michael Swash


In recent years there has been an increased appreciation of the importance of measuring health status from the patient’s point of view, but until now no attempt has been made to develop an amyotrophic lateral sclerosis (ALS)-specific health status measure. The development of such an instrument is especially relevant now with the introduction of drugs that prolong life in ALS but limited data is available on the impact such treatments have on quality of life. This paper reports on the development of an ALS-specific measure, the forty item ALS assessment questionnaire (ALSAQ-40). The development of the ALSAQ-40 followed three main stages. Stage 1 consisted of in-depth, semi-structured exploratory interviews conducted on a sample of 18 patients to identify areas of salience and concern to patients with ALS. These interviews generated 78 candidate questions. In stage 2, the 78-item questionnaire was used in a postal survey to identify appropriate rephrasing/shortening and to determine the acceptability of the measure. In addition, this exercise helped identify sub-scales of the instrument addressing different dimensions of ALS. Finally in stage 3 the data collected in stage 2 was analysed to areas measured by the instrument and to remove redundant question. The resulting measure contains forty questions measuring five areas of health status: Eating and Drinking, Communication, ADL/ independence, Physical mobility, Emotional Functioning. The measure has high face, internal and construct validity and is likely to prove a useful measure in the evaluation of treatment regimes for ALS/MND.

Key words

Amyotrophic lateral sclerosis Quality of life Disease-specific measure ALSAQ-40 


  1. 1.
    Bensimon G, Lacomblez L, Meninger V and the ALS/Riluzole Study Group (1994) A controlled trial of riluzole in amyotrophic lateral sclerosis. N Engl J Med 330:585–951PubMedCrossRefGoogle Scholar
  2. 2.
    Bernat JL, Vincent FM (1987) Neurology. Oradel, NJ: Medical Economics BooksGoogle Scholar
  3. 3.
    Cronbach LJ (1951) Coefficient alpha and the internal structure of tests. Psychometrika 16:297–334CrossRefGoogle Scholar
  4. 4.
    Damiano A, Patrick DL, Guzman G, et al (1999) Measurement of health related quality of life in patients with Amyotrophic Lateral Sclerosis in clinical trials of new therapies. Med Care 37:15–26PubMedCrossRefGoogle Scholar
  5. 5.
    Donaghy M (1996) The motor neurone diseases. In: Weatherall DJ, Ledingham JGG, Warrell DA (eds) Oxford textbook of medicine, Third Edition, Volume 3: Oxford University Press, Oxford, pp 4087–4090Google Scholar
  6. 6.
    Lacomblez L, Bensimon G, Leigh PN, et al for the Amyotrophic Lateral Sclerosis/Riluzole Study Group II (1996) Dose-ranging study of riluzole in amyotrophic lateral sclerosis. Lancet 347: 1425–1431PubMedGoogle Scholar
  7. 7.
    Leigh PN, Swash M (1995) Motor neurone disease: Biology and management. London. Springer-Verlag, London Berlin Heidelberg New York Paris Tokyo Hong Kong Barcelona BudapestGoogle Scholar
  8. 8.
    Nunnally JC, Bernstein IH (1994) Psychometric theory. Third edition. Mc-Graw Hill New YorkGoogle Scholar
  9. 9.
    Robinson I, Hunter M (1998) Motor neurone disease. Routledge, LondonGoogle Scholar
  10. 10.
    Shields RK, Ruhland JL, Ross MA, et al (1998) Analysis of health-related quality of life and muscle impairment in individuals with amyotrophic lateral sclerosis using the medical outcome survey and the Tufts Quantitative Neuromuscular Exam. Arch Phys Med Rehabil 97:855–862CrossRefGoogle Scholar
  11. 11.
    Swash M (1997) Health outcome and quality-of-life measurements in amyotrophic lateral sclerosis. J Neurol 244 [Suppl 2]: S26-S29PubMedCrossRefGoogle Scholar
  12. 12.
    Tandan R, Bradley WG (1985) Amyotrophic lateral sclerosis: Part 1. Clinical features, pathology, and ethical issues in management. Ann Neurol 18: 271–280PubMedCrossRefGoogle Scholar
  13. 13.
    The ALS CNTF treatment study group (ACTS) phase I-II Study Group (1996) The Amyotrophic Lateral Sclerosis Functional Rating Scale. Assessment of activities of daily living in patients with amyotrophic lateral sclerosis. Arch Neurol 53:141–147Google Scholar
  14. 14.
    Walker EA, Katon WJ, Jemelka RP (1993) Psychiatric disorders and medical care utilization among people in the general population who report fatigue. J Gen Intern Med 8:436–440PubMedCrossRefGoogle Scholar
  15. 15.
    Ware JE, Kosinski M, Keller SD (1994) SF-36 Physcial and mental Health Summary Scales: A User’s Manual. The Health Institute, New England Medical Center, Boston, MassachusettsGoogle Scholar
  16. 16.
    Ware JE Jr, Sherbourne CD (1992) The MOS 36-item short-form health survey (sf-36) I: Conceptual framework and item selection. Med Care 30: 473–483PubMedCrossRefGoogle Scholar

Copyright information

© Steinkopff Verlag 1999

Authors and Affiliations

  • Crispin Jenkinson
    • 1
    Email author
  • Cathy Brennan
    • 1
  • Ray Fitzpatrick
    • 2
  • Mark Bromberg
    • 3
  • Michael Swash
    • 4
    • 5
  1. 1.Health Services Research Unit, Division of Public Health & Primary Health CareUniversity of Oxford, Institute of Health SciencesOxfordUK
  2. 2.Nuffield CollegeOxfordUK
  3. 3.Department of Neurology, Neuromuscular Program and EMG LaboratoryThe University of UtahSalt Lake CityUSA
  4. 4.Department of Neurology, St Bartholomew’s(Queen Mary and Westfield College, University of London)LondonUK
  5. 5.Royal London Hospital School of Medicine and Dentistry(Queen Mary and Westfield College, University of London)LondonUK

Personalised recommendations