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

Objective: The Tufts Quantitative Neuromuscular Exam (TQNE) is commonly used to assess the rate of disease progression in individuals with amyotrophic lateral sclerosis (ALS). The Medical Outcome Study Short Form (SF-36) is a general method to assess health-related quality of life (HRQL). This study examined the relationship between the TQNE and SF-36, established the reliability and responsiveness of each, and contrasted the HRQL between individuals with ALS and the general population. Design: Subjects (31) completed the SF-36 and TQNE within 1 week to determine reliability. Subjects (17) also completed both the TQNE and SF-36 each month for 1 year after diagnosis of ALS to establish the relationship between the two assessment tools. Setting: A primary care university teaching hospital. Patients: Thirty-one subjects with an age range of 27 to 76 years (mean 59.1, SD 10.32), recently diagnosed with ALS. Results: Each test was highly reliable and responsive. The intraclass correlations (2, 1) were consistently higher for the TQNE (.93 to .98) than for the SF-36 (.57 to .90). Changes in physical function were correlated to changes in lower extremity force megascores (.48). Conclusion: Both the TQNE and the SF-36 are reliable and responsive and appear important in characterization of patient status after ALS is diagnosed.