Objectives: Physical pain is a known symptom in amyotrophic lateral sclerosis (ALS), but no systematically derived prevalence estimate is available. The aim of this study was to determine the pooled prevalence of pain in ALS, relative to its method of measurement and pain characteristics. Methods: A systematic search across multiple databases was conducted on January 16, 2020. Random-effects meta-analyses of single proportions were performed on prevalence data. Heterogeneity was determined using the I2 statistic. Where available, pain location, intensity, and type or source were compared. Results: 2552 articles were identified. Twenty-one eligible studies were included. All studies used observational designs (14 cross-sectional, 6 cohort, 1 case-control). Pooled prevalence of pain in ALS across all studies was 60% (95% CI = 50-69%), with a high degree of heterogeneity (I2 = 94%, p < .001). Studies that used only validated measures had lower heterogeneity (I2 = 82%, p = 0.002), compared to those that used tailored measures, or tailored supplemented with validated measures (I2 = 90%, p < 0.001 and I2 = 83%, p < 0.001, respectively). In a subset of studies (N = 9), the most commonly reported pain location was the upper limbs including shoulders/extremities (41.5%). A further study subset (N = 7) showed moderate-severe intensity pain was most frequently reported. Type of pain was commonly related to cramp or spasm. Conclusions: Experiencing physical pain in ALS occurs with high prevalence. Deriving consensus on which specific tools should be used to assess, monitor and compare symptoms of pain in this population will reduce current heterogeneity in approaches and increase the likelihood of ameliorating distressing experiences more effectively.
Keywords: Pain; amyotrophic lateral sclerosis; meta-analysis; motor neurone disease.