Prognostic factors for survival in patients with amyotrophic lateral sclerosis: analysis of a multi-centre clinical trial

• This study reports factors influencing survival and quality of life in amyotrophic lateral sclerosis (ALS).
• Older age, bulbar onset disease and a shorter diagnostic delay were poor prognostic factors.
• Low baseline % predicted forced vital capacity and low baseline manual muscle test scores were also poor prognostic factors.
• Low weight and reduced ability to climb stairs correlated with poor quality of life.
• Gastrostomy and non-invasive ventilation had no positive impact on quality of life.

Information regarding factors influencing prognosis and quality of life (QoL) in patients with amyotrophic lateral sclerosis (ALS) is useful for clinicians and also for patients and their carers. The aims of this study are to identify prognostic factors for survival in ALS and to determine the physical factors influencing QoL. This study is a retrospective analysis of a cohort of 512 patients who participated in a phase II/III clinical trial of olesoxime. Cox multivariate regression analysis found older age, bulbar onset disease, low baseline forced vital capacity, low baseline manual muscle test (MMT) scores and a shorter diagnostic delay to be independently associated with poor survival outcome. Physical factors shown to have the strongest correlation with poor QoL were low weight and a reduced ability to climb stairs. Therapeutic interventions including gastrostomy and non-invasive ventilation had no positive impact on QoL in this cohort. The prognostic factors for survival identified here are consistent with other studies of ALS patients, with the additional identification of baseline MMT score as another predictor of prognosis. Furthermore, the correlation between both weight and poor lower limb function with QoL is novel and underlines the importance of careful nutritional management in this hypercatabolic condition.