. Association Between Decline in Slow Vital Capacity and Respiratory Insufficiency, Use of Assisted Ventilation, Tracheostomy, or Death in Patients With Amyotrophic Lateral Sclerosis. JAMA Neurol. 2018 Jan 1;75(1):58-64. PubMed.

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  1. It is not clear in ALS that the SVC decline is linear during the full course of the disease. One may expect ceiling and floor effects. Nevertheless, and although it is surely difficult to evaluate SVC in patients with moderate to severe bulbar involvement and in late stages of the disease, SVC is, in fact, a good biomarker of respiratory involvement and respiratory function decline in ALS. The significant decline when patients are only "in need of emergency ventilation or within three months of death" suggests either a poor follow-up of the patients or patients with extremely fast disease progression.

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  1. In ALS, Respiratory Measure Predicts Pace of Disease