• Alice Pirola, Elisa De Mattia, Andrea Lizio, Giulia Sannicolò, Elena Carraro, Fabrizio Rao, Valeria Sansone, and Christian Lunetta.
• NEuroMuscular Omnicentre, Fondazione Serena Onlus, Milan, Italy. Electronic address: alice.pirola@centrocliniconemo.it.
• Clin Neurol Neurosurg. 2019 Sep 1; 184: 105456.

ObjectiveAmyotrophic lateral sclerosis (ALS) patients tend to develop progressive respiratory muscle weakness, leading to ventilatory failure and ineffective cough, principal causes of morbidity and mortality. Since patients are usually unaware of these symptoms, these are generally not noticed until the advanced stages and are associated with poor prognosis. The monitoring of respiratory function on a regular basis is therefore of great importance. Despite the availability of several pulmonary function tests, none of them was found to be the best indicator of the disease progression throughout the course of this condition. The main aim of our work was to evaluate the prognostic value of these respiratory measures evaluated in a brief period of observation and their correlation with motor functional impairments in an ALS cohort.Patients And MethodsPatients with ALS who had respiratory assessments performed and functional motor scales administered at baseline and six months later were included. All patients were assessed with forced vital capacity, both in seated and supine position (FVC; sFVC), peak expiratory flow (PEF), peak expiratory cough flow (PCEF), the revised ALS functional rating scale (ALSFRS-R), at baseline and after six months, and their disease progression rate (ΔFS) was obtained.ResultsWe included 73 patients with probable or definite ALS according to El-Escorial revised Criteria. At baseline, PCEF and PEF significantly correlated with ALSFRS-R total, bulbar and spinal subscores and ΔFS, while FVC% significantly correlated with ΔFS. After 6 months all the respiratory parameters significantly correlated with ALSFRS-R and all its subscores. Longitudinally, FVC%, sFVC% and PCEF significantly correlated with ΔFS and some of ALSFRS-R subscores. As concerns the survival analysis, monthly declines of FVC% and sFVC%, significantly correlated with the survival. The worse prognosis in terms of survival was finally found in those whose FVC% and sFVC% dropped below their respective cut-offs.ConclusionThroughout the course of ALS disease, the monitoring of several respiratory markers, namely FVC, sFVC, PEF and PCEF, plays a critical role in predicting the prognosis of these subjects, both in terms of survival and functional ability. The implementation of monthly cut-offs in the evaluation of FVC and sFVC may allow a faster recognition of those patients with worse prognosis and therefore an optimized tailored clinical care, as well as a better stratification in clinical trials.Copyright © 2019 Elsevier B.V. All rights reserved.

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