Respiratory function tests in amyotrophic lateral sclerosis: The role of maximal voluntary ventilation

Background: Pulmonary function tests are routinely used to measure progression in ALS. This study aimed to assess the change of various respiratory tests, in particular maximal voluntary ventilation (MVV), which evaluates respiratory endurance.Methods: A group of 51 patients were assessed 3 times (T1, T2, T3, separated by 5.4 months), including slow (SVC) and forced vital capacity (FVC), forced expiratory volume in 1 s (FEV1), peak expiratory flow (PEF), maximal inspiratory (MIP) and expiratory (MEP) pressures, MVV, and sniff nasal inspiratory pressure (SNIP). In addition, body mass index (BMI), ALSFRS-R and phrenic nerve responses were obtained 4 times. Patients with dementia and marked bulbar involvement were excluded.Results: Mean ALSFRS-R was high at entry (42.9) and its decline was moderately slow at 0.4/month. FVC and FEV1 declined significantly in the three time frames analysed. MVV reduced significantly only between T1-T3 and SVC between T2-T3, and MIP, MEP, PEF and SNIP did not change significantly. The amplitude and the latency of the motor response of the phrenic nerve changed significantly, and BMI declined significantly in most time periods, and ALSFRS-R changed significantly in the 4 time periods. We found a strong correlation between MVV, and FVC, SVC, FEV1, SNIP, phrenic nerve amplitude/area (p < 0.001), and markedly with PEF (rho = 0.821) and ALSFRS-R (rho = 0.713).Conclusions: Our study of early affected patients supports the use of a set of volitional and non-volitional respiratory tests to assess disease progression, rather than any single test. We found MVV a potentially useful marker of pulmonary function in ALS.