Mouth occlusion pressure at 100ms (P0.1) as a respiratory biomarker in amyotrophic lateral sclerosis.

Airway pressure in the first 100ms of an occluded inspiration (P0.1) evaluates the respiratory center activity, increasing in the presence of respiratory muscle weakness. It is uncertain if its activity can compensate for respiratory muscles weakness in amyotrophic lateral sclerosis (ALS). Methods: Consecutive ALS patients with P0.1 evaluated at first visit were included. Depending on P0.1 percentile, patients were divided in three groups: G1 (<25th percentile); G2 (25th-74th percentiles); G3 (≥75th percentile); two subgroups were further considered: SG0 (<10th percentile); SG1 (>90th percentile). Body mass index (BMI), functional ALS rating scale and its subscores, respiratory function tests, including forced vital capacity, maximal inspiratory (MIP) and expiratory pressures, percentage of P0.1 (%P0.1), blood gas analyses, phrenic nerve motor amplitude (MeanPhrenAmpl) were compared. P0.1/MIP and %P0.1 predictors were explored by linear and multinomial logistic regression analyses. p < 0.05 was considered as significant. Results: From the 497 patients included, 124 were in G1 and G3 each, 249 in G2, 49 in SG0 and SG1 each. G1 included more men, with higher BMI (p < 0.001). G3 had older women, with predominant bulbar phenotype (p < 0.001). Lower respiratory function (p < 0.05) was present in both groups. SG0 (%P0.1 < 51.73%, P0.1/MIP = 1.48 ± 1.02) had more spinal-onset men (p < 0.001) with lower MeanPhrenAmpl (p < 0.004). SG1 (P0.1 > 147.12, P0.1/MIP = 7.92 ± 4.62) predominantly included older patients (p = 0.033), women (p = 0.012), with lower MeanPhrenAmpl (p = 0.039). Discussion: ALS patients with respiratory failure can show high or low P01 values, related to phenotype. Possible central drive reactivity and exhaustion, and the role of respiratory-metabolic-renal buffering system should be further addressed.