The Role of Diaphragmatic Ultrasound in Early NIV Indication in ALS Patients

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Introduction: Non-invasive ventilation(NIV)is the main treatment of respiratory impairment in amiotrophyc lateral sclerosis(ALS).Its typically guided by pulmonary function tests(PFTs), which some patients,especially bulbar ones,cannot perform. Diaphragmatic ultrasound(DU)offers a non-invasive alternative,but reference values are limited, often linked to FVC <50%.This study evaluates DU for early diaphragmatic weakness diagnosis. Methods: Prospective study between 2021 and 2024.Included ALS patients without respiratory impairment. DU was made at the inclusion and at three months. Indication for NIV was made considering Early NIV,with an forced vital capacity (FVC)<70%.US was carried out on the right diaphragm,between the mid-axillary and posterior axillary lines. Diaphragmatic mobility was assessed using two formulas:diaphragmatic thickening fraction(TFDI)and diaphragmatic thickness index(ATMax). Results: 76 patients were included,49% male.85% had spinal-onset ALS,with an ALSFRS-R of 36 and average FVC of 103%.NIV was initiated in 17 patients during the second study visit. For an FVC value of 70%,TFDI showed a cut-off point of 82.2%,with a high negative predictive value (NPV) of 0.92,along with a sensitivity of 0.73 and a specificity of 0.64.For ATMax, the cut-off point was 0.64,also showing a high NPV of 0.93,with a sensitivity of 0.73 and a specificity of 0.79. Conclusion: Our findings demonstrate good sensitivity and specificity,highlighting a high NPV,which makes DU a valuable tool for the follow-up and management of these patients. ATMax appears to perform slightly better than TFDI. We have identified a new cut-off point for early diaphragmatic impairment in ALS patients, which could enable earlier indication of NIV.

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