Respiratory Muscle Function Tests and Diaphragm Ultrasound Predict Nocturnal Hypoventilation in Slowly Progressive Myopathies

Jens Spiesshoefer; Riccarda Lutter; Hans-Joachim Kabitz; Carolin Henke; Simon Herkenrath; Winfried Randerath; Peter Young; Michael Dreher; Dennis Görlich; Matthias Boentert

doi:10.3389/fneur.2021.731865

Respiratory Muscle Function Tests and Diaphragm Ultrasound Predict Nocturnal Hypoventilation in Slowly Progressive Myopathies

Front Neurol. 2021 Oct 14:12:731865. doi: 10.3389/fneur.2021.731865. eCollection 2021.

Authors

Jens Spiesshoefer^{1

2}, Riccarda Lutter¹, Hans-Joachim Kabitz³, Carolin Henke⁴, Simon Herkenrath^{5

6}, Winfried Randerath^{5

6}, Peter Young⁷, Michael Dreher², Dennis Görlich⁸, Matthias Boentert^{1

9}

Affiliations

¹ Department of Neurology With Institute for Translational Neurology, Muenster University Hospital, Muenster, Germany.
² Department of Pneumology and Intensive Care Medicine, Aachen University Hospital, Aachen, Germany.
³ Department of Pneumology, Cardiology, and Intensive Care Medicine, Klinikum Konstanz, Konstanz, Germany.
⁴ Department of Neurology, Herz-Jesu-Krankenhaus Münster-Hiltrup, Münster, Germany.
⁵ Bethanien Hospital gGmbH Solingen, Solingen, Germany.
⁶ Institute for Pneumology, University of Cologne, Solingen, Germany.
⁷ Medical Park Klinik Reithofpark, Bad Feilnbach, Germany.
⁸ Institute of Biostatistics and Clinical Research, University of Muenster, Muenster, Germany.
⁹ Department of Medicine, Universitätsklinikum Münster (UKM) Marienhospital, Steinfurt, Germany.

Abstract

Introduction: In slowly progressive myopathies, diaphragm weakness early manifests through sleep-related hypoventilation as reflected by nocturnal hypercapnia. This study investigated whether daytime tests of respiratory muscle function and diaphragm ultrasound predict hypercapnia during sleep. Methods: Twenty-seven patients with genetic myopathies (myotonic dystrophy type 1 and 2, late-onset Pompe disease, facioscapulohumeral dystrophy; 48 ± 11 years) underwent overnight transcutaneous capnometry, spirometry, measurement of mouth occlusion pressures, and diaphragm ultrasound. Results: Sixteen out of 27 patients showed nocturnal hypercapnia (peak p_tcCO₂ ≥ 50 mmHg for ≥ 30 min or increase in p_tcCO₂ by 10 mmHg or more from the baseline value). In these patients, forced vital capacity (FVC; % predicted) and maximum inspiratory pressure (MIP; % of lower limit or normal or LLN) were significantly reduced compared to normocapnic individuals. Nocturnal hypercapnia was predicted by reduction in FVC of <60% [sensitivity, 1.0; area under the curve (AUC), 0.82] and MIP (%LLN) <120% (sensitivity, 0.83; AUC, 0.84), the latter reflecting that in patients with neuromuscular disease, pretest likelihood of abnormality is per se higher than in healthy subjects. Diaphragm excursion velocity during a sniff maneuver excluded nocturnal hypercapnia with high sensitivity (0.90) using a cutoff of 8.0 cm/s. Conclusion: In slowly progressive myopathies, nocturnal hypercapnia is predicted by FVC <60% or MIP <120% (LLN). As a novelty, nocturnal hypercapnia can be excluded with acceptable sensitivity by diaphragm excursion velocity >8.0 cm/s on diaphragm ultrasound.

Keywords: diaphragm ultrasound; forced vital capacity; maximum inspiratory pressure; myopathy; nocturnal hypoventilation.