Lack of correlation between the ventilatory response to CO2 and lung function impairment in myotonic dystrophy patients: evidence for a dysregulation at central level

Neuromuscular Disorders : NMD
Mathias PousselBruno Chenuel

Abstract

Myotonic dystrophy Type 1 (DM1) is the most common muscular dystrophy in adults. Respiratory failure is common but clinical findings support a dysregulation of the control of breathing at central level, furthermore contributing to alveolar hypoventilation independently of the severity of respiratory weakness. We therefore intended to study the relationship between the ventilatory response to CO2 and the impairment of lung function in DM1 patients. Sixty-nine DM1 patients were prospectively investigated (43.5 ± 12.7 years). Systematic pulmonary lung function evaluation including spirometry, plethysmography, measurements of respiratory muscle strength, arterial blood gas analysis and ventilatory response to CO2 were performed. Thirty-one DM1 patients (45%) presented a ventilatory restriction, 38 (55%) were hypoxaemic and 15 (22%) were hypercapnic. Total lung capacity decline was correlated to hypoxaemia (p = 0.0008) and hypercapnia (p = 0.0013), but not to a decrease in ventilatory response to CO2 (p = 0.194). Ventilatory response to CO2 was reduced to 0.85 ± 0.67 L/min/mmHg and not correlated to respiratory muscle weakness. Ventilatory response to CO2 was neither different among restricted/non-restricted patients (p = 0.2395) no...Continue Reading

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Citations

Feb 10, 2017·Muscle & Nerve·Catherine ThilMathias Poussel
May 10, 2017·Respirology : Official Journal of the Asian Pacific Society of Respirology·Fergal J O'DonoghueJean-Louis Pépin
Oct 21, 2018·Neurotherapeutics : the Journal of the American Society for Experimental NeuroTherapeutics·Samantha LoRussoW David Arnold
Dec 7, 2016·The European Respiratory Journal·Winfried RanderathPatrick Levy
Mar 22, 2019·Muscle & Nerve·Umesh Vivekananda, Chris Turner
Jun 9, 2017·PloS One·Morgana de Araújo EvangelistaGuilherme Augusto de Freitas Fregonezi
Oct 5, 2018·Journal of Neuromuscular Diseases·Haris BabačićStephan Wenninger
Jul 12, 2020·Acta neurologica Belgica·Marco MazzoliGiuliana Galassi
Feb 11, 2019·Current Neurology and Neuroscience Reports·Andrea RomigiAlessandro Sanduzzi
Oct 30, 2020·Frontiers in Neurology·Costanza SimonciniGabriele Siciliano
Nov 28, 2017·Revue de pneumologie clinique·P PriouF Gagnadoux
Apr 17, 2020·Respiration; International Review of Thoracic Diseases·Matthias BoentertValeria A Sansone
May 15, 2021·Current Opinion in Neurology·Federica Montagnese, Benedikt Schoser

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