title: Cortical drive to breathe in amyotrophic lateral sclerosis: a dyspnoea-worsening defence?
abstract: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease causing diaphragm weakness that can be partially compensated by inspiratory neck muscle recruitment. This disappears during sleep, which is compatible with a cortical contribution to the drive to breathe. We hypothesised that ALS patients with respiratory failure exhibit respiratory-related cortical activity, relieved by noninvasive ventilation (NIV) and related to dyspnoea.We studied 14 ALS patients with respiratory failure. Electroencephalographic recordings (EEGs) and electromyographic recordings of inspiratory neck muscles were performed during spontaneous breathing and NIV. Dyspnoea was evaluated using the Multidimensional Dyspnea Profile.Eight patients exhibited slow EEG negativities preceding inspiration (pre-inspiratory potentials) during spontaneous breathing. Pre-inspiratory potentials were attenuated during NIV (p=0.04). Patients without pre-inspiratory potentials presented more advanced forms of ALS and more severe respiratory impairment, but less severe dyspnoea. Patients with pre-inspiratory potentials had stronger inspiratory neck muscle activation and more severe dyspnoea during spontaneous breathing.ALS-related diaphragm weakness can engage cortical resources to augment the neural drive to breathe. This might reflect a compensatory mechanism, with the intensity of dyspnoea a negative consequence. Disease progression and the corresponding neural loss could abolish this phenomenon. A putative cognitive cost should be investigated.
authors.affiliation: UMRS1158 Neurophysiologie Respiratoire Expérimentale et Clinique, Sorbonne Universités, UPMC Université Paris 06, INSERM, Paris, France Service de Pneumologie et Réanimation Médicale (Département R3S), AP-HP, Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Paris, France Service de Pneumologie et Réanimation Respiratoire, Centre Hospitalier Universitaire de Dijon, Dijon, France.
authors.forename: Marjolaine
authors.initials: M
authors.lastname: Georges
authors.affiliation: UMRS1158 Neurophysiologie Respiratoire Expérimentale et Clinique, Sorbonne Universités, UPMC Université Paris 06, INSERM, Paris, France Service de Pneumologie et Réanimation Médicale (Département R3S), AP-HP, Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Paris, France.
authors.forename: Elise
authors.initials: E
authors.lastname: Morawiec
authors.affiliation: UMRS1158 Neurophysiologie Respiratoire Expérimentale et Clinique, Sorbonne Universités, UPMC Université Paris 06, INSERM, Paris, France Service de Pneumologie et Réanimation Médicale (Département R3S), AP-HP, Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Paris, France Département d'Anesthésie et Réanimation, AP-HP, Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Paris, France.
authors.forename: Mathieu
authors.initials: M
authors.lastname: Raux
authors.affiliation: UMRS1158 Neurophysiologie Respiratoire Expérimentale et Clinique, Sorbonne Universités, UPMC Université Paris 06, INSERM, Paris, France Service de Pneumologie et Réanimation Médicale (Département R3S), AP-HP, Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Paris, France Service de Pneumologie et Réanimation Respiratoire, Centre Hospitalier Universitaire de Dijon, Dijon, France.
authors.forename: Jésus
authors.initials: J
authors.lastname: Gonzalez-Bermejo
authors.affiliation: Service de Neurologie, AP-HP, Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Paris, France.
authors.forename: Pierre-François
authors.initials: PF
authors.lastname: Pradat
authors.affiliation: UMRS1158 Neurophysiologie Respiratoire Expérimentale et Clinique, Sorbonne Universités, UPMC Université Paris 06, INSERM, Paris, France Service de Pneumologie et Réanimation Médicale (Département R3S), AP-HP, Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Paris, France Service de Pneumologie et Réanimation Respiratoire, Centre Hospitalier Universitaire de Dijon, Dijon, France Both authors contributed equally.
authors.forename: Thomas
authors.initials: T
authors.lastname: Similowski
authors.affiliation: UMRS1158 Neurophysiologie Respiratoire Expérimentale et Clinique, Sorbonne Universités, UPMC Université Paris 06, INSERM, Paris, France Service de Pneumologie et Réanimation Médicale (Département R3S), AP-HP, Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Paris, France Service de Pneumologie et Réanimation Respiratoire, Centre Hospitalier Universitaire de Dijon, Dijon, France Both authors contributed equally capucine.morelot@psl.aphp.fr.
authors.forename: Capucine
authors.initials: C
authors.lastname: Morélot-Panzini
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000503:physiopathology*; D003071:Cognition; D003964:Diaphragm; D004417:Dyspnea / Q000503:physiopathology*; D004569:Electroencephalography; D004576:Electromyography; D006801:Humans; D008875:Middle Aged; D018908:Muscle Weakness; D063087:Noninvasive Ventilation; D012119:Respiration; D012131:Respiratory Insufficiency / Q000503:physiopathology*; D012890:Sleep