Human diaphragm atrophy in amyotrophic lateral sclerosis is not predicted by routine respiratory measures. Issue 2 (14th February 2019)

• Record Type: Journal Article
• Authors: Guimarães-Costa, Raquel; Similowski, Thomas; Rivals, Isabelle; Morélot-Panzini, Capucine; Nierat, Marie-Cécile; Bui, Mai Thao; Akbar, David; Straus, Christian; Romero, Norma Beatriz; Michel, Patrick Pierre; Menegaux, Fabrice; Salachas, François; Gonzalez-Bermejo, Jésus; Bruneteau, Gaëlle
• Other Names: author non-byline.; Del Mar Amador Maria author non-byline.; Antoine Jean Christophe author non-byline.; Arne-Bes Marie Christine author non-byline.; Attali Valérie author non-byline.; Beauvais Katell author non-byline.; Brunaud-Danel Veronique author non-byline.; Bruneteau Gaelle author non-byline.; Camdessanche Jean-Philippe author non-byline.; Camu William author non-byline.; Carluer Laurence author non-byline.; Cassereau Julien author non-byline.; Chapart Maud author non-byline.; Chenuel Bruno author non-byline.; Chereau Nathalie author non-byline.; Cintas Pascal author non-byline.; Clavelou Pierre author non-byline.; Corcia Philippe author non-byline.; Cornette André author non-byline.; Couratier Philippe author non-byline.; Court-Fortune Isabelle author non-byline.; Cuvelier Antoine author non-byline.; Delclaux Christophe author non-byline.; Desnuelle Claude author non-byline.; Di Maria Sophie author non-byline.; Fargeot Catherine author non-byline.; Fauroux Brigitte author non-byline.; Finet-Monnier Armelle author non-byline.; Celine Fleury Marie author non-byline.; Gaillat Carmen author non-byline.; Georges Marjolaine author non-byline.; Giroud Maurice author non-byline.; Gonzalez-Bermejo Jesus author non-byline.; Greil Annick author non-byline.; Guerrier Muriel author non-byline.; Nicolas Guillaume author non-byline.; Guy Nathalie author non-byline.; Hannequin Didier author non-byline.; Juillien Valérie author non-byline.; Juntas-Morales Raul author non-byline.; Kessler Romain author non-byline.; Kolev Yvan author non-byline.; Lagarde Julien author non-byline.; Lautrette Geraldine author non-byline.; Le Cam Pierre author non-byline.; LeForestier Nadine author non-byline.; Lemasson Gwendal author non-byline.; Lenglet Thimothée author non-byline.; Magro Pascal author non-byline.; Mallart Anne author non-byline.; Martinaud Olivier author non-byline.; Meininger Vincent author non-byline.; Menegaux Fabrice author non-byline.; Meslier Nicole author non-byline.; Moirot Pierre author non-byline.; Morelot-Panzini Capucine author non-byline.; Nadjar Yann author non-byline.; Niel-Duriez Myriam author non-byline.; Nierat Marie-Cecile author non-byline.; Noullet Severine author non-byline.; Pageot Nicolas author non-byline.; Perez Thierry author non-byline.; Perrin Christophe author non-byline.; Pillet Odile author non-byline.; Pittion Sophie author non-byline.; Pouget Jean author non-byline.; Pradat Pierre-François author non-byline.; Prigent Hélène author non-byline.; Rabec Claudio author non-byline.; Ricard Jean-Damien author non-byline.; Romero Norma author non-byline.; Royer Catherine author non-byline.; Salachas François author non-byline.; Schaup Barbara author non-byline.; Sedkaoui Kamila author non-byline.; Similowski Thomas author non-byline.; Soriani Marie-Hélène author non-byline.; Straus Christian author non-byline.; Tanguy Marie-Laure author non-byline.; Tranchant Christine author non-byline.; Vandenberghe Nadia author non-byline.; Vershueren Annie author non-byline.; Viader Fausto author non-byline.; Wienalek-Bachelet Anne-Cecile author non-byline.;
• Is Part Of: European respiratory journal. Volume 53:Issue 2(2019)
• Publication Date: 2019-02-14
• Subjects: Respiratory organs -- Diseases -- Periodicals; Respiration -- Periodicals; 616.2
• DOI: 10.1183/13993003.01749-2018
• Languages: English
• ISSNs: 0903-1936

Abstract

Amyotrophic lateral sclerosis (ALS) patients show progressive respiratory muscle weakness leading to death from respiratory failure. However, there are no data on diaphragm histological changes in ALS patients and how they correlate with routine respiratory measurements. We collected 39 diaphragm biopsies concomitantly with laparoscopic insertion of intradiaphragmatic electrodes during a randomised controlled trial evaluating early diaphragm pacing in ALS (https://clinicaltrials.gov ; NCT01583088). Myofibre type, size and distribution were evaluated by immunofluorescence microscopy and correlated with spirometry, respiratory muscle strength and phrenic nerve conduction parameters. The relationship between these variables and diaphragm atrophy was assessed using multivariate regression models. All patients exhibited significant slow- and fast-twitch diaphragmatic atrophy. Vital capacity (VC), maximal inspiratory pressure, sniff nasal inspiratory pressure (SNIP) and twitch transdiaphragmatic pressure did not correlate with the severity of diaphragm atrophy. Inspiratory capacity (IC) correlated modestly with slow-twitch myofibre atrophy. Supine fall in VC correlated weakly with fast-twitch myofibre atrophy. Multivariate analysis showed that IC, SNIP and functional residual capacity were independent predictors of slow-twitch diaphragmatic atrophy, but not fast-twitch atrophy. Routine respiratory tests are poor predictors of diaphragm structural changes. Improved detection of diaphragm atrophy is essential for clinical practice and for management of trials specifically targeting diaphragm muscle function. Vital capacity and other widely used respiratory measures are poor predictors of diaphragmatic atrophy. Vital capacity should not be used as the sole selection criteria in clinical trials targeting diaphragm mass or function http://ow.ly/7GEg30mIpCK