The nature of respiratory muscle weakness in patients with late-onset Pompe disease.
Adult
Age of Onset
Aged
Case-Control Studies
Cross-Sectional Studies
Diaphragm
/ diagnostic imaging
Female
Glycogen Storage Disease Type II
/ complications
Humans
Magnetic Fields
Male
Middle Aged
Muscle Weakness
/ diagnosis
Neural Conduction
/ physiology
Phrenic Nerve
/ physiology
Physical Stimulation
Respiratory Muscles
/ diagnostic imaging
Spirometry
Diaphragm
Late-onset Pompe disease
Phrenic nerve conduction studies
Phrenic nerves
Respiratory muscle strength
Journal
Neuromuscular disorders : NMD
ISSN: 1873-2364
Titre abrégé: Neuromuscul Disord
Pays: England
ID NLM: 9111470
Informations de publication
Date de publication:
08 2019
08 2019
Historique:
received:
02
02
2019
revised:
31
03
2019
accepted:
18
06
2019
pubmed:
23
7
2019
medline:
25
8
2020
entrez:
23
7
2019
Statut:
ppublish
Résumé
Late-onset Pompe disease (LOPD) causes myopathy of skeletal and respiratory muscles, and phrenic nerve pathology putatively contributes to diaphragm weakness. The aim of this study was to investigate neural contributions to diaphragm dysfunction, usefulness of diaphragm ultrasound, and involvement of expiratory abdominal muscles in LOPD. Thirteen patients with LOPD (7 male, 51±17 years) and 13 age- and gender-matched controls underwent respiratory muscle strength testing, ultrasound evaluation of diaphragm excursion and thickness, cortical and cervical magnetic stimulation (MS) of the diaphragm with simultaneous recording of surface electromyogram and twitch transdiaphragmatic pressure (twPdi; n = 6), and MS of the abdominal muscles with recording of twitch gastric pressure (twPgas; n = 6). The following parameters were significantly reduced in LOPD patients versus controls: forced vital capacity (p<0.01), maximum inspiratory and expiratory pressure (both p<0.001), diaphragm excursion velocity (p<0.05), diaphragm thickening ratio (1.8 ± 0.4 vs. 2.6 ± 0.6, p<0.01), twPdi following cervical MS (12.0 ± 6.2 vs. 19.4 ± 4.8 cmH
Identifiants
pubmed: 31327549
pii: S0960-8966(19)30055-0
doi: 10.1016/j.nmd.2019.06.011
pii:
doi:
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
618-627Informations de copyright
Copyright © 2019 Elsevier B.V. All rights reserved.