PMID- 31839403
OWN - NLM
STAT- MEDLINE
DCOM- 20210104
LR  - 20210104
IS  - 1873-2364 (Electronic)
IS  - 0960-8966 (Linking)
VI  - 30
IP  - 1
DP  - 2020 Jan
TI  - Characteristics of respiratory muscle involvement in myotonic dystrophy type 1.
PG  - 17-27
LID - S0960-8966(19)31169-1 [pii]
LID - 10.1016/j.nmd.2019.10.011 [doi]
AB  - The pathophysiology of respiratory muscle weakness in myotonic dystrophy type 1 
      (DM1) remains incompletely understood. 21 adult patients with DM1 (11 men, 
      42 +/- 13 years) and 21 healthy matched controls underwent spirometry, manometry, 
      and diaphragm ultrasound. In addition, surface electromyography of the diaphragm 
      and the obliquus abdominis muscle was performed following cortical and posterior 
      cervical magnetic stimulation (CMS) of the phrenic nerves or magnetic stimulation 
      of the lower thoracic nerve roots. Magnetic stimulation was combined with 
      invasive recording of the twitch transdiaphragmatic and gastric pressure (twPdi 
      and twPgas) in 10 subjects per group. The following parameters were reduced in 
      DM1 patients compared to control subjects: maximum inspiratory pressure (MIP; 
      40.3 +/- 19.2 vs. 95.8 +/- 28.5 cmH(2)O, p < 0.01), diaphragm thickening ratio (DTR; 
      2.0 +/- 0.4 vs. 2.7 +/- 0.6, p < 0.01), twPdi following CMS (10.8 +/- 8.3 vs. 
      21.4 +/- 10.1 cmH2O, p = 0.03), and amplitude of diaphragm compound muscle action 
      potentials (0.10 +/- 0.25 vs. 0.46 +/- 0.35 mV; p = 0.04). MIP and DTR were 
      significantly correlated with the muscular impairment rating scale (MIRS) score. 
      Maximum expiratory pressure (MEP) was reduced in DM1 patients compared to 
      controls (41.3 +/- 13.4 vs. 133.8 +/- 28.0 cmH2O, p < 0.01) and showed negative 
      correlation with the MIRS score. Pgas following a maximum cough was markedly 
      lower in patients than in controls (71.9 +/- 43.2 vs. 102.4 +/- 35.5 cmH(2)O) but 
      without statistical significance (p = 0.06). In DM1, respiratory muscle weakness 
      relates to clinical disease severity and involves inspiratory and probably 
      expiratory muscle strength. Axonal phrenic nerve pathology may contribute to 
      diaphragm dysfunction.
CI  - Copyright (c) 2019 Elsevier B.V. All rights reserved.
FAU - Henke, Carolin
AU  - Henke C
AD  - Respiratory Physiology Laboratory, Institute for Sleep Medicine and Neuromuscular 
      Disorders, University Hospital Muenster, Muenster, Germany.
FAU - Spiesshoefer, Jens
AU  - Spiesshoefer J
AD  - Respiratory Physiology Laboratory, Institute for Sleep Medicine and Neuromuscular 
      Disorders, University Hospital Muenster, Muenster, Germany.
FAU - Kabitz, Hans-Joachim
AU  - Kabitz HJ
AD  - Department of Pneumology, Cardiology and Intensive Care Medicine, Klinikum 
      Konstanz, Konstanz, Germany.
FAU - Herkenrath, Simon
AU  - Herkenrath S
AD  - Bethanien Hospital gGmbH Solingen, Solingen, Germany; Institute for Pneumology at 
      the University of Cologne, Solingen, Germany.
FAU - Randerath, Winfried
AU  - Randerath W
AD  - Bethanien Hospital gGmbH Solingen, Solingen, Germany; Institute for Pneumology at 
      the University of Cologne, Solingen, Germany.
FAU - Brix, Tobias
AU  - Brix T
AD  - Institute of Medical Informatics, University of Muenster, Muenster, Germany.
FAU - Gorlich, Dennis
AU  - Gorlich D
AD  - Insitute for Biostatistics and Clinical Research, University Hospital, Muenster, 
      Germany.
FAU - Young, Peter
AU  - Young P
AD  - Medical Park Klinik Reithofpark, Bad Feilnbach, Germany.
FAU - Boentert, Matthias
AU  - Boentert M
AD  - Respiratory Physiology Laboratory, Institute for Sleep Medicine and Neuromuscular 
      Disorders, University Hospital Muenster, Muenster, Germany. Electronic address: 
      matthias.boentert@ukmuenster.de.
LA  - eng
PT  - Journal Article
DEP - 20191105
PL  - England
TA  - Neuromuscul Disord
JT  - Neuromuscular disorders : NMD
JID - 9111470
SB  - IM
MH  - Adult
MH  - Case-Control Studies
MH  - Cross-Sectional Studies
MH  - Diaphragm/diagnostic imaging/physiopathology
MH  - Electromyography
MH  - Female
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Muscle Strength/physiology
MH  - Muscle Weakness/etiology/*physiopathology
MH  - Myotonic Dystrophy/complications/*physiopathology
MH  - Neural Conduction/physiology
MH  - Phrenic Nerve/*physiopathology
MH  - Respiratory Function Tests
MH  - Respiratory Muscles/diagnostic imaging/*physiopathology
MH  - Severity of Illness Index
MH  - Spirometry
MH  - Ultrasonography
OTO - NOTNLM
OT  - Diaphragm
OT  - Diaphragm ultrasound
OT  - Myotonic dystrophy type 1
OT  - Phrenic nerve conduction studies
OT  - Respiratory muscle weakness
EDAT- 2019/12/17 06:00
MHDA- 2021/01/05 06:00
CRDT- 2019/12/17 06:00
PHST- 2019/03/08 00:00 [received]
PHST- 2019/09/05 00:00 [revised]
PHST- 2019/10/29 00:00 [accepted]
PHST- 2019/12/17 06:00 [pubmed]
PHST- 2021/01/05 06:00 [medline]
PHST- 2019/12/17 06:00 [entrez]
AID - S0960-8966(19)31169-1 [pii]
AID - 10.1016/j.nmd.2019.10.011 [doi]
PST - ppublish
SO  - Neuromuscul Disord. 2020 Jan;30(1):17-27. doi: 10.1016/j.nmd.2019.10.011. Epub 
      2019 Nov 5.