Characteristics of respiratory muscle involvement in myotonic dystrophy type 1
| Autor: | Winfried Randerath, Carolin Henke, Peter Young, Matthias Boentert, Jens Spiesshoefer, Tobias Brix, Simon Herkenrath, Dennis Görlich, Hans-Joachim Kabitz |
|---|---|
| Rok vydání: | 2020 |
| Předmět: |
Adult
Male 0301 basic medicine Spirometry medicine.medical_specialty Nerve root Diaphragm Neural Conduction Stimulation Electromyography Severity of Illness Index Myotonic dystrophy 03 medical and health sciences 0302 clinical medicine Internal medicine medicine Respiratory muscle Humans Myotonic Dystrophy Muscle Strength Genetics (clinical) Ultrasonography Muscle Weakness medicine.diagnostic_test business.industry Middle Aged medicine.disease Respiratory Muscles Pathophysiology Respiratory Function Tests Diaphragm (structural system) Phrenic Nerve Cross-Sectional Studies 030104 developmental biology Neurology Case-Control Studies Pediatrics Perinatology and Child Health Cardiology Female Neurology (clinical) business 030217 neurology & neurosurgery |
| Zdroj: | Neuromuscular Disorders. 30:17-27 |
| ISSN: | 0960-8966 |
| DOI: | 10.1016/j.nmd.2019.10.011 |
| Popis: | 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 cmH2O, p |
| Databáze: | OpenAIRE |
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