Biomaterial Database

[Respiratory failure: respiratory disorder during sleep in patients with myotonic dystrophy]. 1995

T Takasugi, and T Ishihara, and J Kawamura, and T Kawashiro

National Higashisaitama Hospital.

Myotonic dystrophy (MyD) patients have been reported to show severe nocturnal desaturation related to sleep respiratory disorders. However, the reason of respiratory failure in MyD has remained unclear. In this study, ten patients with MyD underwent overnight polysomnography to evaluate the mechanisms which would cause respiratory failure, compared with Duchenne muscular dystrophy (DMD) patients in these three views: 1) sleep-related nocturnal desaturation, 2) histopathological evaluation of respiratory muscles, and 3) abnormalities of respiratory center. Nocturnal desaturation was more prominent in MyD rather than DMD. Apnea-hypopnea index (AHI) was higher in MyD than DMD. Type of respiratory disorder during sleep was mainly central apnea-hypopnea pattern, including Cheyne-Stokes respiration. In histopathological findings, central core change in respiratory muscle related to respiratory muscle fatigue was found less frequently in MyD than DMD. In respiratory center function, MyD showed hyporesponse to both alveolar hypercapnic and hypoxic stimulation. However, DMD showed normal response to both stimulations. We concluded that respiratory failure in patients with MyD would be attributed to respiratory center disorder rather than respiratory muscle weakness, which is the main cause of respiratory failure in patients with DMD.

UI	MeSH Term	Description	Entries
D009223	Myotonic Dystrophy	Neuromuscular disorder characterized by PROGRESSIVE MUSCULAR ATROPHY; MYOTONIA, and various multisystem atrophies. Mild INTELLECTUAL DISABILITY may also occur. Abnormal TRINUCLEOTIDE REPEAT EXPANSION in the 3' UNTRANSLATED REGIONS of DMPK PROTEIN gene is associated with Myotonic Dystrophy 1. DNA REPEAT EXPANSION of zinc finger protein-9 gene intron is associated with Myotonic Dystrophy 2.	Dystrophia Myotonica,Myotonic Dystrophy, Congenital,Myotonic Myopathy, Proximal,Steinert Disease,Congenital Myotonic Dystrophy,Dystrophia Myotonica 1,Dystrophia Myotonica 2,Myotonia Atrophica,Myotonia Dystrophica,Myotonic Dystrophy 1,Myotonic Dystrophy 2,PROMM (Proximal Myotonic Myopathy),Proximal Myotonic Myopathy,Ricker Syndrome,Steinert Myotonic Dystrophy,Steinert's Disease,Atrophica, Myotonia,Atrophicas, Myotonia,Congenital Myotonic Dystrophies,Disease, Steinert,Disease, Steinert's,Dystrophia Myotonica 2s,Dystrophia Myotonicas,Dystrophica, Myotonia,Dystrophicas, Myotonia,Dystrophies, Congenital Myotonic,Dystrophies, Myotonic,Dystrophy, Congenital Myotonic,Dystrophy, Myotonic,Dystrophy, Steinert Myotonic,Myopathies, Proximal Myotonic,Myopathy, Proximal Myotonic,Myotonia Atrophicas,Myotonia Dystrophicas,Myotonic Dystrophies,Myotonic Dystrophies, Congenital,Myotonic Dystrophy, Steinert,Myotonic Myopathies, Proximal,Myotonica, Dystrophia,Myotonicas, Dystrophia,PROMMs (Proximal Myotonic Myopathy),Proximal Myotonic Myopathies,Steinerts Disease,Syndrome, Ricker
D012125	Respiratory Center	Part of the brain located in the MEDULLA OBLONGATA and PONS. It receives neural, chemical and hormonal signals, and controls the rate and depth of respiratory movements of the DIAPHRAGM and other respiratory muscles.	Center, Respiratory,Centers, Respiratory,Respiratory Centers
D012132	Respiratory Muscles	These include the muscles of the DIAPHRAGM and the INTERCOSTAL MUSCLES.	Ventilatory Muscles,Respiratory Muscle,Muscle, Respiratory,Muscle, Ventilatory,Muscles, Respiratory,Muscles, Ventilatory,Ventilatory Muscle
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012891	Sleep Apnea Syndromes	Disorders characterized by multiple cessations of respirations during sleep that induce partial arousals and interfere with the maintenance of sleep. Sleep apnea syndromes are divided into central (see SLEEP APNEA, CENTRAL), obstructive (see SLEEP APNEA, OBSTRUCTIVE), and mixed central-obstructive types.	Apnea, Sleep,Hypersomnia with Periodic Respiration,Sleep-Disordered Breathing,Mixed Central and Obstructive Sleep Apnea,Sleep Apnea, Mixed,Sleep Apnea, Mixed Central and Obstructive,Sleep Hypopnea,Apnea Syndrome, Sleep,Apnea Syndromes, Sleep,Apneas, Sleep,Breathing, Sleep-Disordered,Hypopnea, Sleep,Hypopneas, Sleep,Mixed Sleep Apnea,Mixed Sleep Apneas,Sleep Apnea,Sleep Apnea Syndrome,Sleep Apneas,Sleep Apneas, Mixed,Sleep Disordered Breathing,Sleep Hypopneas