Central sleep apnoea in congenital muscular dystrophy.

Kryger, Meir H.; Steljes, Darlene G.; Yee, Woon Chee; Mate, Eva; Smith, Stephen A.; Mahowald, Mark

doi:10.1136/jnnp.54.8.710

Cited by 21 publications

(4 citation statements)

References 23 publications

(1 reference statement)

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“…Pulmonary function studies revealed that 19 (68%) of the 28 patients who underwent spirometry studies had restrictive syndrome ( PSG data for TST and percentage of sleep stages were compared with data taken from a review of current literature 31 , revealing that 64,5% (20) of our patients experienced a decrease in TST; 61,3% (19) had an increase in percent of stage 1 sleep and that 77,4% (24) experienced a reduction in percent of REM sleep.…”

Section: Resultsmentioning

confidence: 95%

“…The other 26 (81%) snored between 6 and up to 97% of their TST. Sleep disruption represented by an elevated number of movement arousals (MA) was observed in 5 patients ( Patients 3,10,19,23,26). All of these presented frequent snoring.…”

Section: Commentsmentioning

confidence: 99%

“…hypertension, congestive heart failure, morning headaches, orthopnea, snoring, or hypoxia induced nocturnal seizures 19 . Continuous and/or intermittent nocturnal positive pressure through the nose have been in use over recent years for the treatment of the hypoventilation associated to NM diseases, thus helping patients along the way to alter the natural evolution of their respiratory insufficiencies and to considerably increase their life expectancy even to the point of enhancing the quality of their life.…”

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confidence: 99%

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Sleep polygraphic parameters in neuromuscular diseases

Pradella¹

1994

Arq. Neuro-Psiquiatr.

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In a polysomnographic study of 32 neuromuscular patients-22 with a form of muscular dystrophy, 3 with a form of congenital myopathy, 4 with a form of spinal muscular atrophy, 1 with a recurrent form of polymyositis and 1 with osteogenesis imperfecta syndrome--of which 21 were nonambulatory, we observed sleep related respiratory disturbances represented by: drops in oxygen saturation (SaO2), cardiac arrhythmia, sleep disruption, apneas, tachypnea, tachycardia and snoring. Nine out of the cohort of 32 patients presented with significant desaturations periods. These patients presented with an associated restrictive syndrome and thoracic deformities, some with tachypnea and/or SaO2 below 90% during wakefulness. In this group, snoring was observed in those patients with a form of muscular dystrophy while tachypnea was observed in patients who presented the highest desaturations levels. Sleep quantification revealed an increase of stage 1 sleep coupled with a decrease or even total absence of REM sleep. This is, we believe, a likely consequence of episodic desaturations that may accompany sleep hypoventilation which is potentialised during REM sleep stage.

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confidence: 95%

Section: Commentsmentioning

confidence: 99%

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Sleep polygraphic parameters in neuromuscular diseases

Pradella¹

1994

Arq. Neuro-Psiquiatr.

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“…Neuromuscular disorders, particularly Duchenne's and other forms of muscular dystrophy [6,7,[95][96][97][98][99][100][101][102], are also typically associated with hypoventilation, which becomes more severe as the disease progresses [101,102], particularly during sleep. Muscular dystrophy produces respiratory insufficiency because of progressive degeneration of the muscles of respiration, and respiratory failure is the major cause of death in this condition, although cardiomyopathy due to degeneration of cardiac muscle is also a common finding [103].…”

Section: Neurological and Neuromuscular Disordersmentioning

confidence: 99%

Impact of sleep in respiratory failure

McNicholas¹

1997

Eur Respir J

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Sleep has a physiological influence on respiration, which can have major adverse effects on gas exchange in patients with respiratory insufficiency. These effects relate largely to a reduction in various stimulant inputs to the brainstem respiratory centre. Conditions that may be associated with sleep-related respiratory insufficiency range from pulmonary disorders (such as chronic obstructive pulmonary disease (COPD)), to central respiratory insufficiency (such as central alveolar hypoventilation), neurological and neuromuscular disorders (such as polio and muscular dystrophy), and thoracic cage disorders (such as kyphoscoliosis). All these conditions have in common the finding of hypoxaemia and hypercapnia, which become more pronounced during sleep. The relative hypoventilation, which is common to each condition, is due to varying combinations of an inadequate respiratory drive and an increase in the work of breathing. Management of respiratory insufficiency during sleep should be directed first at optimizing the underlying disorder, then at correcting hypoxaemia with controlled low-flow supplemental oxygen. Pharmacological therapy may be effective in some instances, but the choice of agent varies with the underlying disorder. Assisted ventilation is an important part of the management of advanced cases, and the recent development of intermittent positive pressure ventilation by nasal mask (NIPPV) has been an important advance in this area. Use of NIPPV during the night is associated with beneficial effects during the day, particularly improved awake gas exchange and respiratory muscle strength, in addition to less dyspnoea and improved quality of life. Electrophrenic pacing of the diaphragm is helpful in highly selected cases, particularly patients with central respiratory insufficiency and high quadriplegia, but is frequently complicated by the development of obstructive sleep apnoea.

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Sleep, Breathing, and Neurologic Disorders

Chokroverty

Provini

2017

Sleep Disorders Medicine

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Central sleep apnoea in congenital muscular dystrophy.

Cited by 21 publications

References 23 publications

Sleep polygraphic parameters in neuromuscular diseases

Sleep polygraphic parameters in neuromuscular diseases

Impact of sleep in respiratory failure

Sleep, Breathing, and Neurologic Disorders

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