Duchenne Muscular Dystrophy

Duchenne muscular dystrophy (DMD) is a degenerative disease of voluntary muscles that leads to generalized weakness and muscle wasting. It affects one in 3300 live male births. A less severe variant is known as Becker muscular dystrophy. Weakness of ventilatory muscles eventually develops in DMD patients, leading to eventual death typically in the third decade.

Respiratory insufficiency during sleep is the earliest sign of respiratory compromise in this patient population. REM-related hypoventilation and central apneas are common (80,81). Similarly to patients with MG, these patients are also at risk for isolated oxygen desaturation in REM sleep (Fig. 3). A bimodal presentation of SRBD was noted in 34 DMD patients, in which OSA was more prominent in the first decade and hypoventilation at the beginning of the second decade; lung function values did not accurately predict the severity of the SRBD (82). This study also recommends that each patient with SRBD be initiated on bilevel support at the start of treatment since transition from CPAP to bilevel was of short duration. Polysomnography should be considered when daytime PaCO2 is > 45 mmHg, and

FIGURE 3 Muscular dystrophy and REM-related oxygen desaturations. Hypnogram of a young male with muscular dystrophy showing isolated oxygen desaturations occurring in association with each period of REM sleep (arrow). Abbreviations: REM, rapid eye movement; SWS, slow-wave sleep.

the study should include continuous end tidal CO2 monitoring (82). Patients with DMD frequently require assistance to use CPAP/BPAP. They encounter similar issues as those seen in patients with MG or spinal cord disease.

Sleep Apnea

Sleep Apnea

Have You Been Told Over And Over Again That You Snore A Lot, But You Choose To Ignore It? Have you been experiencing lack of sleep at night and find yourself waking up in the wee hours of the morning to find yourself gasping for air?

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