Sleep apnoea in well-controlled myasthenia gravis and the effect of thymectomy

Amino, Akiyoshi; Shiozawa, Zenji; Nagasaka, Takaharu; Shindo, Koichiro; Ohashi, Kenichi; Tsunoda, S.; Sato, Shuzo

doi:10.1007/s004150050181

Cited by 45 publications

(42 citation statements)

References 5 publications

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“…Older patients with a higher BMI, lower TLC and abnormal daytime arterial blood gases are most vulnerable [16]. Sleep-disordered breathing and nocturnal desaturation may improve following treatment with thymectomy [17] or prednisolone [18].…”

Section: Myasthenia Gravismentioning

confidence: 99%

“…Duchenne9s muscular dystrophy (DMD) [6][7][8][9][10], and myotonic dystrophy (MD) [11][12][13][14], myopathies (e.g. nemaline myopathy [15]), Guillian-Barré syndrome and myasthenia gravis [16][17][18]. Respiratory muscle weakness, apnoeas and hypopnoeas, and abnormalities of breathing pattern and ventilatory drive may all contribute to nocturnal desaturation and sleep disruption, even before daytime ventilatory failure ensues.…”

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confidence: 99%

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Sleep and breathing in neuromuscular disease

Bourke¹,

Gibson²

2002

Eur Respir J

151

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Respiratory muscle weakness in neuromuscular disease causes significant morbidity and mortality. The published data on respiratory muscle activity and breathing during sleep in normal subjects, the impact of respiratory muscle weakness on sleep and breathing and the relations to daytime respiratory function in neuromuscular disease are reviewed here. In normal subjects during sleep upper airway resistance increases, chemosensitivity is reduced and the wakefulness drive to breathe is lost, resulting in a fall in ventilation. During rapid eye movement (REM) sleep, ribcage and accessory breathing muscles are suppressed, particularly during bursts of eye movements, and breathing is more irregular, rapid and shallow, with a further fall in ventilation.In subjects with respiratory muscle weakness sleep is fragmented, with shorter total sleep time, frequent arousals, an increase in stage 1 sleep and a reduction in, or complete suppression of, REM sleep. Sleep-disordered breathing and nocturnal desaturation are common and most severe during REM sleep. Correlations between daytime respiratory function and nocturnal desaturation are moderate or weak, but daytime respiratory function has greater prognostic value than nocturnal measurements.Noninvasive ventilation improves sleep quality and breathing in subjects with respiratory muscle weakness. However, the optimal criteria for initiation of ventilation and its role in rapidly progressive neuromuscular diseases are unclear.

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Section: Myasthenia Gravismentioning

confidence: 99%

mentioning

confidence: 99%

Sleep and breathing in neuromuscular disease

Bourke¹,

Gibson²

2002

Eur Respir J

151

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“…Only one study reported improvement of OSA after treatment of MG, but with thymectomy. 1 Our patient represents the fi rst report of OSA resolution after treatment of anti-MuSKR-Abpositive MG.…”

Section: Discussionmentioning

confidence: 62%

“…[1][2][3] MG is often caused by acetylcholine receptor antibodies (AchR-Abs) blocking neuromuscular transmission. However, approximately 40% of MG patients who do not have AchR-Abs have anti-muscle kinase receptor antibodies (anti-MuSKR-Ab).…”

mentioning

confidence: 99%

Resolution of Severe Obstructive Sleep Apnea after Treatment of Anti-Muscle Kinase Receptor-Positive Myasthenia Gravis Despite 60-Pound Weight Gain

Morgenstern

Singas

Zleik

et al. 2014

Journal of Clinical Sleep Medicine

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Obstructive sleep apnea (OSA) in patients with myasthenia gravis (MG) may be caused by reduced pharyngeal dilator muscle activity. We report a patient with anti-muscle kinase receptor MG with severe OSA and hypoventilation that resolved upon successful treatment of MG despite a 60-lb weight gain. Keywords: myasthenia gravis, obstructive sleep apnea, antimusk antibody, hypoventilation Citation: Morgenstern M, Singas E, Zleik B, Greenberg H. Resolution of severe obstructive sleep apnea after treatment of anti-muscle kinase receptor-positive myasthenia gravis despite 60-pound weight gain. J Clin Sleep Med 2014;10(7):813-814.http://dx.doi.org/10.5664/jcsm.3884 C A S E R E P O R T SM yasthenia gravis (MG) may contribute to development of obstructive sleep apnea (OSA) by inducing pharyngeal dilator muscle weakness, although not all studies have demonstrated this association.1-3 MG is often caused by acetylcholine receptor antibodies (AchR-Abs) blocking neuromuscular transmission. However, approximately 40% of MG patients who do not have AchR-Abs have anti-muscle kinase receptor antibodies (anti-MuSKR-Ab). This likely alters clustering of acetylcholine receptors during neuromuscular junction formation.4 MG has a variable clinical course that may be exacerbated by stress including infection, exertion or various drugs that may lead to "myasthenic crisis" with respiratory compromise. REPORT OF CASEA 45-year-old female with 6 months of headaches, weakness, dysarthria, dysphagia, and 60-pound weight loss (BMI 26 kg/m 2 ) was admitted for hypercapnic respiratory failure (ABGsupplemental O 2 : pH 7.27 / PCO 2 60 mm Hg / PO 2 99 mm Hg / HCO3 -28 mmol/L). Neurological evaluation showed fatigable diplopia, ptosis, and bi-facial and genioglossus weakness. Neck fl exor and extensor and proximal extremity muscles were spared. Formal swallowing evaluation confi rmed dysphagia. However, AchR-Abs were negative. Electromyography, nerve conduction studies and repetitive nerve stimulation (no decremental response) were not consistent with MG. No defi nitive neurological diagnosis was made. The patient was discharged on nocturnal bilevel positive airway pressure for treatment of persistent hypercapnia (ABG after 2 weeks: pH 7. -27 mm Hg), fatigue, dyspnea, and weakness persisted. Subsequent serology revealed antiMuSKR-Abs prompting treatment with plasma exchange, maintenance mycophenolate mofetil and prednisone, which normalized her neurological exam and muscle weakness. After 3 months, despite a 60-lb weight increase (BMI 35 kg/m 2 ), repeat diagnostic PSG showed resolution of OSA and nocturnal hypoventilation (AHI = 1/h; T90% = 0) with normal serum HCO3-(25 mmol/L). DISCUSSIONBoth insuffi cient neural drive to upper airway dilator muscles and anatomic factors that compromise the upper airway contribute to the pathophysiology of OSA. In MG, muscular strength varies based on muscle exertion, fl uctuating levels of autoantibodies that affect neuromuscular junction transmission, and other factors. The impact of MG may not be uniformly distr...

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“…Also sleep related disorders are among the clinical manifestations of MG which include sleep disordered breathing (SDB) [i.e. central sleep apnea (CSA), obstructive sleep apnea (OSA), mixed apnea (OSA and CSA), sleep-related hypoventilation or hypoxemic syndromes] [5][6][7][8][9][10], poor sleep quality, reduced sleep efficiency, reduced sleep and awakening quality, increased number of nocturnal awakenings, excessive daytime sleepiness, reduced rapid eye movement sleep (REM sleep) and altered sleep perception and dreaming [6,8,[11][12][13] and excessive limb movements during sleep [e.g. restless leg syndrome (RLS)] [14].…”

Section: Introductionmentioning

confidence: 99%

Sleep Related Conditions with Myasthenia Gravis: Evidence, Causes and Implications

Hamed¹

2016

SJPH

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Myasthenia gravis (MG) is an autoimmune disease caused mainly by antibodies against skeletal muscle nicotinic acetylcholine receptors (nAChRs) at the postsynaptic membrane resulting in depletion of acetylcholine at the neuromuscular junction (NMJ). Muscle fatigue is the cardinal symptom of MG. Respiratory muscle weakness and breathing problems are manifestations of MG even in mild stages of the disease resulting in significant morbidity and mortality. Also sleep related conditions are among the manifestations of MG which include sleep disordered breathing (SDB) [i.e. central (CSA) and obstructive (OSA) sleep apneas, hypoventilation or hypoxemic syndromes], reduced sleep efficiency and quality, increased number of nocturnal awakenings, excessive daytime sleepiness, reduced rapid eye movement sleep and altered sleep perception and dreaming. On the other hand, sleep abnormalities may result in lack of concentration, cognitive impairments and mood disturbances as depression and anxiety. Central and peripheral mechanisms have been suggested for the association between MG and central nervous system manifestations and sleep abnormalities. Neurologists and sleep medicine professionals should be aware of the associations between OSA/CSA, hypoventilation and other sleep related conditions with MG and consider systematic investigations including polysomnography and ventilatory support. This will reduce morbidity and mortality and improve quality of lives of patients with MG.

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Sleep apnoea in well-controlled myasthenia gravis and the effect of thymectomy

Cited by 45 publications

References 5 publications

Sleep and breathing in neuromuscular disease

Sleep and breathing in neuromuscular disease

Resolution of Severe Obstructive Sleep Apnea after Treatment of Anti-Muscle Kinase Receptor-Positive Myasthenia Gravis Despite 60-Pound Weight Gain

Sleep Related Conditions with Myasthenia Gravis: Evidence, Causes and Implications

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