Pulmonary Manifestations of Systemic Disease: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease II |

Myasthenia Gravis: Don't Let It Take Your Breath Away FREE TO VIEW

Mariam Alexander, MD; Pallavi Kopparthy, MBBS; Serena Grewal, MD; Vincent Frechette, MD
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SUNY Upstate Medical University, Syracuse, NY

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):1104A. doi:10.1016/j.chest.2016.08.1211
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SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease II

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Myasthenia gravis (MG) is an autoimmune neuromuscular disease characterized by fluctuating weakness. Respiratory weakness can occur in later stages in a subset of patients and is rarely an initial presenting symptom of the disease. Here, we report a case of a patient who presented with respiratory distress and was ultimately diagnosed and treated for anti-MuSK antibody positive MG, with complete resolution of symptoms.

CASE PRESENTATION: A 40 year old female with a history of autism and iron deficiency anemia presented for an elective endoscopy. During the procedure, she developed respiratory distress and was intubated. She was successfully extubated after the procedure but became apneic and desaturated, requiring re-intubation. Initial blood gases showed hypoxia and hypercarbia. Chest x-ray, CT-thorax and basic laboratory studies were negative for any causes that could explain her respiratory failure. She was eventually extubated but continued to require BiPAP support. Her neurological exam was notable for neck flexion weakness, difficulty sustaining effort and nasal voice. Reflexes were normal. LP and CT-head were negative. A barium swallow study showed oropharyngeal dysphagia with aspiration of thin liquids. Workup was then initiated to rule out neuromuscular disease and anti-MuSK antibody levels was found to be elevated at 83.4 nmol/L, which indicated anti-MuSK antibody positive MG. She received 5 sessions of IVIG with complete resolution of her respiratory failure, dysphagia and weakness. She completed a prednisone taper and is currently asymptomatic on pyridostigmine.

DISCUSSION: Myasthenia gravis is a progressive neuromuscular disorder, characterized by a decrease in available acetycholine receptors (AChR) at the neuromuscular junction. Most patients have detectable anti-AchR antibodies but a subset of patients have antibodies to muscle specific tyrosine kinase (MuSK), which is required for AchR clustering. Anti-MuSK antibody positive MG patients usually present with oculobulbar weakness. Myasthenic crisis, characterized by respiratory failure, is a late complication of MG . It occurs in 15-20% of patients and can be precipitated by infection, post-operative stress and specific medications. Anti-MuSK antibody positive MG patients are known to have a higher risk of respiratory failure [1], but isolated respiratory failure as the initial presenting symptom is unusual. The endoscopy and associated moderate sedation medications could have triggered a myasthenic crisis in our patient. Early recognition and treatment likely led to an improved clinical outcome.

CONCLUSIONS: This case highlights the need to consider neuromuscular disorders in cases of unexplained respiratory failure in a critical care setting.

Reference #1: Evoli A et. al. Clinical correlates with anti-MuSK antibodies in generalized seronegative myasthenia gravis. Brain. 2003 Oct; 126:2304-11

DISCLOSURE: The following authors have nothing to disclose: Mariam Alexander, Pallavi Kopparthy, Serena Grewal, Vincent Frechette

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