Myasthenia Gravis Presenting as Isolated Respiratory Failure

doi:10.1378/chest.97.1.232

Chest

Volume 97, Issue 1, January 1990, Pages 232-234

https://doi.org/10.1378/chest.97.1.232 Get rights and content

A patient with myasthenia gravis presenting as respiratory failure was unusual in his lack of peripheral neuromuscular involvement, negative results on many commonly used diagnostic tests, and lack of response to firstline therapeutic measures. Review of the pertinent literature revealed no previously described presentation of myasthenia gravis in this manner.

(Chest 1990; 97:232-34)

Section snippets

CASE REPORT

A 64-year-old water pipe installer was diagnosed as having obesity-hypoventilation syndrome associated with recurrent cor pulmonale. Three months earlier, he had presented to another hospital with peripheral edema and weight gain. Values of a room air blood gas analysis were as follow: Po₂, 61 mm Hg; Pco₂, 51 mm Hg; pH, 7.37; (Table 1) and the chest x-ray film showed a reduction in lung volumes interpreted as poor inspiratory effort. No other abnormalities were noted. Spirometry showed FEV₁ of

DISCUSSION

Myasthenia gravis can frequently be complicated by respiratory failure.¹⁵ However, myasthenic involvement limited solely to the muscles of ventilation has not been reported in well-characterized patients. In retrospective series of 22 myasthenics requiring mechanical ventilation reported by Gracey et al², only four presented in this manner. None was described as having isolated respiratory failure. The conclusions of two earlier series by Ferguson et al³ (31 patients) and Ashworth and Hunter⁴

CONCLUSIONS

This case of myasthenia gravis was unusual in three respects: the patient presented with what appeared to be primary respiratory failure of unknown etiology; associated symptoms of myasthenia were suggested by history but could not be verified by objective means. Diagnosis was difficult despite a correct clinical impression in that several edrophonium trials as well as other standard tests for myasthenia gravis were negative. Treatment following diagnosis with accepted firstline agents,

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Remission of myasthenia gravis following plasma-exchange
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DR Gracey et al.
Plasmapheresis in the treatment of ventilator-dependent myasthenia gravis patients: report of four cases
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RP Lisak et al.
Myasthenia gravis
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DR Gracey et al.
Mechanical ventilation for respiratory failure in myasthenia gravis: two-year experience with 22 patients
Mayo Clin Proc
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IT Ferguson et al.
Ventilatory failure in myasthenia gravis
J Neurol Neurosurg Psychiatry
(1982)

There are more references available in the full text version of this article.

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Weakness of respiratory muscles is common in neuromuscular disease. It contributes significantly to morbidity, loss of quality of life and is a major cause of mortality. It is therefore important for the clinical neurologist to be familiar with early signs of respiratory muscle compromise in order to start accurately timed treatment. The review outlines clinical symptoms and signs of respiratory muscle dysfunction and the differential diagnosis of neuromuscular diseases leading to respiratory muscle weakness. In addition to tests of respiratory function (spirometry, blood gas analysis etc.), the application of electrodiagnostic methods for trunk and respiratory muscles is guided by the clinical context. Comparable to most extremity muscles, the diaphragm can be examined with needle-EMG, neurography and repetitive stimulation. The continued breathing activitiy of respiratory muscles assists in identifying the chest wall muscle layers. Due to the small muscle bulk of the diaphragm and the small number of safe access points, the number of possible insertion sites is more limited compared to extremity muscles.
Pediatric myasthenia gravis and velopharyngeal incompetence
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Objective: To determine the clinical course of velopharyngeal incompetence in children with myasthenia gravis (MG). Methods: A 30-year retrospective study was performed using the medical records of 538 children who presented with velopharyngeal inadequacy (VPI) to a tertiary care academic pediatric center. Children with velopharyngeal incompetence due to myasthenia gravis were identified and their clinical courses were reviewed. Results: Four children were identified with velopharyngeal incompetence associated with myasthenia gravis. All four children required intervention for improvement of speech intelligibility. A speech prosthesis was the uniform intervention. Conclusion: Neonatal myasthenia gravis patients should be followed long-term as symptoms may recur as speech impairment. In addition, a high index of suspicion for this entity is required for early diagnosis due to the highly variable presentation and clinical course.
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Chest

Selected ReportsMyasthenia Gravis Presenting as Isolated Respiratory Failure

Section snippets

CASE REPORT

DISCUSSION

CONCLUSIONS

Lancet

Chest

Myasthenia gravis

Mechanical ventilation for respiratory failure in myasthenia gravis: two-year experience with 22 patients

Mayo Clin Proc

Ventilatory failure in myasthenia gravis

J Neurol Neurosurg Psychiatry

Selected Reports
Myasthenia Gravis Presenting as Isolated Respiratory Failure