Clinical features of laryngeal myasthenia gravis: A case series

Publication date: Available online 5 November 2018

Source: American Journal of Otolaryngology

Author(s): XiangLi Yang, Liu Niu, ChunWei Yang, Lin Wang, JiXiang Liu, GuoYing He

Abstract

Background

Myasthenia gravis (MG) is an autoimmune disease. Dysarthria, dysphagia, and difficulty swallowing as exclusive initial and primary complaints in MG (laryngeal MG) are rare and seldom reported.

Methods

Here we review and analyze the largest series of laryngeal MG patients.

Results

A total of 30 patients with laryngeal MG as primary manifestation were found in 20 case reports/series. Dysarthria was the most frequent primary symptom (14/30), followed by dysphagia (11/30), slurred speech (4/30) and dysphonia (1/30). Sixty-three percent visited the otolaryngology department first. Only 23.33% of patients were diagnosed with MG at the first clinic visit. Forty-five percent laryngeal MG patients were acetylcholine receptor (AChR) antibody positive, 52.9% showed decremental response in the repetitive nerve stimulation (RNS) test, and 92.6% were positive in the neostigmine/edrophonium test. Fluctuating weakness was examined in 16 of 30 patients and observed in 14/16 patients.

Conclusion

Laryngeal MG is a rare and possibly under-diagnosed condition. The patients can present with dysarthria, dysphagia, or difficulty swallowing. Fluctuation in severity of disease by neostigmine/edrophonium test is a typical feature for MG patients. AChR antibody and RNS tests should be included to evaluate the pathologic changes in the neuromuscular junction.

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