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Clinical features of laryngeal myasthenia gravis: A case series
XiangLi Yang1, Lin Niu1, ChunWei Yang1
1Department of Otolaryngology Head and Neck Surgery, Tianjin Union Medical Center, Tianjin 300121, China.
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.
Insights
Laryngeal myasthenia gravis (MG) is rare, often presenting as speech or swallowing issues. Early diagnosis is challenging, highlighting the need for specific tests like AChR antibody and RNS to confirm this underdiagnosed condition.
Area of Science:
- Neurology
- Autoimmune Diseases
- Clinical Research
Background:
- Myasthenia gravis (MG) is an autoimmune disorder.
- Laryngeal MG, characterized by exclusive initial symptoms like dysarthria and dysphagia, is rarely reported.
Purpose of the Study:
- To review and analyze the largest series of laryngeal MG patients.
- To understand the clinical presentation and diagnostic challenges of laryngeal MG.
Main Methods:
- Systematic review and analysis of 20 case reports/series.
- Inclusion of 30 patients with laryngeal MG as their primary manifestation.
Main Results:
- Dysarthria (14/30) and dysphagia (11/30) were the most common primary symptoms.
- Only 23.33% of patients received an MG diagnosis at their first visit; 63% initially saw an otolaryngologist.
- Acetylcholine receptor (AChR) antibody positivity was 45%, repetitive nerve stimulation (RNS) showed a decremental response in 52.9%, and neostigmine/edrophonium tests were positive in 92.6%.
Conclusions:
- Laryngeal MG is a rare, potentially underdiagnosed condition presenting with speech and swallowing difficulties.
- Fluctuating weakness, confirmed by neostigmine/edrophonium testing, is a key feature.
- AChR antibody and RNS testing are crucial for diagnosing laryngeal MG by assessing neuromuscular junction pathology.
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