Researchers from the United States described the case of an 82-year-old female with achalasia and cricopharyngeal sphincter dysfunction who was later diagnosed with myasthenia gravis (MG).
There is limited data about the link between MG and achalasia, and the underlying mechanism is unknown, the researchers noted. Dysphagia and achalasia should be evaluated for the differential diagnosis of MG, they added.
Read more about the differential diagnosis of MG
“Treatment should be directed toward the underlying disease process,” the study authors wrote in a report published in the journal Cureus.
The patient presented to the hospital with worsening dysphagia and was diagnosed with cricopharyngeal sphincter and esophageal achalasia.
Her palatal elevation was significantly reduced, and she had a hypernasal voice, especially in the upright position, suggestive of vagus nerve involvement. She also had bulbar weakness.
She had a blood test to check for antiacetylcholine receptor antibodies, which came back positive.
Based on this result, the patient was diagnosed with MG.
She was treated with immunoglobulin, prednisone, and pyridostigmine, significantly improving her dysphagia.
“While myasthenia gravis leads to muscle weakness and should not cause achalasia, there have been a few case reports describing a link between the two disorders,” the researchers wrote. “Cricopharyngeal dysfunction, which is a common disorder causing dysphagia in the elderly, was also noted in the patient.”
Achalasia is a rare swallowing disorder affecting the esophagus, which makes it difficult for food and liquid to pass through to the stomach. It is usually caused by esophagal nerve damage causing the esophagus to become paralyzed and dilated over time and not being able to squeeze food down into the stomach.
Cricopharyngeal sphincter dysfunction occurs due to the upper esophageal sphincter not relaxing to allow food to enter the esophagus or relaxing in an uncoordinated manner, causing dysphagia.
Acharya S, Anwar S, Thapa K, Maroun R, Kilkenny TM. Achalasia and cricopharyngeal sphincter dysfunction in a patient with myasthenia gravis: a case report. Cureus. Published online July 27, 2023. doi:10.7759/cureus.42575