University of Florida College of Medicine-Jacksonville Jacksonville, FL, United States
Aleem Ali, BSc, MBBS, Radhika Sharma, DO, Herit Vachhani, MD, Ron Schey, MD, FACG University of Florida College of Medicine-Jacksonville, Jacksonville, FL
Introduction: Oropharyngeal dysphagia and dysarthria are common in myasthenia gravis (MG) secondary to bulbar weakness and fatigue. At the onset of an acute myasthenic exacerbation, cholinergic agents and immunomodulating therapies are initiated or doses increased. When standard pharmacotherapy fails, non-neurologic etiologies of persisting dysphagia should be assessed. New onset dysphagia or dysphagia refractory to pharmacotherapy in elderly patients warrant evaluation to exclude an underlying sinister pathology.
Case Description/Methods: A 93-year-old female with a history of myasthenia gravis reported a 7-week history of dysphagia. Her dysphagia was thought secondary to MG-related dysmotility prompting treatment with immunoglobulin therapy. Dysmotility symptoms persisted despite optimal pharmacotherapy, requiring a nasogastric tube to facilitate nutrition and medication management. CT Chest/Abd excluded an obstructive etiology of her persisting dysphagia. Subsequent EGD revealed no evidence of stricture or stenosis. GI fluoroscopy with air contrast revealed dysmotility and dilation of the lower esophagus concerning for presybesophagus and likely element of achalasia (Fig 1). Esophageal manometry revealed elevated lower esophageal sphincter (LES) pressure. Repeat EGD with Endoflip revealed a tortuous esophagus with proximal dilation and a spastic LES. Endoflip findings were consistent with achalasia with a lack of relaxation of the LES. The patient underwent successful LES dilation with marked improvement in symptoms.
Discussion: The presentation of dysphagia secondary to MG can be difficult to differentiate from that related to achalasia. Contrast esophagram, manometry and upper GI endoscopy can assist in elucidating the underlying etiology. In patients with MG and dysphagia, esophageal manometric studies show a progressive depreciation in the amplitude of pharyngeal contractions with repeated swallows and normalization of amplitude after rest. In patients with achalasia, esophageal manometric studies show the conventional findings of aperistalsis in the distal portions of the esophagus, incomplete LES relaxation, and elevated LES pressures. Incomplete LES relaxation is key in the distinction between the dysphagia of MG and achalasia. Manometric studies in achalasia will show an elevated resting LES pressure >45 mmHg and a severely decreased or absent LES response to swallow as opposed to no difference in LES function in MG. New onset or persisting dysphagia in the elderly warrants a thorough evaluation.
Figure: Figure 1: GI fluoroscopy with air contrast revealed dysmotility and dilation of the lower esophagus (Image A). EGD revealed impaired LES relaxation (Image B) with EndoFlip (Image C) showing a lack of LES distensibility and impaired LES relaxation. Esoflip dilation (Image D) with post dilation view of the LES (Image E).
Disclosures: Aleem Ali indicated no relevant financial relationships. Radhika Sharma indicated no relevant financial relationships. Herit Vachhani indicated no relevant financial relationships. Ron Schey indicated no relevant financial relationships.
Aleem Ali, BSc, MBBS, Radhika Sharma, DO, Herit Vachhani, MD, Ron Schey, MD, FACG. P0360 - Dysphagia Refractory to Pharmacotherapy in a Myasthenic Patient, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.