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Table 1 A literature review of case reports with MG and achalasia with and without thymoma

From: Myasthenia gravis with achalasia secondary to thymoma: a case report and literature review

 

Kaminski (1999) [16]

Kornizky et al. (2000) [13]

Rison (2009) [14]

Desuter et al. (2015) [11]

Seabi and Mokgoko (2020) [17]

Vinod (2020) [15]

The current study

Gender and age

F/ 75

F/ 69

M/ 83

M/ 79

M/ 35

M/ 11

M/ 43

Ethnicity

Unknown

Unknown

USA

Unknown

Unknown

Indian

Egyptian

GIT symptoms

Food sticking in the upper chest and regurgitation of undigested food

Dysphagia and occasional non-specific retrosternal pain

Painless secretions in the back of his throat inhibit swallowing

Aphagia, followed by nasogastric feeding, then percutaneous endoscopic gastrostomy

Dysphagia

Persistent vomiting, difficulty swallowing, and regurgitation

Dysphagia (food sticking in the upper chest and regurgitation of undigested food)

Other associated conditions

No

Polymyositis

DM2, arthritis, cervical spinal stenosis. Past peptic ulcers with a previous gastrointestinal bleed secondary to past steroid use. Left nephrectomy secondary to prior staghorn calculi, previous lumbar spine surgery, previous cholecystectomy, and previous bariatric surgery

No

No

No

No

Acetylcholine antibody titer

4.4 nmol/ L (negative if < 0.4 nmol/l)

Positive

7.94 nmol/ L (negative if < 0.4 nmol/l)

N/A

N/A

0.69 nmol/l (serum reference value < 0.25 nmol/l)

7.9 nmol/l (negative if < 0.4 nmol/l)

CT chest

Unremarkable

Unremarkable

Unremarkable

Unremarkable

N/A

Dilated esophagus

Anterior mediastinal soft tissue mass iso-dense in the non-contrast study with minute foci of calcification with homogenous enhancement in the post-contrast study

Treatment of MG

Anticholinesterase medication

Six session PE

Pyridostigmine

Pyridostigmine (60 mg PO q 4 h) and azathioprine (50 mg PO bid, for the last 8–9 years). PE in case of MG crisis

Anticholinesterase medication

Anticholinesterase inhibitor and immunosuppression with prednisone and azathioprine

Steroids, Acetylcholinesterase inhibitor (pyridostigmine), and nifedipine

Acetylcholinesterase inhibitor (pyridostigmine). Immunosuppression with prednisolone and azathioprine. PE in case of MG crisis

Thymoma/type

Yes/ resection of mixed epithelial and lymphocytic thymoma one year before the symptoms

Recurrent thymoma and carcinoid tumor in the lung

No thymoma

No thymoma

No thymoma

Yes/ resection of thymoma in 2017

No thymoma

Yes/ resection of thymoma in 2022. Thymoma type B2, p T1

Esophageal motility study

Absence of lower esophageal sphincter relaxation with multiple peristalses and simultaneous contraction of the body

Absent peristaltic waves in the esophageal body, with a normal pressure in the LES (17 mmHg, normal 15–30)

Not done

Not done

Not done

Failure of the lower esophageal sphincter to relax, elevated basal pressure, and peristalsis of the esophageal body suggestive of achalasia cardia

HRM showed achalasia type II. Failure of LES relaxation, high resting LES pressure, and absent esophageal peristalsis (100%) with panesophageal pressurization to greater than 30 mmHg

Barium swallow/esophageal transit study

Moderate esophageal dilatation

Marked dilated esophageal body with a beak-like narrowing of the terminal portion

Prominent cricopharyngeal sphincter with incomplete relaxation and no evidence of any cricopharyngeal bar

N/A

N/A

Dilated esophagus

Dilated esophagus with bird beak appearance

Endoscopy findings

Not done

Not done

Not done

Primary aspiration did not reveal regurgitation or UES blockage, but it did identify a significant lack of hypopharyngeal contraction

N/A

Dilated esophagus

Dilated esophagus with retained fluid and spastic cardia

Pathology/ histopathology

Loss of myenteric ganglion cells was confirmed/ no antibody binds to the Auerbach plexus

Not done

Not done

Not done

Not done

 

Not done

Management of dysphagia/ response to the treatment

Botulinum toxin injection

Laparoscopic Heller myotomy. Improvement in swallowing and weight loss after the procedure

Prednisone and methotrexate. Some improvement in the dysphagia

Direct laryngoscopy followed by esophagoscopy with mechanical dilation of the cricopharyngeal sphincter. The patient’s swallowing markedly improved

Medical treatment of MG (pyridostigmine). The 4-year follow-up showed no deterioration in his oral feeding ability

N/A

Medical treatment for MG improves the dysphagia

Elective pneumatic balloon dilatation was successful and remarkably improved the patient’s symptoms

  1. CT computed tomography, DM2 diabetes mellitus type 2, F female, GIT gastrointestinal, HRM high-resolution esophageal manometry, LES lower esophageal sphincter, MG myasthenia gravis, M male, N/A not available, PO per oral, PE plasma exchange, UES upper esophageal sphincter