Ann Rehabil Med.  2017 Dec;41(6):1082-1087. 10.5535/arm.2017.41.6.1082.

Bulbar Myasthenia Gravis Superimposed in a Medullary Infarction Diagnosed by a Fiberoptic Endoscopic Evaluation of Swallowing With Simultaneous Tensilon Application

• Kim SJ
• Park GY
• Choi YM
• Sohn DG
• Kang SR
• Im S

• KMID: 2400294
• DOI: http://doi.org/10.5535/arm.2017.41.6.1082

Abstract

In the elderly, myasthenia gravis (MG) can present with bulbar symptoms, which can be clinically difficult to diagnose from other neurological comorbid conditions. We describe a case of a 75-year-old man who had been previously diagnosed with dysphagia associated with medullary infarction but exhibited aggravation of the dysphagia later on due to a superimposed development of bulbar MG. After recovering from his initial swallowing difficulties, the patient suddenly developed ptosis, drooling, and generalized weakness with aggravated dysphagia. Two follow-up brain magnetic resonance imaging (MRI) scans displayed no new brain lesions. Antibodies to acetylcholine receptor and muscle-specific kinase were negative. Subsequent electrodiagnosis with repetitive nerve stimulation tests revealed unremarkable findings. A diagnosis of bulbar MG could only be established after fiberoptic endoscopic evaluation of swallowing (FEES) with simultaneous Tensilon application. After application of intravenous pyridostigmine, significant improvement in dysphagia and ptosis were observed both clinically and according to the FEES.

Keyword

Myasthenia gravis; Stroke; Edrophonium

MeSH Terms

Acetylcholine
Aged
Antibodies
Brain
Deglutition Disorders
Deglutition*
Diagnosis
Edrophonium*
Electrodiagnosis
Fees and Charges
Follow-Up Studies
Humans
Infarction*
Magnetic Resonance Imaging
Myasthenia Gravis*
Phosphotransferases
Pyridostigmine Bromide
Sialorrhea
Stroke
Acetylcholine
Antibodies
Edrophonium
Phosphotransferases
Pyridostigmine Bromide

Figure

• 

  Fig. 1 (A) Initial brain magnetic resonance imagery (MRI) of T2-weighted image show subacute infarction in the right medulla. After the onset of ptosis, a follow-up MRI of the brain demonstrated no acute lesion in the diffusion weighted image at (B) post 2 days and (C) 5 days.

• 

  Fig. 2 (A) Six days after onset of ptosis, videofluoroscopic swallowing study (VFSS) images demonstrated maximal amount of residues in the valleculae and pyriform sinus with overflow aspiration in consistencies in the lateral view. (B) Five weeks prior to the onset of ptosis, VFSS images had initially revealed mild residues in the valleculae and pyriform sinus with only mild aspiration in the lateral view.

• 

  Fig. 3 Videoendoscopic findings of the FEES-Tensilon test displayed (A) at baseline, prior to Tensilon application, continuous silent saliva aspiration and pooling of secretions in the pyriform sinus and (B) after applying a cumulative dose of 5 mg of Tensilon, improved clearance of secretions and tolerable swallowing of nectar bolus. (C) In comparison to pre-Tensilon application state, (D) ptosis significantly improved after applying a cumulative dose of 5 mg of Tensilon. FEES, fiberoptic endoscopic evaluation of swallowing.

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