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• Vol.60 No.11
• CLINICA NEUROL, 60: 791−794, 2020
• Vol.60 No.11 contents

Case Report

A case of anti-MuSK positive MG with recurrent right-sided heart failure as the initial manifestation

Yasuko Matsumoto, M.D., Ph.D.¹⁾, Keisuke Shima, M.D., Ph.D.¹⁾²⁾, Kazuyoshi Yamaguchi, M.D.¹⁾ and Ai Shimizu, M.D.³⁾

¹⁾ Department of Neurology, Ishikawa Prefectural Central Hospital
²⁾ Department of Neurology, Toyama Prefectural Central Hospital
³⁾ Department of Neurology, Kanazawa Medical Center

We report a case of anti-MuSK antibody (Ab)-positive myasthenia gravis (MG) in a patient who developed recurrent right-sided congestive heart failure. The patient presented with right-sided congestive heart failure of unknown etiology, necessitating hospitalization on three occasions over a 6-month period. During the third episode of hospitalization, she developed disturbance of consciousness, and heart failure was attributed to carbon dioxide narcosis. We performed various investigations including an anti-MuSK Ab assay, which showed positive results, and she was diagnosed with MG based solely on anti-MuSK Ab positivity. Selective plasma exchange did not produce a satisfactory therapeutic effect, and she received additional intravenous immunoglobulin, plasmapheresis, and oral immunosuppressive therapy after which she was successfully weaned off the ventilator. This case report highlights the following points: (a) Recurrent right-sided congestive heart failure may be the first manifestation of anti-MuSK Ab-positive MG and, (b) detection of the anti-MuSK Ab alone is a convincing rationale to diagnose patients with MG.
Full Text of this Article in Japanese PDF (1123K)

(CLINICA NEUROL, 60: 791−794, 2020)
key words: MuSK antibody, myasthenia gravis, right heart failure, first manifestation

(Received: 8-May-20)