Abstract

AbstractWe report an 81‐year‐old woman with anti‐muscle‐specific kinase (MuSK) antibody‐positive myasthenia gravis (MG) associated with solitary lymphadenopathy and insidious encephalopathy. At the age of 74, her myasthenic symptoms were remitted by immunosuppressive therapy and solitary abdominal lymphadenopathy was removed from the mesentery. Histopathological examinations showed follicular hyperplasia with marked IgG4‐positive plasma cell infiltration in the interfollicular connective tissues. Simultaneous head MRI examinations revealed diffuse brain lesions predominantly in the white matter without any neurological symptoms. The biopsied brain specimen showed scattered lymphocyte infiltration around vessels and reactive gliosis without IgG4‐positive plasma cells suggesting an autoimmune‐like encephalopathy. At the age of 81, MuSK‐MG was remittent, but the head MRI abnormalities sustained.

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