• Internal medicine · Jul 2021

    Case Reports

    A Unique Case of Encephalopathy with an Elevated IgG-4 and Extremely High Interleukin-6 Level and Delayed Myelodysplastic Syndrome.

    • Namiko Matsumoto, Nozomi Hishikawa, Ken Ikegami, Kota Sato, Yoshio Omote, Mami Takemoto, Toru Yamashita, Kohei Taniguchi, and Koji Abe.
    • Departments of Neurology, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, Japan.
    • Intern. Med. 2021 Jul 1; 60 (13): 2125-2128.

    AbstractWe herein report a 75-year-old man who developed disturbed consciousness with polynuclear cell dominant pleocytosis and low glucose and extremely high interleukin (IL)-6 levels in his cerebrospinal fluid. The biopsy specimen from his right supraclavicular lymph node showed the infiltration of inflammatory cells positive for IgG, IgG4 and IL-6. Prednisolone and azathioprine administered under suspicion of IgG4-related disease (IgG4-RD) or multicentric Castleman's disease (MCD) successfully remitted the symptoms. However, he developed myelodysplastic syndrome (MDS) and died 18 months later. The extremely high IL-6 may have been related to the rare neurological manifestations and development of MDS in the present case.

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