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

We 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 inflam...

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Published in:Internal Medicine 2021/07/01, Vol.60(13), pp.2125-2128
Main Authors: Matsumoto, Namiko, Hishikawa, Nozomi, Ikegami, Ken, Sato, Kota, Omote, Yoshio, Takemoto, Mami, Yamashita, Toru, Taniguchi, Kohei, Abe, Koji
Format: Article
Language:English
Subjects:
Azathioprine
Biopsy
Case Report
Castleman's disease
Cerebrospinal fluid
Clavicle
Cytokines
Encephalopathy
IgG4 related disease
Immunoglobulin G
Inflammation
Interleukin 6
Internal medicine
Lymph nodes
multicentric Castleman's disease
Myelodysplastic syndrome
Pleocytosis
Prednisolone
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container_end_page 2128
container_issue 13
container_start_page 2125
container_title Internal Medicine
container_volume 60
creator Matsumoto, Namiko
Hishikawa, Nozomi
Ikegami, Ken
Sato, Kota
Omote, Yoshio
Takemoto, Mami
Yamashita, Toru
Taniguchi, Kohei
Abe, Koji
description We 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.
doi_str_mv 10.2169/internalmedicine.6098-20
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subjects Azathioprine
Biopsy
Case Report
Castleman's disease
Cerebrospinal fluid
Clavicle
Cytokines
Encephalopathy
IgG4 related disease
Immunoglobulin G
Inflammation
Interleukin 6
Internal medicine
Lymph nodes
multicentric Castleman's disease
Myelodysplastic syndrome
Pleocytosis
Prednisolone
title A Unique Case of Encephalopathy with an Elevated IgG-4 and Extremely High Interleukin-6 Level and Delayed Myelodysplastic Syndrome
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