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; Yosio Omote; Mami Takemoto; Toru Yamashita; Kohei Taniguchi; Koji Abe

doi:10.2169/internalmedicine.6098-20

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, Yosio Omote, Mami Takemoto, Toru Yamashita, Kohei Taniguchi, Koji Abe

Research output: Contribution to journal › Article › peer-review

1 Citation (Scopus)

Abstract

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.

Original language	English
Pages (from-to)	2125-2128
Number of pages	4
Journal	Internal Medicine
Volume	60
Issue number	13
DOIs	https://doi.org/10.2169/internalmedicine.6098-20
Publication status	Published - 2021

Keywords

Encephalopathy
IgG4 related disease
Interleukin-6
Multicentric Castleman’s disease

ASJC Scopus subject areas

Internal Medicine

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10.2169/internalmedicine.6098-20

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title = "A unique case of encephalopathy with an elevated igg-4 and extremely high interleukin-6 level and delayed myelodysplastic syndrome",

abstract = "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{\textquoteright}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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author = "Namiko Matsumoto and Nozomi Hishikawa and Ken Ikegami and Kota Sato and Yosio Omote and Mami Takemoto and Toru Yamashita and Kohei Taniguchi and Koji Abe",

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doi = "10.2169/internalmedicine.6098-20",

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journal = "Internal Medicine",

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publisher = "Japanese Society of Internal Medicine",

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T1 - A unique case of encephalopathy with an elevated igg-4 and extremely high interleukin-6 level and delayed myelodysplastic syndrome

AU - Matsumoto, Namiko

AU - Hishikawa, Nozomi

AU - Ikegami, Ken

AU - Sato, Kota

AU - Omote, Yosio

AU - Takemoto, Mami

AU - Yamashita, Toru

AU - Taniguchi, Kohei

AU - Abe, Koji

PY - 2021

Y1 - 2021

N2 - 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.

AB - 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.

KW - Encephalopathy

KW - IgG4 related disease

KW - Interleukin-6

KW - Multicentric Castleman’s disease

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JF - Internal Medicine

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ER -

A unique case of encephalopathy with an elevated igg-4 and extremely high interleukin-6 level and delayed myelodysplastic syndrome

Abstract

Keywords

ASJC Scopus subject areas

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