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A Unique Case of Encephalopathy with an Elevated IgG-4 and Extremely High Interleukin-6 Level and Delayed
Namiko Matsumoto1, Nozomi Hishikawa1, Ken Ikegami1
1Departments of Neurology, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, Japan.
Insights
A rare case of neurological symptoms linked to high Interleukin-6 (IL-6) was treated successfully with immunosuppressants. The patient later developed myelodysplastic syndrome (MDS), suggesting a potential connection to elevated IL-6 levels.
Area of Science:
- Immunology
- Neurology
- Hematology
Background:
- A 75-year-old male presented with altered consciousness, characterized by cerebrospinal fluid findings of polynuclear cell-dominant pleocytosis, low glucose, and extremely high Interleukin-6 (IL-6) levels.
- Lymph node biopsy revealed inflammatory cell infiltration positive for IgG, IgG4, and IL-6.
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.
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