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Steroid-Responsive Relapsing-Remitting Neutrophilic Encephalitis: A Case Report
Matthias N Ungerer1, Ricarda Diem1, Arne Wrede2,3
1Department of Neurology, University Hospital Heidelberg, Heidelberg, Germany.
Journal of Neuropathology and Experimental Neurology
|October 10, 2018
Summary
This study describes a unique case of granulocytic encephalitis, a relapsing-remitting brain inflammation. Prompt immunosuppressive treatment led to rapid improvement, suggesting a potential new immune-mediated central nervous system disease.
Area of Science:
- Neuroimmunology
- Neurology
- Pathology
Background:
- Encephalitis presents a diagnostic challenge, particularly when infectious etiologies are ruled out.
- Rapidly progressive neurological symptoms necessitate prompt and accurate diagnosis.
- Immune-mediated central nervous system (CNS) disorders are increasingly recognized.
Observation:
- A 22-year-old male presented with severe, rapidly progressing neurological decline.
- Cerebrospinal fluid analysis and brain biopsy revealed extensive neutrophilic granulocyte infiltration without infection.
- Magnetic resonance imaging (MRI) demonstrated multiple T2/FLAIR hyperintense lesions.
Findings:
- The patient showed rapid clinical and radiological improvement following high-dose steroid pulse therapy.
- A subsequent relapse also responded well to steroid treatment.
- Long-term remission was achieved with azathioprine, indicating an immune-mediated process.
Implications:
- This case suggests a novel immune-mediated relapsing-remitting granulocytic encephalitis.
- Immunosuppressive therapy should be considered in cases of unexplained neutrophilic encephalitis.
- Further research is warranted to characterize this potential new CNS disease entity.
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