An Autopsy Case of Bing-Neel Syndrome: Discrepancy between the Radiological and Pathological Findings.
Sakino Matsuda1, Naohiro Sekiguchi2, Kenichi Ito2
1Neurology Division, National Hospital Organization Disaster Medical Center, Japan.
Internal Medicine (Tokyo, Japan)
|March 29, 2019
Summary
Bing-Neel syndrome (BNS), a rare complication of Waldenstrom's macroglobulinemia, can lead to widespread central nervous system infiltration. Autopsy revealed more extensive malignant cell infiltration than MRI suggested, highlighting BNS diagnostic challenges.
Area of Science:
- Neurology
- Hematology
- Oncology
Background:
- Waldenstrom's macroglobulinemia (WM) is a rare B-cell lymphoproliferative disorder.
- Bing-Neel syndrome (BNS) is a rare neurological complication of WM characterized by lymphoplasmacytic infiltration of the central nervous system.
Observation:
- A 64-year-old male with known WM presented with confusion.
- Cerebrospinal fluid (CSF) analysis revealed an elevated IgM index and monoclonal IgM protein, consistent with BNS.
- Magnetic resonance imaging (MRI) showed diffuse meningeal enhancement.
Findings:
- Despite treatment with intrathecal and systemic chemotherapy, the patient succumbed to pneumonia.
- Autopsy demonstrated extensive meningeal and perivascular infiltration by malignant cells throughout the brain and spine.
- The autopsy findings indicated a greater extent of central nervous system (CNS) involvement than detected by MRI.
Implications:
- BNS may present with more widespread CNS infiltration than typically visualized by standard neuroimaging.
- Early and accurate diagnosis of BNS is crucial for effective management.
- Further research into advanced imaging techniques and diagnostic markers for BNS is warranted.
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