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[Bickerstaff brainstem encephalitis preceding central nervous system relapse of diffuse large B-cell lymphoma]
Takuto Ishikawa1, Masakatsu Kawamura1, Junko Takeshita1
1Division of Hematology, Jichi Medical University Saitama Medical Center.
[Rinsho Ketsueki] the Japanese Journal of Clinical Hematology
|February 12, 2025
Summary
Bickerstaff brainstem encephalitis preceded central nervous system (CNS) relapse of diffuse large B-cell lymphoma (DLBCL). Prompt diagnosis and plasma exchange improved neurological symptoms, but CNS relapse eventually occurred, highlighting the need for careful differential diagnosis.
Area of Science:
- Neurology
- Oncology
- Immunology
Background:
- Diffuse large B-cell lymphoma (DLBCL) can involve the central nervous system (CNS).
- Bickerstaff brainstem encephalitis (BBE) is a rare autoimmune disorder.
- Distinguishing CNS lymphoma relapse from paraneoplastic syndromes is clinically challenging.
Purpose of the Study:
- To report a case of BBE preceding CNS relapse of DLBCL.
- To emphasize the importance of considering autoimmune encephalitis in lymphoma patients with neurological symptoms.
- To highlight diagnostic and therapeutic challenges in such cases.
Main Methods:
- Case report of a 65-year-old male with paranasal sinus DLBCL.
- Initial treatment with R-GCVP and high-dose methotrexate (HD-MTX).
- Diagnostic workup included MRI, cerebrospinal fluid (CSF) analysis, and anti-GQ1b IgG antibody testing. Treatment involved HD-MTX and plasma exchange.
Main Results:
- Patient achieved complete remission of DLBCL after initial treatment.
- Developed neurological symptoms suggestive of BBE, with positive anti-GQ1b IgG antibodies.
- Initial treatment for presumed CNS relapse (HD-MTX) was ineffective; plasma exchange led to neurological improvement.
- Recurrence of neurological symptoms and subsequent CSF analysis revealed B-cell clonality, confirming CNS relapse.
Conclusions:
- Autoimmune encephalitis, such as BBE, can mimic or precede CNS lymphoma relapse.
- Early consideration of autoimmune encephalitis is crucial in lymphoma patients presenting with neurological deficits.
- Accurate diagnosis is essential for appropriate treatment and management of these complex cases.
Keywords:
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