Autoimmune-Associated Lymphocytic Central Nervous System (CNS) Vasculitis Presenting as a Tumefactive Cerebral Infarct Mimicking Multicentric Glioma

Vollständiger Abstract

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Purpose: Central nervous system vasculitis may rarely present as a tumor-like lesion with enhancement, edema, mass effect and multifocality, closely mimicking glioma. We report an uncommon case of autoimmune-associated lymphocytic CNS vasculitis presenting as a tumefactive cerebral infarct radiologically mimicking multicentric glioma. Methodology: Clinical history, neurological examination, preoperative imaging, operative findings, histopathology, immunohistochemistry, postoperative course and blood-based autoimmune serology were reviewed and summarized. Key Findings: A 50-year-old hypertensive woman presented with increased forgetfulness, slurring of speech, expressive speech difficulty and mild headache. MRI demonstrated an irregular, ill-defined, heterogeneously enhancing left fronto-temporo-parietal/perisylvian lesion with significant perilesional edema, mass effect, approximately 5 mm midline shift and an additional right cerebellar lesion, raising suspicion of multicentric glioma. She underwent exoscopic neuronavigation-guided maximum safe resection under intraoperative neurophysiological monitoring. Histopathology revealed cerebral infarction, while follow-up pathology/IHC assessment supported lymphocytic vasculitis. Blood-based autoimmune serology showed ANA, anti-dsDNA, anti-RNP/RNP-C, p-ANCA and ANCA-lactoferrin positivity. Corticosteroid therapy was initiated by the rheumatology team. Postoperatively, speech improved and no new neurological deficit was observed. Conclusion: Autoimmune-associated lymphocytic CNS vasculitis may present as a tumefactive cerebral infarct mimicking multicentric glioma. Tissue diagnosis is essential when clinical and radiological findings are discordant.