<p>Ring-enhancing lesions on cerebral imaging may result from a broad range of underlying pathologies, with primary malignant brain tumors, metastases, and brain abscesses being the most common etiologies. Lymphomatoid granulomatosis (LYG) is an uncommon EBV-associated angiocentric and angio-destructive lymphoproliferative disease which most commonly affects the lungs. While extrapulmonary manifestations are observed frequently, isolated cerebral involvement is exceedingly rare. Herein, we report a case of an immunosuppressed 61-year-old woman who presented with progressive gait disturbance. Past medical history included myasthenia gravis and systemic lupus erythematosus, treated with immunosuppressants and prednisone. Magnetic resonance imaging showed a ring-enhancing intra-axial lesion in the left paracentral lobule. EEG demonstrated recurrent left parietal polyspikes, consistent with focal epilepsy. Stereotactic biopsy was performed for further diagnosis and neuropathology showed polymorphous, angiocentric cells, positive for CD20, CD45, and EBV, indicative of LYG, potentially related to preceding immunosuppression. This case highlights the diagnostic complexity of a rare etiology of a ring-enhancing cerebral lesion and emphasizes the importance of a structured and multimodal diagnostic approach.</p>

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A rare etiology of a solitary ring-enhancing lesion: isolated cerebral lymphomatoid granulomatosis

  • Jan Hellmeyer,
  • Zeynep Özdemir,
  • Andrea Kerkhoff,
  • Gerd Meyer zu Hörste,
  • Martin Hasselblatt,
  • Walter Stummer,
  • Michael Müther

摘要

Ring-enhancing lesions on cerebral imaging may result from a broad range of underlying pathologies, with primary malignant brain tumors, metastases, and brain abscesses being the most common etiologies. Lymphomatoid granulomatosis (LYG) is an uncommon EBV-associated angiocentric and angio-destructive lymphoproliferative disease which most commonly affects the lungs. While extrapulmonary manifestations are observed frequently, isolated cerebral involvement is exceedingly rare. Herein, we report a case of an immunosuppressed 61-year-old woman who presented with progressive gait disturbance. Past medical history included myasthenia gravis and systemic lupus erythematosus, treated with immunosuppressants and prednisone. Magnetic resonance imaging showed a ring-enhancing intra-axial lesion in the left paracentral lobule. EEG demonstrated recurrent left parietal polyspikes, consistent with focal epilepsy. Stereotactic biopsy was performed for further diagnosis and neuropathology showed polymorphous, angiocentric cells, positive for CD20, CD45, and EBV, indicative of LYG, potentially related to preceding immunosuppression. This case highlights the diagnostic complexity of a rare etiology of a ring-enhancing cerebral lesion and emphasizes the importance of a structured and multimodal diagnostic approach.