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Similar to Gazzola et al.,1 we treated a patient with primary marginal zone leptomeningeal lymphoma. A 58-year-old man presented with headaches, papilledema, and confusion on a background of left face/arm paresthesias for 3 years, attributed to migraine. CT brain scan showed sulcal-pattern right frontal hyperdensity, suspicious for subarachnoid blood, but normal size ventricles. Catheter arteriogram was normal. MRI showed frontal, basal, and spinal leptomeningeal enhancement. Lumbar punctures showed persistently elevated CSF pressure (>30 cm H2O) and protein (3.95 g/L) with monoclonal B lymphocytes and reactive T cell. Leptomeningeal biopsy showed a low-grade B cell neoplasm. Biopsy flow cytometry showed CD19, CD20, CD22, and FMC7-positive, kappa light-chain restricted B cells interpreted as low-grade marginal zone lymphoma. The patient was given high-dose methotrexate, rituximab IV, and intrathecal methotrexate for 6 weeks without clear response, then intrathecal rituximab plus methotrexate, with some improvement in CSF and MRI abnormalities. Our patient similarly had 2–3 years of intermittent cortical symptoms, then confusion and neuroimaging suggested subarachnoid hemorrhage. In addition, our patient also developed pseudotumor cerebri,2 probably due to very high CSF protein, and had incomplete response to chemotherapy. He completed a course of low-dose (2 Gy x 2) craniospinal radiotherapy and is now headache-free. These observations expand the clinical spectrum of chronic low-grade leptomeningeal lymphoma.
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Author disclosures are available upon request (journal{at}neurology.org).
- © 2017 American Academy of Neurology
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