## Abstract Klinefelter's syndrome is known to be associated with various malignancies including male breast cancer and germ cell tumour. We report a patient with Klinefelter's syndrome who developed a primary B‐cell lymphoma of the central nervous system (CNS). To the best of our knowledge, this i
Primary central nervous system Burkitt's lymphoma presenting as Guillain-Barré syndrome
✍ Scribed by Toren, A. ;Mandel, M. ;Shahar, E. ;Rimmoni, E. ;Roizin, H. ;Neuman, Y. ;Brok-Simoni, F. ;Mark, Z. ;Biniaminov, M. ;Rosenthal, E. ;Segal, M. ;Frand, M. ;Ben-Bassat, I. ;Rechavi, G.
- Publisher
- John Wiley and Sons
- Year
- 1994
- Tongue
- English
- Weight
- 690 KB
- Volume
- 23
- Category
- Article
- ISSN
- 0098-1532
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✦ Synopsis
Abstract
A rare case of CNS Burkitt's lymphoma presenting as acute Guillain‐Barré syndrome is presented. A 6‐year‐old previously healthy female presented with acute onset of limb and truncal weakness, involvement of ocular and bulbar nerves, and areflexia. The clinical diagnosis of Guillain‐Barré syndrome prompted treatment with intravenous gammaglobulin with no response. A lumbar puncture following revealed marked pleocytosis, elevated protein, and decreased glucose. Immunological, cytological, and molecular studies of these cells confirmed the diagnosis of Burkitt's lymphoma IgM, kappa with t(8;14) and rearrangement of the J and kappa immunoglobulin chains. Aggressive systemic and intrathecal chemotherapy were started and within 5 days remission was achieved. The child is in complete remission 2 years from diagnosis. Although very rare, CNS lymphoma should be taken into account in every patient presenting with the clinical features of acute polyneuropathy. © 1994 Wiley‐Liss, Inc.
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