PRIMARY CENTRAL-NERVOUS-SYSTEM BURKITT-LYMPHOMA PRESENTING AS GUILLAIN-BARRE-SYNDROME

A rare case of CNS Burkitt's lymphoma presenting as acute Guillain-Bar re syndrome is presented. A 6-year-old previously healthy female prese nted with acute onset of limb and truncal weakness, involvement of ocu lar and bulbar nerves, and areflexia. The clinical diagnosis of Guilla in-Barre syndrome prompted treatment with intravenous gammaglobulin wi th no response. A lumbar puncture following revealed marked pleocytosi s, elevated protein, and decreased glucose. Immunological, cytological , and molecular studies of these cells confirmed the diagnosis of Burk itt's lymphoma IgM, kappa with t(8;14) and rearrangement of the J and kappa immunoglobulin chains. Aggressive systemic and intrathecal chemo therapy were started and within 5 days remission was achieved. The chi ld is in complete remission 2 years from diagnosis. Although very rare , CNS lymphoma should be taken into account in every patient presentin g with the clinical features of acute polyneuropathy. (C) 1994 Wiley-L iss, Inc.