AGGRESSIVE TREATMENT WITH COMPLETE REMISSION IN PRIMARY DIFFUSE LEPTOMENINGEAL GLIOMATOSIS - A CASE-REPORT

Primary leptomeningeal gliomatosis is rare, and the diffuse form (PLDG ) is even more unusual. The following report is an example. A 17 year- old man developed a syndrome characterized by extensive basal and chro nic spinal meningitis. Routine biological tests showed elevated levels of CSF proteins, and moderate mononuclear pleocytosis, with no direct evidence of neoplasia, leading to a diagnosis of chronic meningitis. A second meningeal biopsy, guided by MRI and performed in the left fro ntal region, led to the specific diagnosis of primary diffuse leptomen ingeal gliomatosis. Treatment including ventricular and lumbar shuntin g, a course of cortico-spinal radiation, and three courses of an eight -drug systemic chemotherapy with intrathecal methotrexate lead to comp lete remission over 15 months. We believe that this is the first repor t of such a remission in the literature.