Primary diffuse leptomeningeal gliomatosis predominantly affecting the spinal cord: case report and review of the literature

Primary leptomeningeal gliomatosis is a rare, fatal neoplastic syndrome. A 71 year old man is reported on, who after a 2 month history of back stiffne ss, epigastric pain, and weight loss developed visual blurring. Cranial CT and MRI studies showed no leptomeningeal enhancement. Examination of CSF 10 weeks premortem showed an increase in protein and decrease in glucose but no malignant cells. He became increasingly confused and repeated CSF examin ation showed inflammation and a few suspicious cells but no definitive evid ence of neoplasia. He died 7 months after onset of his initial symptoms. At postmortem meningeal whitening was seen at the base of the brain and over the spinal cord. Histology disclosed diffuse leptomeningeal gliomatosis (GF AP positive, cytokeratin negative) over the brain, optic nerves, and spinal cord without parenchymal involvement. No tumour was found in internal orga ns. The diagnosis of primary leptomeningeal gliomatosis was not evident aft er cranial CT and MRI and CSF examination premortem. Suspected cases need M RI scanning of the entire neuraxis and meningeal biopsy.