Leptomeningeal gliomatosis with spinal cord or cauda equina compression: a complication of supratentorial gliomas in adults

Seven patients with supratentorial gliomas developed leptomeningeal gliomatosis (LMG) without symptomatic recurrence at the primary tumor site. In all, severe back and radicular pain, often simulating disc disease, preceded the development of spinal cord or cauda equina dysfunction. In 4 instances, intracranial hypertension due to hydrocephalus developed prior to spinal involvement. Cytological examination of the CSF revealed malignant cells in only 2/7 but a myelogram was diagnostic in all 7. All patients received spinal irradiation (RT) and 5 received chemotherapy. Two patients with low-grade gliomas improved transiently; 5 with malignant gliomas responded poorly, became paraplegic over 4 months and eventually died of LMG. When fatal LMG occurs in young adults suffering from supratentorial glioma, the primary tumor is often quiescent. Hydrocephalus is often the first manifestation of LMG and, when it is detected, a myelogram and CSF cytology study should be performed in the hope that diagnosis and treatment of spinal cord lesion at a very early stage will prove beneficial. Irradiation of the entire spinal canal is probably required as there is a high risk of rapid development of new lesions in non irradiated segments of the spinal canal.