Diffuse leptomeningeal gliomatosis associated with multifocal CNS infarcts

Abstract

Widespread dissemination of astrocytoma throughout the cerebrospinal fluid is unusual, especially as the initial presentation in a patient, and association with cerebral infarcts is rare. CASE DESCRIPTION A 47-year-old man subacutely developed progressive headache and backache, vomiting, altered consciousness, and numbness in both arms. Brain computerized axial tomography showed calcification in the fourth ventricle and magnetic resonance imaging demonstrated diffuse meningeal enhancement, a lesion in the canal of Magendie, and T2-weighted hyperintense lesions in several locations. Angiography showed vasospasm/vasculopathy involving multiple cerebral vessels. Biopsy of the fourth ventricular mass revealed a subependymoma with an overlying leptomeningeal anaplastic astrocytoma with numerous Rosenthal fibers. The patient was treated with three doses of intrathecal methotrexate via lumbar puncture but suddenly became unresponsive and died 6 days later, 8 weeks after initial symptoms. Autopsy demonstrated the subependymoma and a widespread leptomeningeal anaplastic astrocytoma, which showed no clear-cut origin from the subependymoma, but microscopically infiltrated the left medial temporal lobe. Multiple subacute and acute infarcts of the brain, brain stem, and upper spinal cord were seen only in areas with leptomeningeal tumor. CONCLUSION Glioma cells surrounding and focally permeating central nervous system vessels without lumenal occlusion can lead to vasospasm and widespread infarcts.