Abstract
Dysembryoplastic neuroepithelial tumors(DNT) are low grade glial neuronal tumors typically occuring in the cortex and are removed as a part of epilepsy surgery. Many times when found on imaging and asymptomatic, observation is recommended. Spinal cord and leptomeningeal involvement has rarely been recorded. A 35 yo man presented with hydrocephalus in the setting of a newly diagnosed spinal cord mass. 8 months prior, he had been treated for viral meningoencephalitis due to West Nile virus. MRI of the brain revealed a multifocal cystic lesion in the cerebellum, consistent with a low grade glioneuronal tumor. Spinal MRI revealed a T2 cord lesion with meningeal enhancement, and a nodular lumbar spine lesion with meningeal enhancement. Lumbar spine biospy revealed chronic inflammation and myxoid-like foci. Since no evidence of neoplasm was found, effect of West Nile meningoencephalitis was assumed, as he still had IgM antibodies present in CSF. There was some improvement in the spinal cord imaging, with decreased size of the T2 mass, with chronic steroids, but continued lumbar enhancement. Then the patient developed progressive leptomeningeal enhancement, encasing the brain and spine, growth of the spinal cord mass and new enhancement in the cerebellar lesion over the course of the year, becoming quadriparetic. Biopsy of the cerebellar lesion and meninges again only showed a thickened dura with chronic inflammation and normal cerebellar tissue. Patient elected hospice care. Autopsy revealed a disseminated DNT. A disseminated DNT has rarely been reported. The development of this aggressive course following West Nile virus infection was noted. It has been established that West Nile virus causes S100B pathway activation in astrocytes, which is one of the oncogenic pathways that is activated in dysembryoplastic neuroepithelial tumors. We propose this additive effect may have led to an unusually aggressive behavior in a low grade tumor. Additional studies are needed.
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