Primary leptomeningeal gliomatosis: case report and review of the literature.

A 62-year-old woman developed dysphasia, signs of intracranial hypertension and seizures. An MRI scan evidenced extra-axial contrast enhancement in the absence of mass lesions. CSF analysis was negative for malignant cytology and viral or bacterial microbiology. In the absence of other evidence, considering the acute clinical onset and MRI picture, a viral encephalitis was suspected and antiviral therapy was started, however, with no effect. Death occurred three weeks after presentation because of acute brain swelling. Autopsy revealed leptomeningeal gliomatosis and diffuse parenchymal infiltration at the cerebellar and left temporal lobe. Primary leptomeningeal gliomatosis (PLMG) is a rare event. The reported case and the reviewed literature evidence that clinical signs at presentation are non-specific, CSF negative findings are common, the diagnosis is always delayed and the prognosis dismal.