Clinicopathological considerations in acute disseminated encephalomyelitis (ADEM): a fulminant case with favorable outcome

Abstract

We report the clinical course and pathological features of a 52-year-old woman with fulminant acute disseminated encephalomyelitis (ADEM). She presented with subacute tetraparesis, which developed over 2 days, and progressive loss of consciousness. CCT showed pronounced bihemispherical and periventricular edema. Multifocal glioma was suspected, and the patient was referred for brain biopsy to the neurosurgery department at our hospital. On admission, the patient presented with progressive tetraparesis and aphasia. Subsequently, she developed epileptic seizures. Cranial magnetic resonance imaging (MRI) revealed confluent bihemispherical and periventricular white matter edema (Fig. 1a) with abnormal patchy gadolinium enhancement, compatible with progressive multifocal leukoencephalopathy (PML), multifocal brain tumor or ADEM. Cerebrospinal fluid (CSF) analysis revealed mild pleocytosis (11 cells/ll) with increased protein levels, and normal glucose and lactate levels. No intrathecal IgG and IgM synthesis or oligoclonal bands (OCB) were observed in CSF. Antiviral and antibiotic treatment was initiated. Stereotactic brain biopsy from right frontal periventricular white matter (Fig. 1b) was performed 5 days after disease onset. Histopathology ruled out malignant brain tumor but showed acute perivascular inflammation.