Intravascular lymphomatosis mimicks a cerebral demyelinating disorder

Intravascular lymphomatosis is a rare extra-nodal form of diffuse, large B-cell non-Hodgkin's lymphoma that causes a myriad of neurological symptoms, including focal neurological deficits, cognitive decline, and seizure. Intravascular lymphomatosis is difficult to make ante-mortem diagnosis because of its variable clinical presentation and non-specific laboratory findings. Neuroimaging findings also vary widely in patients of intravascular lymphomatosis. We present, herein, a patient with rapidly deteriorating neurological deficit whose brain MRI revealed rapidly progressing white matter diseases. This patient was diagnosed and treated as having a cerebral demyelinating disorder. Intravascular lymphomatosis involving multiple organs was finally diagnosed after autopsy.