Patients are most often male, usually children or young adults. Clinical presentation is characterized by the abrupt onset of fever and headache, followed by a rapid progression to stupor or coma. The CSF pressure is typically elevated and analysis reveals numerous neutrophils. Patients usually die within one to six days.
Gross examination reveals a diffusely swollen and soft brain.
Indeed, numerous attempts to identify an infectious agent in cases of acute hemorrhagic leukoencephalopathy have met with failure. Thus, mainstays of treatment have included supportive care and immunosuppression with corticosteroids. Plasmapheresis in fulminant acute disseminated encephalomyelitis. Contributed by Andrew P. Lieberman, Robert I. Grossman, and Ehud Lavi.