acute hemorrhagic leukoencephalitis
Also written as: AHLE — acute hemorrhagic leukoencephalitis
A fulminant demyelinating disease characterized by widespread white matter necrosis and hemorrhage.
Full Definition
Acute hemorrhagic leukoencephalitis (AHLE), also known as Hurst disease, is a rare, fulminant variant of acute disseminated encephalomyelitis characterized by widespread white matter necrosis, hemorrhage, and neutrophilic infiltration. The condition typically follows a viral infection or vaccination and represents an hyperacute autoimmune response. Histologically, AHLE shows necrotizing inflammation with fibrin ring hemorrhages, neutrophilic vasculitis, and extensive white matter destruction. The gray matter is relatively spared. The disease has a rapid, often fatal course over days to weeks, distinguishing it from other demyelinating conditions. Neuroimaging shows extensive white matter abnormalities with hemorrhagic components.
Usage
Usage note: Note fulminant course and fibrin ring hemorrhages as key features.
In Context
- "The autopsy revealed acute hemorrhagic leukoencephalitis with extensive white matter necrosis." — Forensic neuropathology report
- "Fibrin ring hemorrhages were characteristic of acute hemorrhagic leukoencephalitis." — Emergency brain biopsy report