Case of the Month
Section Editor: Nicholas Stence, MD
Children's Hospital Colorado, Aurora, CO
February 2015
Next Case of the Month coming March 3 . . .
Hurst Disease: Acute Hemorrhagic Leukoencephalitis
- Background: Hurst disease, or acute hemorrhagic leukoencephalitis, is a rare form of acute demyelinating encephalomyelitis that is characterized by rapid deterioration with perivascular hemorrhage and edema.
- Relevant Clinical Information: Some reported cases are fulminant and fatal, while some improve with corticosteroids and plasma exchange. They are often preceded by upper respiratory or viral infections. CSF usually reveals a polymorphonuclear pleocytosis.
- Key Diagnostic Features:
- Non-specific imaging features
- Heterogeneous-appearing lesion with minimal edema and mass effect for the size of the lesion
- Patchy enhancement may be seen but no mass-like enhancement. Essentially, a diagnosis of exclusion.
- Correlation with history — prior viral illness, multifocal complaints, altered sensorium — can be helpful.
- DDx:
- Acute disseminated encephalomyelitis (ADEM)
- Infarction secondary to venous sinus thrombosis
- Herpes simplex encephalitis
- Rx:Treatment options include IVIG, plasmapheresis, steroids, and hyperosmolar therapy