Acute hemorrhagic leukoencephalitis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Acute hemorrhagic leukoencephalitis (AHL, or AHLE), also known as acute necrotizing encephalopathy (ANE), acute hemorrhagic encephalomyelitis (AHEM), acute necrotizing hemorrhagic leukoencephalitis (ANHLE), Weston-Hurst syndrome, or Hurst's disease, is a hyperacute and frequently fatal form of ADEM. AHL is relatively rare (less than 100 cases have been reported in the medical literature as of 2006),[1] it is seen in about 2% of ADEM cases, and is characterized by necrotizing vasculitis of venules and hemorrhage, and edema.[2] Death is common in the first week[3] and overall mortality is about 70%,[1] but increasing evidence points to favorable outcomes after aggressive treatment with corticosteroids, immunoglobulins, cyclophosphamide, and plasma exchange. About 70% of survivors show residual neurological deficits,[2] but some survivors have shown surprisingly little deficit considering the magnitude of the white matter affected.[3] This disease has been occasionally associated with ulcerative colitis and Crohn's disease, septicemia associated with immune complex deposition, methanol poisoning, and other underlying conditions.

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References

  1. 1.0 1.1 Davies NW, Sharief MK, Howard RS (2006). "Infection-associated encephalopathies: their investigation, diagnosis, and treatment". J. Neurol. 253 (7): 833–45. doi:10.1007/s00415-006-0092-4. PMID 16715200. Unknown parameter |month= ignored (help)
  2. 2.0 2.1 Stone MJ, Hawkins CP (2007). "A medical overview of encephalitis". Neuropsychol Rehabil. 17 (4–5): 429–49. doi:10.1080/09602010601069430. PMID 17676529.
  3. 3.0 3.1 Archer H, Wall R (2003). "Acute haemorrhagic leukoencephalopathy: two case reports and review of the literature". J. Infect. 46 (2): 133–7. doi:10.1053/jinf.2002.1096. PMID 12634076. Unknown parameter |month= ignored (help)



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