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Disease Profile

Acute hemorrhagic leukoencephalitis

Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.


US Estimated

Europe Estimated

Age of onset





Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.


Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.


dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.


recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.


Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.


Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.


Not applicable


Other names (AKA)



Acute hemorrhagic leukoencephalitis (AHLE) is a very rare form of acute disseminated encephalomyelitis that frequently results in death. It is characterized by a brief but intense attack of inflammation in the brain and spinal cord that damages the myelin (the protective covering of the nerve fibers). It may also cause bleeding in the brain, leading to damage of the white matter. Symptoms usually come on quickly and may include fever, neck stiffness, fatigue, headache, nausea, vomiting, seizures, and coma. AHLE has a very poor prognosis, with rapid deterioration and death usually occurring within days to one week after onset of symptoms.[1][2][3][4] Although the exact cause is unclear, AHLE usually follows a viral or bacterial infection, or less often, vaccination for measles or rabies. Some researchers think that an infection or vaccination can initiate an autoimmune process in the body thus leading to AHLE.[1][2][3] While favorable outcomes are uncommon, timely treatment with immunosuppressant medications and plasma exchange may lead to an improved outlook.[3][4]

Learn more

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Acute hemorrhagic leukoencephalitis. Click on the link to view a sample search on this topic.


  1. Lann MA, Lovell MA, Kleinschmidt-DeMasters BK. Acute hemorrhagic leukoencephalitis: a critical entity for forensic pathologists to recognize. Am J Forensic Med Pathol. 2010; 31(1):7-11. https://www.ncbi.nlm.nih.gov/pubmed/20010289.
  2. Ryan LJ, Bowman R, Zantek ND, Sherr G, Maxwell R, Clark HB, Mair DC. Use of therapeutic plasma exchange in the management of acute hemorrhagic leukoencephalitis: a case report and review of the literature. Transfusion. 2007; 47(6):981-6. https://www.ncbi.nlm.nih.gov/pubmed/17524086.
  3. Duggal N, Ahmed I, Duggal N. Acute Hemorrhagic Leukoencephalitis Associated With Autoimmune Myopathy. J Vasc Interv Neurol. 2014 Nov; 7(4):19-22. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4241410/.
  4. Khademi GR, Aelami MH. Acute Hemorrhagic Leukoencephalitis in Children: A Case Report. Iran J Med Sci. 2016 May; 41(3):245-8. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4876304/.