Acute Hemorrhagic Leukoencephalitis - A Rare but Fatal Form of Acute Disseminated Encephalomyelitis - Complicated by Brain Herniation: A Case Report and Literature Review

Am J Case Rep. 2022 Feb 27:23:e935636. doi: 10.12659/AJCR.935636.


BACKGROUND Acute hemorrhagic leukoencephalitis (AHLE) is a very rare fulminant post-infectious demyelinating disease of the CNS. We report an atypical presentation of AHLE involving unique brain areas 2 weeks following a viral upper-respiratory tract infection (URTI). Early diagnosis and proper management improve the prognosis of this disease, and AHLE can have a very poor prognosis and high mortality rate. CASE REPORT A 52-year-old male patient was referred for deteriorating consciousness 2 weeks after a viral URTI. An initial brain CT scan showed multiple patchy bilateral and diffuse hypodense areas including the cerebellar, occipital, parietal, and frontal lobes. The diagnostic workup also included CSF analysis and MRI of the brain, which revealed multiple areas of hemorrhagic involvement. Management included broad-spectrum antibiotics, acyclovir, mannitol, steroids, and plasmapheresis. On the fifth day of admission, brain CT showed severe diffuse edema and brain herniation. Unfortunately, despite prompt aggressive treatment measures, within 48 hours the patient died due to centrally-mediated hemodynamic instability. CONCLUSIONS We report a rare case of AHLE with a unique presentation and extensive unusual involvement of regions of periventricular and subcortical white matter, cerebellum, and midbrain. Early diagnosis along with appropriate management measures and intensive care can help decrease morbidity and mortality; therefore, prompt referral and high-level care should be sought for all patients who present with acute deteriorating consciousness. We hope that this report can help future studies to better characterize this rare disease and provide further guidance regarding prognosis and management.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Brain / diagnostic imaging
  • Encephalomyelitis, Acute Disseminated* / therapy
  • Humans
  • Leukoencephalitis, Acute Hemorrhagic* / diagnosis
  • Leukoencephalitis, Acute Hemorrhagic* / etiology
  • Leukoencephalitis, Acute Hemorrhagic* / therapy
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Neuroimaging