Acute Hemorrhagic Leukoencephalitis

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Introduction

  • Inflammatory disease of the brain
  • First described by Weston Hurst in 1941
  • 50-75% of patients have a history of viral or bacterial infections.
  • Most of the patients are children or young adults
  • The most commonly affected site is the cerebrum followed by the cerebellum, brain stem, or spinal cord.
Acute hemorrhagic leukoencephalitis (AHL) is also known as Weston-Hurst syndrome.
A very rare form of demyelinating disease
Occurs sporadically. More common in children and young adults
Considered most severe form of acute disseminated encephalomyelitis (ADEM)

Pathophysiology

  • Acute and rapidly progressive hemorrhagic demyelination of white matter
  • Acute immunological response induced by cross-reaction between human myelin antigens and viral or bacterial antigens, causing demyelination.
  • Injecting the VP2121–130 viral capsid of the Theiler’s murine encephalomyelitis virus in the first murine model of AHLE -induced a strong in vivo activation of CD8+ T cells in C57BL/6 mice leading to the development of hemorrhagic demyelination within 24 hrs.

Clinical Features

  • Headache
  • Seizure
  • Focal neurologic signs
  • Encephalopathy
  • Coma

Differential Diagnosis

  • Optic Neuritis
  • Multiple Sclerosis
  • Transverse Myelitis
  • Neuromyelitis Optica
  • Collagen vascular disease
  • Primary angiitis of the central nervous system
  • Myelin Oligodendrocyte Glycoprotein antibody syndrome

Diagnosis

💡
MRI is the diagnostic test of choice
  • MRI is the diagnostic test of choice - diffuse edema, larger and often confluent lesions, petechial hemorrhage
  • Perivascular demyelination and hemorrhage, inflammatory infiltrate with predominant neutrophil and macrophage, fibrinoid necrosis of vessel wall are the pathologic hallmarks.
  • CSF study shows mild polymorphonuclear leukocytosis or elevated protein or presence of oligoclonal bands
  • In some cases, CSF study showed the presence of anti-myelin oligodendrocyte glycoprotein (anti-MOG) antibodies and anti-aquaporin-4 (anti-AQP4) antibodies
  • Serial MR Imaging Findings of Acute Hemorrhagic Leukoencephalitis can be found here.
 
Figure 1: MRI Finding of Acute Hemorrhagic Leukoencephalitis
notion image
Cerebral MRI presenting the temporal evolution within 3 days (from left to right). First row: axial T2-weighted FLAIR images showing increasing bilateral confluent widespread hyperintensities of the supratentorial white matter predominantly on the left. Second row: axial T2-weighted FLAIR images revealing new hyperintensities of the left cerebellar peduncle. Third row: axial SWI demonstrating subtle and small susceptibility artifacts in the splenium of the corpus callosum. Fourth row: axial pre- and post-contrast T1-weighted MPRAGE showing enhancement of the left parieto-occipital region. FLAIR, Fluid-Attenuated Inversion Recovery; SWI, Susceptibility Weighted Imaging; MPRAGE, Magnetization-Prepared Rapid Acquisition with Gradient Echo
Source: Acute Hemorrhagic Leukoencephalitis: A Case and Systematic Review of the Literature Grzonka P, Scholz MC, De Marchis GM, et al. Acute Hemorrhagic Leukoencephalitis: A Case and Systematic Review of the Literature. Frontiers in Neurology. 2020;11. doi:10.3389/fneur.2020.00899

Treatment

  • No definitive treatment
  • Early treatment is very important
  • Individual or combination immunosuppressive therapy corticosteroids
    • methylprednisolone 2 gram per day for 3 days followed by tapering
    • cyclophosphamide (15 milligrams per kilogram body weight)
    • plasma exchange
  • Control of intracranial pressure with hyper-osmolar therapy (mannitol or hypertonic saline), hyperventilation, and/or therapeutic coma (phenobarbitol)

Prognosis

  • Prognosis is poor
  • It is Fatal disease
  • Death in a few days

Further Reading

  • Wellnitz, K., Sato, Y., & Bonthius, D. J. (2021). Fatal Acute Hemorrhagic Leukoencephalitis Following Immunization Against Human Papillomavirus in a 14-Year-Old Boy. Child neurology open8, 2329048X211016109. https://doi.org/10.1177/2329048X211016109
  • Wang C. X. (2021). Assessment and Management of Acute Disseminated Encephalomyelitis (ADEM) in the Pediatric Patient. Paediatric drugs23(3), 213–221. https://doi.org/10.1007/s40272-021-00441-7

Bibliography

  • Grzonka, P., Scholz, M. C., De Marchis, G. M., Tisljar, K., Rüegg, S., Marsch, S., Fladt, J., & Sutter, R. (2020). Acute Hemorrhagic Leukoencephalitis: A Case and Systematic Review of the Literature. Frontiers in neurology11, 899. https://doi.org/10.3389/fneur.2020.00899
  • Yildiz, Ö., Pul, R., Raab, P. et al. Acute hemorrhagic leukoencephalitis (Weston-Hurst syndrome) in a patient with relapse-remitting multiple sclerosis. J Neuroinflammation 12, 175 (2015). https://doi.org/10.1186/s12974-015-0398-1
  • Fugate JE, Lam EM, Rabinstein AA, Wijdicks EFM. Acute Hemorrhagic Leukoencephalitis and Hypoxic Brain Injury Associated With H1N1 Influenza. Arch Neurol. 2010;67(6):756–758. doi:10.1001/archneurol.2010.122
  • Manzano GS, McEntire CRS, Martinez-Lage M, Mateen FJ, Hutto SK. Acute Disseminated Encephalomyelitis and Acute Hemorrhagic Leukoencephalitis Following COVID-19: Systematic Review and Meta-synthesis. Neurol Neuroimmunol Neuroinflamm. 2021 Aug 27;8(6):e1080. doi: 10.1212/NXI.0000000000001080. PMID: 34452974; PMCID: PMC8404207.
  • Rosman NP, Gottlieb SM, Bernstein CA. Acute hemorrhagic leukoencephalitis: recovery and reversal of magnetic resonance imaging findings in a child. J Child Neurol. 1997 Oct;12(7):448-54. doi: 10.1177/088307389701200707. PMID: 9373802.
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