Table of Contents
Primary Category
Neuroimmunology
P-Category
Secondary Category
S-Category
Authors:
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
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 open, 8, 2329048X211016109. https://doi.org/10.1177/2329048X211016109
- Wang C. X. (2021). Assessment and Management of Acute Disseminated Encephalomyelitis (ADEM) in the Pediatric Patient. Paediatric drugs, 23(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 neurology, 11, 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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