Acute Hemorrhagic Leukoencephalitis (AHLE)
Acute hemorrhagic leukoencephalitis (AHLE) is a rare, hyperacute form of inflammatory demyelination. Protheragen is committed to providing cutting-edge diagnostic and therapeutic development solutions to address the challenges of AHLE management. As your reliable partner in AHLE therapeutic research, we provide comprehensive and high-quality services to meet all your scientific research needs.
Overview of Acute Hemorrhagic Leukoencephalitis (AHLE)
Acute hemorrhagic leukoencephalitis (AHLE) is a rare, fulminant neuroinflammatory disorder characterized by rapid-onset demyelination, vasculitis, and hemorrhagic necrosis of the white matter. Often considered a hyperacute variant of acute disseminated encephalomyelitis (ADEM), AHLE presents with severe neurological deterioration, including encephalopathy, seizures, and focal deficits, frequently progressing to coma or death within days. Mortality rates exceed 50%, with survivors often experiencing long-term cognitive and motor impairments.
Fig.1 Symptoms and causes of acute hemorrhagic leukoencephalitis (AHLE). (Alromaihi M., 2022)
Pathogenesis of Acute Hemorrhagic Leukoencephalitis (AHLE)
The pathogenesis of acute hemorrhagic leukoencephalitis (AHLE) is primarily driven by a hyperactive immune response targeting the brain's white matter, often triggered by infections (e.g., viral or bacterial) or post-vaccination reactions. Key mechanisms include dysregulated T-cell activation, autoantibody-mediated damage, and cytokine storm, leading to blood-brain barrier disruption, fibrinoid necrosis of small vessels, and hemorrhagic necrosis. Complement activation and matrix metalloproteinase (MMP-9)-induced vascular injury further exacerbate inflammation and microbleeds, resulting in rapid neurological deterioration.
Therapeutic Development for Acute Hemorrhagic Leukoencephalitis (AHLE)
| Drug Names | Mechanism of Action | Targets | Research Phase |
| Methylprednisolone | Potent immunosuppressant that broadly inhibits inflammatory cytokines and immune cell activation via glucocorticoid receptor signaling. | Glucocorticoid receptor | Approved |
| Rituximab | Monoclonal antibody that selectively depletes CD20+ B-cells to reduce autoantibody production and downstream inflammation. | CD20 protein on B-cells | Early research |
Disclaimer: Protheragen focuses on providing preclinical research services. This table is for information exchange purposes only. This table is not a treatment plan recommendation. For guidance on treatment options, please visit a regular hospital.
Our Services
As a leader in rare neuroinflammatory diseases research, Protheragen offers comprehensive preclinical services to advance acute hemorrhagic leukoencephalitis (AHLE) therapeutics from discovery to validation. Our specialized platforms integrate genetic analysis and disease-specific modeling to replicate disease pathology with high fidelity. Using advanced blood-brain barrier (BBB) models, we optimize central nervous system (CNS) drug delivery while evaluating neuroprotection and off-target effects.
Therapeutic Development Services
By Mechanism of Action
Disease Model Development Services
In Vitro Model Development
Theilerian Murine Encephalitis Virus (TMEV) Infection Model: This model combines intracranial TMEV infection in C57BL/6 mice with intravenous VP2121-130 CD8+ T-cell peptide administration, recapitulating key human AHLE features including microhemorrhages, BBB disruption, and demyelination.
At Protheragen, we offer comprehensive pharmacodynamic (PD), pharmacokinetic (PK), and toxicology research services to support the development and regulatory approval of potential therapies for acute hemorrhagic leukoencephalitis (AHLE). If you are interested in our services, please feel free to contact usfor more details and quotation information of related services.
Reference
- Alromaihi M. Acute Hemorrhagic Leukoencephalitis (AHLE): A Comprehensive Review on Causes, Symptoms, Link with COVID‐19, Diagnosis, and Treatment[J]. BioMed Research International, 2022(1): 6008375.