Rasmussen encephalitis is a rare, progressive, and severe inflammatory disease of the brain that typically affects one cerebral hemisphere, predominantly in children. Microglia play a central role in the immunopathogenesis of this condition, mediating both the inflammatory response and the cytotoxic mechanisms that lead to progressive neuronal loss and cortical atrophy[1].
Microglia are the resident immune cells of the central nervous system and serve as the primary mediators of neuroinflammation in Rasmussen encephalitis. Their activation and interactions with T-cells drive the characteristic progressive neurological decline seen in this condition[2].
| Property | Value |
|---|---|
| Category | Immune-Mediated |
| Location | Unilateral cortex |
| Cell Type | Activated microglia |
| Pathology | CD8+ T-cell mediated |
| Taxonomy | ID | Name / Label |
|---|---|---|
| Cell Ontology (CL) | CL:0000129 | microglial cell |
| Database | ID | Name | Confidence |
|---|---|---|---|
| Cell Ontology | CL:0000129 | microglial cell | Medium |
| Cell Ontology | CL:4042028 | immature neuron | Medium |
Rasmussen encephalitis is an immune-mediated disease characterized by a focal inflammatory process that progressively destroys one cerebral hemisphere. The pathogenesis involves a complex interplay between microglia, CD8+ cytotoxic T-cells, and neurons[3].
CD8+ T-cell mediated cytotoxicity:
Microglial activation:
| Feature | Description |
|---|---|
| Focal seizures | Epilepsia partialis continua (continuous focal motor seizures) |
| Progressive hemiparesis | Unilateral weakness progressing over months to years |
| Cognitive decline | Perisylvian involvement leads to intellectual disability |
| Cortical atrophy | Progressive hemiatrophy visible on MRI |
Varadkar S, et al. Rasmussen encephalitis. The Lancet Neurology. 2014. ↩︎
Bien CG, et al. Pathogenesis, diagnosis and treatment of Rasmussen encephalitis. Expert Opinion on Therapeutic Targets. 2002. ↩︎
Granata T, et al. Rasmussen's encephalitis. Brain and Development. 2003. ↩︎
Gupta PC, et al. Microglial activation in Rasmussen encephalitis. Indian Journal of Pediatrics. 2004. ↩︎
Chen W, et al. Longitudinal PET imaging in Rasmussen encephalitis. Journal of Nuclear Medicine. 2012. ↩︎
Loring M, et al. Surgical outcome and biomarkers in Rasmussen encephalitis. Epilepsia. 2020. ↩︎