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Updated: May 11, 2026

Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
Published on: July 4, 2007
Rasmussen encephalitis
Tiziana Granata1, Frederick Andermann
1Department of Pediatric Neuroscience, Carlo Besta Neurological Institute, Milan, Italy.
Abstract:
Rasmussen encephalitis (RE) is a rare, inflammatory, and possibly immuno-mediated disease that typically affects one hemisphere. The two cardinal symptoms are progressive neurological deficits and intractable seizures, often in the form of epilepsia partialis continua and recurring epileptic status. Distinctive MRI features include progressive unilateral focal cortical atrophy and gray or white matter high-signal changes with basal ganglion involvement. Histopathology is characterized by brain inflammation dominated by T cells, microglial activation, and microglial nodules, followed by neuronal loss and astrogliosis. The diagnosis of RE, which may be particularly challenging in the initial stages, is based on clinical and laboratory findings. The diagnosis requires the exclusion of other causes of epilepsia partialis continua, and other cerebral focal inflammatory diseases. The treatment of RE is often demanding: antiepileptic drugs are of limited effect, whereas the surgical exclusion of the affected hemisphere offers a very high chance of seizure freedom but at the price of irreversible neurological deficits. By contrast, long-term immunotherapy may delay hemispheric tissue loss and neurological deficits, but has a lesser effect on total seizure burden. Given that the severity of symptoms varies among different patients and phases, the therapeutic strategy, including medical and surgical options, must be tailored to the need of each patient.
Insights
Rasmussen encephalitis (RE) is a rare inflammatory brain disease causing progressive neurological decline and severe seizures. Treatment requires personalized strategies, balancing surgery for seizure control with immunotherapy to slow disease progression.
Area of Science:
- Neurology
- Immunology
- Neuroinflammation
Background:
- Rasmussen encephalitis (RE) is a rare, unilateral, inflammatory brain disorder.
- Characterized by progressive neurological deficits and intractable seizures, often epilepsia partialis continua.
- Distinctive MRI findings include unilateral atrophy and focal cortical/subcortical signal changes.
Purpose of the Study:
- To summarize the key features, diagnostic challenges, and therapeutic approaches for Rasmussen encephalitis.
- To highlight the need for tailored treatment strategies based on individual patient needs.
Main Methods:
- Review of clinical, neuroimaging (MRI), and histopathological findings in Rasmussen encephalitis.
- Analysis of treatment outcomes for antiepileptic drugs, surgery, and immunotherapy.
- Emphasis on differential diagnosis to exclude other causes of focal encephalitis and seizures.
Main Results:
- Histopathology reveals T-cell dominated inflammation, microglial activation, neuronal loss, and astrogliosis.
- Surgical hemispherectomy offers high seizure freedom but causes permanent deficits.
- Immunotherapy can slow disease progression but has limited impact on seizure burden.
Conclusions:
- Diagnosis of RE is challenging, especially early on, requiring exclusion of other conditions.
- Treatment must be individualized, considering the trade-offs between seizure control and neurological deficits.
- A multidisciplinary approach is crucial for managing this complex neuroinflammatory disease.
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