Spotlight On

Rasmussen Encephalitis

Rasmussen encephalitis is a chronic inflammatory neurological disease that usually affects only one hemisphere of the brain. It most often occurs in children under the age of 10, although adolescents and adults may also be affected

Prevalence

unknown

N/A

US Estimated

N/A

Europe Estimated

Age of Onset

childhood

ICD-10

G04.81

Inheritance Pattern

Believed to be an autoimmune disorder targeting one hemisphere of the brain

5 Facts you should know

FACT

Rasmussen encephalitis pathogenesis is thought to involve cytotoxic T-cell–mediated neuronal injury, although viral and autoimmune mechanisms have also been proposed

FACT

The disease typically presents in childhood with focal seizures, often epilepsia partialis continua (EPC), that are resistant to antiseizure medications and worsen over time

FACT

Progressive neurological deterioration includes hemiparesis, cognitive impairment, and language deficits, reflecting the unilateral cortical inflammation and atrophy

FACT

MRI findings often reveal progressive hemispheric atrophy with cortical T2 hyperintensities, which, along with EEG and clinical features, support the diagnosis

FACT

Treatment is challenging: immunotherapy (e.g., corticosteroids, IVIG, plasma exchange, rituximab, or tacrolimus) may slow progression, but the only definitive intervention is hemispherectomy, which can effectively control seizures at the cost of permanent hemiplegia

Interest over time

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