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Disease found:Rasmussen encephalitis
Current as of:October 8, 2024
Disease Overview:chronic inflammatory neurological disease affecting one hemisphere of the brain, usually seen in children under 10 [more info]
Signs and Symptoms:severe unilateral focal epilepsy (refractory to medication), hemiparesis, hemianopia, progressive cognitive decline, developmental arrest/regression aphasia, encephalitis. [more info] Possible years-long prodromal phase of milder hemiparesis and seizures. [more info]
Diagnosis:No specific test: Progressive clinical decline, MRI brain showing unilateral atrophy. Series of MRIs over months-years show progressive atrophy. [more info]
Treatment:Seizure control: surgical hemidisconnection; seizures unlikely to respond to anti-epileptic therapy. Encephalitis: Recent case series suggest use for immunosuppressive therapy (corticosteroids, intravenous immunoglobulins, tacrolimus, azathioprine)
Clinical Management:Supportive treatment for progressive neurologic decline. Support group: Pediatric Epilepsy Surgery Alliance
Referral:Center for Pediatric Rasmussen Syndrome, Johns Hopkins University Health System, Baltimore MD, 410-955-9100. List of additional providers specializing in Rasmussen encephalitis. Referral to Medical Genetics Department, if available. Initial virtual care is also available through organizations like TeleRare Health.
Clinical Trials:There are two clinical trials currently recruiting; one in USA one in France.

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