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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. |