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| Disease found: | Rasmussen encephalitis |
| Current as of: | Mon Sep 16 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. TeleRare Health for virtual care and consults is also recommended. |
| Clinical Trials: | There are two clinical trials currently recruiting; one in USA one in France. |