Rasmussen encephalitis (RE) is a chronic progressive inflammation of one hemisphere of the brain. GLUR3 auto-antibodies and activated T-cells found in parenchyma reflect the autoimmunity1. Severe progressive contralateral functional deficits occur. Drug-resistant focal seizures commonly prompt resective surgery. Onset is typically is in childhood (< 10 years of age). Seizures are prolonged and if motor cortex is affected epilepsia partialis continua often results. Hemispherectomy is a standard of care for advanced RE. Long term neurological sequella may be minimized by modified hemispherectomy and hemispherotomy. Minimal cognitive dysfunction or no change is usually evident1. In 33 children and adolescents, hemiplegia was unchanged in 22/33, improved in 5, and worsened in 6 patients2. RE like other progressive and acquired pathology has the best outcome with surgery while developmental lesions have less favorable results. Optimal timing and relationship to pathology is individualized. Our patient had a left hemiparesis that worsened after surgery. His seizures remained lateralized to the left body despite surgical absence of the right hemisphere. Possibilities include remaining tissue with residual connections and transferred function to the intact hemisphere (neuroplasticity) associated with operation at an early age. Immunotherapy with steroids and IVIG is advancing as a primary adjunctive treatment1.
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