Rasmussen encephalitis: Response to early immunotherapy in a case of immune-mediated encephalitis

Background. Rasmussen encephalitis (RE) is a severe immune-mediated neurologic disease characterized by unihemispheric inflammation, progressive neurologic deficits, and intractable focal epilepsy. Functional hemispherectomy is considered to be the only effective intervention for this devastating disease. Reports on immunotherapy use in RE, including steroids, IV immunoglobulin, and tacrolimus, have been published. These therapies may lead to “arrest” of the disease in patients with pharmacoresistant epilepsy in whom motor function is not impaired enough to be offered a functional hemispherectomy. Information regarding the effect of early and aggressive immunotherapy on cognitive function, MRI disease activity, and seizure control in this population is limited. Here we present a case of a child satisfying 2005 Bien diagnostic criteria for RE in whom disease control and motor and cognitive preservation was achieved using early aggressive immunotherapy.