Diagnosis and treatment of Rasmussen's encephalitis pose a big challenge: Two case reports and literature review.

ABSTRACT: Rasmussen encephalitis (RE) is a rare disease of unknown etiology that causes severe chronic unihemispheric inflammatory disease of the central nervous system mainly in children. It leads to intractable seizures, cognitive decline and progressive neurological deficits in the affected hemisphere. We report two cases of RE, as defined by fulfillment of the 2005 Bien criteria. The diagnostic challenge of characterizing this rare disease will be highlighted by the extensive serum, CSF, MR imaging and EEG data in the two patients. In addition, we will review the various forms of therapy attempted in these two patients, namely anti-epileptic drug therapy and immunomodulatory therapy. Hemispherectomy was done for the second patient with favorable outcomes of controlling seizures, but unfortunatel

SUBMITTER: Hammed A 

PROVIDER: S-EPMC8358639 | biostudies-literature | 2021 Aug

REPOSITORIES: biostudies-literature