Different modalities of the treatment of Rasmussen encephalitis: A systematic review of case reports of a rare disease

Abstract

Background: Rasmussen’s encephalitis (RE) is a rare chronic neurological disorder that presents with progressive neurological and cognitive deterioration, and intractable seizures that lacks definitive management. We aimed to identify case reports of RE in the literature to assess the treatment based on the symptoms and outcomes after the treatment. Methods: We searched the databases PubMed, Google Scholar, and the Cochrane Library from 1990 to 22nd February 2022 in order to review the case reports that included the treatment options and clinical outcomes of RE. A pre-established protocol was registered on the International Prospective Register of Systematic Reviews (PROSPERO) database (ID: CRD42021261999). Results: The mean age of onset of seizure was 31.44 and 6.87 years in adult onset RE and children (below 15 years of age) with RE respectively. A total of four (44.44%) out of nine adult patients and 81 (66.39%) out of 122 children underwent surgery. Tacrolimus was used in 17 (13.93%) and steroid therapy was used in 11 (9.01%) of the children. Conclusions: Large scale-controlled trials are needed to study the beneficial effect of tacrolimus, IV Ig, and plasma therapy. Hemispherectomy should be opted as a treatment modality by assessing the impairment of patients’ motor or language functions following the treatment procedure.