Absence of epileptic seizures onset in Rasmussen encephalitis: a case report

Abstract

　　Rasmussen encephalitis (RE), a childhood-onset autoimmune disease that causes chronic hemispheric encephalitis, typically displays unilateral focal seizures as the initial clinical feature, followed by subsequent progressive contralateral cerebral hemiatrophy and spastic hemiparesis within a few years. We report a 4-year-6-month-old female with atypical RE onset without initial epileptic seizures. The earliest clinical manifestation of RE in this patient was spastic hemiparesis, which was followed by subsequent progressive cerebral hemiatrophy, thus fulfilling two out of three criteria for diagnosis of RE. In addition, the patient's cerebrospinal fluid showed strongly positive signal for an anti-GluR antibody and significantly elevated granzyme B levels, supporting the diagnosis of RE. Methylprednisolone pulse therapy and intravenous immunoglobulin had beneficial effects on the patient's neurological symptoms. Similar cases were reported in the European consensus statement as RE with delayed seizure onset. Consistent with previous reports, the clinical course of our patient also suggests that an autoimmune reaction occurs at the beginning of RE, which subsequently causes chronic encephalopathies. Thus, the existence of RE cases without epileptic seizures during the early course of the disease should be recognized.