Rasmussen’s Encephalitis Presenting as Refractory Nocturnal Focal Seizures with Unilateral Cerebral Hemiatrophy in a 14-Year-Old Girl: A Case Report

Abstract

Rasmussen’s encephalitis (RE) is a rare, chronic, progressive inflammatory neurological disorder that causes unilateral hemispheric inflammation, drug-resistant focal seizures, and progressive neurological deficits. It mainly affects children and often mimics other forms of refractory epilepsy, leading to diagnostic delays. Case Presentation: A 14-year-old girl presented with a one-year history of refractory nocturnal seizures and chronic headaches that began at age 13. Despite one year of Brivaracetam therapy, seizures remained uncontrolled. Examination revealed right-sided hemiparesis (power 4/5) and brisk reflexes. Laboratory tests showed elevated serum creatinine kinase (652.7 U/L). MRI brain demonstrated left cerebral hemisphere atrophy with ex-vacuo dilatation of the left lateral ventricle, reduced left cerebral peduncle, and frontoparietal white-matter changes. EEG revealed abnormal frontotemporal discharges. The diagnosis was revised to Rasmussen’s encephalitis. Addition of Clobazam to ongoing Brivaracetam therapy resulted in complete seizure freedom during admission. Headache and photophobia resolved by Day 4. Conclusion: This case highlights the importance of advanced neuroimaging and EEG in differentiating Rasmussen’s encephalitis from generalized epilepsy in children with drug-resistant seizures. Early diagnosis enables timely immunotherapy or surgery (e.g., hemispherotomy) to prevent irreversible brain damage and improve long-term functional and cognitive outcomes.