Publication:

A rare and challenging case of Rasmussen’s encephalitis: a pediatric case report

Rasmussen’s encephalitis (RE) is a rare, chronic, and progressive neurological disorder characterized by unilateral hemispheric inflammation, intractable seizures, and neurological decline, primarily affecting children under 10 years old. This case report describes a 9-year-old male child born to consanguineous parents presented with a 9-month history of progressive myoclonic jerks, generalized tonic-clonic seizures (GTCS), and right-sided hemiparesis. Despite undergoing left temporo-parieto-occipital craniotomy and aggressive pharmacological management with antiepileptics, immunosuppressants, and corticosteroids, the patient developed super-refractory status epilepticus. Diagnostic imaging revealed left hemispheric atrophy, and cerebrospinal fluid analysis ruled out infections, leading to a diagnosis of Rasmussen’sencephalitis with drug resistant epilepsy. The patient underwent left vertical thalamic functional hemispherotomy, resulting in reduced seizure frequency, improved motor function, and decreased inflammatory markers postoperatively. This case underscores the challenges in managing RE, emphasizing the importance of early diagnosis, a multidisciplinary approach, and timely surgical intervention and challenging medication management.