Abstracts

Rationale: To describe the diagnosis and management of a 12 year old female who presented with new onset seizures and a brain MRI demonstrating atrophy of the left cerebral and right cerebellar hemispheres. Initially, eletrophysiologic studies suggested a left posterior frontal-parietal focus. Approximately six months into her course, the seizure semiology changed to involve frequent left face, arm and leg clonic movements and epilepsia partialis continua (EPC) of the left upper extremity. Additionally, the EEG and PET imaging studies demonstrated a right parasagittal seizure focus. Methods: Chart review of the clinical course, electrophysiologic, imaging, laboratory evaluations and treatment of a patient presenting to Cincinnati Children's Hospital Medical Center with intractable epilepsy, subsequently diagnosed with bilateral Rasmussen's encephalitis. Results: An extensive evaluation for paraneoplastic, mitochondrial and genetic epilepsy syndromes was unrevealing. The cerebrospinal fluid studies showed normal glucose and cell counts but were positive for seven oligoclonal bands not found in her serum. The initial brain MRI showed left cerebral and right cerebellar atrophy with T2 hyperintensity in the left parietal region. Over the course of six months, an additional T2 hyperintensity was noted in the right frontal lobe. On biopsy evaluation seven months after her initial seizure, bilateral chronic encephalitis was demonstrated. This patient's clinical EPC with the biopsy confirmation of a T-cell encephalitis with activated microglia and reactive astrogliosis, supports the diagnosis of bilateral Rasmussen encephalitis. She was treated with immunomodulatory therapy with improvement in her seizure frequency and motor function. At this time, surgical intervention has been declined. Conclusions: Rasmussen's encephalitis can be a challenging disgnosis. We have described a rare case of bilateral Rasmussen's encephalitis highlighting the diagnostic challenges and the treatment dilemmas that arise in an adolescent presenting with bilateral inflammatory lesions of Rasmussen's encephalitis.