Abstracts

Rationale: Rasmussen's encephalitis (RE) is a rare acquired inflammatory encephalopathy characterized by cerebral hemispheric dysfunction and hemiatrophy, refractory partial epilepsy, cognitive deterioration and hemiparesis. Since the current data on optimum treatment modalities is inadequate, a retrospective review of patients with Rasmussen's encephalitis was carried out. Methods: A retrospective review of SUNY Upstate Medical University records for 2011 and 2012 revealed 2 cases of brain biopsy-diagnosed and 1 case of possible Rasmussen's encephalitis. Results: Patients were in their 20's and 30's, and all had intractable epilepsy. Two of them had refractory complex partial status epilepticus and neurocognitive disability. All had brain MRIs, while functional imaging in the form of SPECT was performed in 2 cases, and fMRI was performed in one. All had repeated video-EEG monitoring. Two patients underwent epilepsy surgery: 1 had a temporal lobectomy at 10 years of age, eventually necessitating a right peri-insular hemispherectomy, with postoperative left-sided hemiparesis and nonobstructive hydrocephalus. One had right parietal and posterior temporal resection as well as VNS, under consideration for further surgery. One had cerebral palsy, autism and possible RE; her seizures were controlled medically. All were on 3 antiepileptic medications. One needed an immune modulator, Imuran and her seizures improved after the addition of Coenzyme Q10. None of the patients had a family history of epilepsy. Rasmussen's encephalitis must be diagnosed and managed appropriately. Although a biopsy would diagnose RE definitively, the European consensus statement proposed diagnostic criteria focusing on clinical, electroencephalographic, and neuroimaging features. The pathology remains unclear. One case study suggests a reduction in benzodiazepine receptors in the affected hemisphere while others suggest an immune-mediated process with auto-antibodies against GluR3. The use of corticosteroids, IVIG and plasma exchange has been suggested as an alternative therapy for refractory status epilepticus by the Neurocritical Care Society Status Epilepticus Guideline Writing Committee. Tailored AED therapy is essential. Functional and anatomic hemispherectomy interventions may contribute greatly to seizure control, but the risks and benefits must be thoroughly assessed. Conclusions: RE can be highly disabling, and diagnosis with appropriate pharmacological and surgical management is imperative to control seizures, reduce morbidity and improve quality of life. Given the low prevalence of RE, multi center clinical studies are needed.