Hashimoto Encephalopathy Presenting with New Onset Refractory Status Epilepticus and Clinical Hyperthyroidism

Abstract

NORSE occurs secondary to multiple etiologies including a rare diagnosis of Hashimoto Encephalopathy (HE). The mechanism of HE is not fully understood. Most reported patients with HE are hypothyroid or euthyroid at the time of presentation. Patients with HE commonly present with subacute clinical features, namely behavioral changes, and rarely with the acute presentation of status epilepticus. We report a case of a 14 years old female presenting with New Onset Refractory Status Epilepticus (NORSE) and clinical hyperthyroidism, subsequently developing behavioral symptoms with multiple relapses requiring various treatment modalities including pulse steroid therapy, intravenous immunoglobulin therapy, plasmapheresis and eventually partial thyroidectomy.