Steroid-responsive Hashimoto encephalopathy mimicking Creutzfeldt-Jakob disease

Hashimoto's encephalopathy (HE) is a rare neurological disorder with a heterogeneous group of neurological symptoms associated with high titres of antithyroid antibodies. Clinical manifestations may include encephalopathic features such as seizures, behavioural and psychiatric manifestations, movement disorders and coma. The objective of this presentation is to describe a patient with this rare and controversial clinical syndrome mimicking Creutzfeldt-Jakob disease, associated with a Hashimoto euthyroid thyroiditis and with a significant response to high dose intravenous prednisone. The responsiveness of this syndrome to steroids suggests that this disorder involves immune pathogenic mechanisms, as previous reviews reported.