• Neurol. Sci. · Aug 2011

    Case Reports

    Steroid-responsive Hashimoto encephalopathy mimicking Creutzfeldt-Jakob disease.

    • Domenico Santoro, Irene Colombo, Isabella Ghione, Lorenzo Peverelli, Nereo Bresolin, Monica Sciacco, and Alessandro Prelle.
    • Department of Neurological Sciences, University of Milan, Milan, Italy. domenicosantoro@fastwebnet.it
    • Neurol. Sci. 2011 Aug 1; 32 (4): 719-22.

    AbstractHashimoto's encephalopathy (HE) is a rare neurological disorder with a heterogeneous group of neurological symptoms associated with high titres of anti-thyroid 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.

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