Case report| Volume 35, P72-73, January 2017

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Hashimoto’s encephalopathy mimicking Creutzfeldt-Jakob disease

Published:October 13, 2016DOI:


      • Hashimoto’s encephalopathy is a rare, ill-defined, autoimmune neurologic disorder.
      • A woman presents with subacute cognitive decline, ataxia, and myoclonus.
      • Treatment with high dose corticosteroids quickly reverses symptoms.
      • Consider Hashimoto’s encephalopathy after eliminating other etiologies.


      Hashimoto’s encephalopathy is a rare, imprecisely defined autoimmune neurologic syndrome associated with Hashimoto’s thyroiditis that normally responds to corticosteroids. Here, we describe the case of a 55-year-old woman who presented with subacute cognitive decline and ataxia. Neoplastic, paraneoplastic, infectious, and metabolic etiologies were ruled out. Anti-TPO antibody level was markedly elevated at 966 U/mL. After one month of 60 mg/day of oral prednisone, she felt back to baseline and her Montreal Cognitive Assessment dramatically improved. Physicians should strongly consider this uncommon diagnosis in patients with rapid cognitive decline and no other clear etiology.


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