title: Steroid-responsive Hashimoto encephalopathy mimicking Creutzfeldt-Jakob disease.
abstract: Hashimoto'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.
authors.affiliation: Department of Neurological Sciences, University of Milan, Milan, Italy. domenicosantoro@fastwebnet.it
authors.forename: Domenico
authors.initials: D
authors.lastname: Santoro
authors.forename: Irene
authors.initials: I
authors.lastname: Colombo
authors.forename: Isabella
authors.initials: I
authors.lastname: Ghione
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Peverelli
authors.forename: Nereo
authors.initials: N
authors.lastname: Bresolin
authors.forename: Monica
authors.initials: M
authors.lastname: Sciacco
authors.forename: Alessandro
authors.initials: A
authors.lastname: Prelle
mesh_terms: D000368:Aged; D000374:Aggression; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001143:Arousal / Q000502:physiology; D001323:Autoantibodies / Q000032:analysis; D001921:Brain / Q000473:pathology; D001927:Brain Diseases / Q000175:diagnosis* / Q000188:drug therapy* / Q000523:psychology; D002540:Cerebral Cortex / Q000473:pathology; D003221:Confusion / Q000209:etiology / Q000523:psychology; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis*; D003937:Diagnosis, Differential; D004569:Electroencephalography; D004660:Encephalitis; D006212:Hallucinations / Q000209:etiology / Q000523:psychology; D050031:Hashimoto Disease / Q000175:diagnosis* / Q000188:drug therapy* / Q000523:psychology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D009460:Neurologic Examination; D011595:Psychomotor Agitation / Q000209:etiology; D013954:Thyroglobulin / Q000276:immunology; D014057:Tomography, X-Ray Computed
chemical_list: D000893:Anti-Inflammatory Agents; D001323:Autoantibodies; D013954:Thyroglobulin; D008775:Methylprednisolone