title: Hashimoto's encephalopathy: A rare proteiform disorder.
abstract: Hashimoto's encephalopathy (HE) is a rare not well understood, progressive and relapsing multiform disease, characterized by seizures, movement disorders, subacute cognitive dysfunction, psychiatric symptoms and responsiveness to steroid therapy. The disorder is generally associated with thyroid diseases and the most common feature is the presence of anti-thyroperoxidase antibodies (TPOAb). Patients are usually euthyroid or mildly hypothyroid at presentation. All age groups can be affected. The pathophysiology is still unclear, especially the link between elevated serum TPOAb and the encephalopathy. Most reported cases occurred in women and girls. Unspecific symptoms, non-pathognomonic laboratory neurophysiology and neuroimaging features make its diagnosis a real challenge for clinicians. The case of a 16 year old boy, with a clinical picture of HE associated with hypothyroidism, demonstrating an excellent response to high dose steroids is presented together with a systematic review of the literature.
authors: Department of Pediatrics, Ente Ospedaliero Cantonale, Lugano, Switzerland.
authors: Giacomo
authors: G
authors: Montagna
authors: Department of Laboratory Medicine, Ente Ospedaliero Cantonale, Lugano, Switzerland.
authors: Mauro
authors: M
authors: Imperiali
authors: Division of Neurology, Neurocenter of Southern Switzerland, Lugano, Switzerland.
authors: Pamela
authors: P
authors: Agazzi
authors: Department of Laboratory Medicine, S. Maria degli Angeli Hospital, Pordenone, Italy.
authors: Federica
authors: F
authors: D'Aurizio
authors: Department of Laboratory Medicine, S. Maria degli Angeli Hospital, Pordenone, Italy. Electronic address: renato.tozzoli@aas5.sanita.fvg.it.
authors: Renato
authors: R
authors: Tozzoli
authors: Department of Endocrinology, Copenhagen University Hospital, Copenhagen, Denmark.
authors: Ulla
authors: U
authors: Feldt-Rasmussen
authors: Division of Nuclear Medicine and Thyroid Centre, Oncology Institute of Southern Switzerland, Bellinzona, Switzerland.
authors: Luca
authors: L
authors: Giovanella
mesh_terms: D001324:Autoantigens / Q000276:immunology; D015415:Biomarkers / Q000032:analysis; D003072:Cognition Disorders / Q000209:etiology; D003937:Diagnosis, Differential; D004660:Encephalitis / Q000276:immunology* / Q000628:therapy; D050031:Hashimoto Disease / Q000276:immunology* / Q000628:therapy; D006801:Humans; D007453:Iodide Peroxidase / Q000276:immunology; D033862:Iron-Binding Proteins / Q000276:immunology; D013256:Steroids / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D001324:Autoantigens; D015415:Biomarkers; D033862:Iron-Binding Proteins; D013256:Steroids; C056658:TPO protein, human; D007453:Iodide Peroxidase
keywords: Anti-thyroperoxidase antibodies; Anti-α-enolase antibodies; Autoimmune encephalopathies; Autoimmune thyroid diseases; Hashimoto's encephalopathy