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Hashimoto's encephalopathy: A rare proteiform disorder

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  • Giacomo Montagna
  • Mauro Imperiali
  • Pamela Agazzi
  • Federica D'Aurizio
  • Renato Tozzoli
  • Ulla Feldt-Rasmussen
  • Luca Giovanella
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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.

Original languageEnglish
JournalJournal of Autoimmunity
Volume15
Issue number5
Pages (from-to)466-76
Number of pages11
ISSN0896-8411
DOIs
Publication statusPublished - May 2016

    Research areas

  • Autoantigens, Biomarkers, Cognition Disorders, Diagnosis, Differential, Encephalitis, Hashimoto Disease, Humans, Iodide Peroxidase, Iron-Binding Proteins, Steroids, Treatment Outcome, Journal Article, Review

ID: 49765425