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: Giacomo Montagna, Mauro Imperiali, Pamela Agazzi, Federica D'Aurizio, Renato Tozzoli, Ulla Feldt-Rasmussen, Luca Giovanella
• Journal: Autoimmunity reviews (Autoimmun Rev) Vol. 15 Issue 5 Pg. 466-76 (May 2016) ISSN: 1873-0183 [Electronic] Netherlands
• PMID: 26849953 (Publication Type: Journal Article, Review, Systematic Review)
• Copyright: Copyright © 2016 Elsevier B.V. All rights reserved.

Chemical References

• Autoantigens
• Biomarkers
• Iron-Binding Proteins
• Steroids
• TPO protein, human
• Iodide Peroxidase

Topics

• Autoantigens (immunology)
• Biomarkers (analysis)
• Cognition Disorders (etiology)
• Diagnosis, Differential
• Encephalitis (immunology, therapy)
• Hashimoto Disease (immunology, therapy)
• Humans
• Iodide Peroxidase (immunology)
• Iron-Binding Proteins (immunology)
• Steroids (therapeutic use)
• Treatment Outcome