Hashimoto's encephalopathy: A rare proteiform disorder

TY - JOUR

T1 - Hashimoto's encephalopathy

T2 - A rare proteiform disorder

AU - Montagna, Giacomo

AU - Imperiali, Mauro

AU - Agazzi, Pamela

AU - D'Aurizio, Federica

AU - Tozzoli, Renato

AU - Feldt-Rasmussen, Ulla

AU - Giovanella, Luca

PY - 2016

Y1 - 2016

N2 - 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.

AB - 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.

KW - Anti-thyroperoxidase antibodies

KW - Anti-α-enolase antibodies

KW - Autoimmune encephalopathies

KW - Autoimmune thyroid diseases

KW - Hashimoto's encephalopathy

U2 - 10.1016/j.autrev.2016.01.014

DO - 10.1016/j.autrev.2016.01.014

M3 - Review

C2 - 26849953

AN - SCOPUS:84958191389

SN - 1568-9972

VL - 15

SP - 466

EP - 476

JO - Autoimmunity Reviews

JF - Autoimmunity Reviews

IS - 5

ER -