Encephalopathy associated with autoimmune thyroid disease in patients with Graves' disease: clinical manifestations, follow-up, and outcomes.
BACKGROUND
The encephalopathy associated with autoimmune thyroid disease (EAATD) is characterized by neurological/psychiatric symptoms, high levels of anti-thyroid antibodies, increased cerebrospinal fluid protein concentration, non-specific electroencephalogram abnormalities, and responsiveness to the corticosteroid treatment in patients with an autoimmune thyroid disease. Almost all EAATD patients are affected by Hashimoto's thyroiditis (HT), although fourteen EAATD patients with Graves' disease (GD) have been also reported.


METHODS
We have recorded and analyzed the clinical, biological, radiological, and electrophysiological findings and the data on the therapeutic management of all GD patients with EAATD reported so far as well as the clinical outcomes in those followed-up in the long term.


RESULTS
Twelve of the fourteen patients with EAATD and GD were women. The majority of GD patients with EAATD presented with mild hyperthyroidism at EAATD onset or shortly before it. Active anti-thyroid autoimmunity was detected in all cases. Most of the patients dramatically responded to corticosteroids. The long term clinical outcome was benign but EAATD can relapse, especially at the time of corticosteroid dose tapering or withdrawal. GD and HT patients with EAATD present with a similar clinical, biological, radiological, and electrophysiological picture and require an unaffected EAATD management.


CONCLUSIONS
GD and HT equally represent the possible background condition for the development of EAATD, which should be considered in the differential diagnosis of all patients with encephalopathy of unknown origin and an autoimmune thyroid disease, regardless of the nature of the underlying autoimmune thyroid disease.
Tamagno
Gianluca
G
Department of Endocrinology and Diabetes Mellitus, St Vincent's University Hospital, University College Dublin, Dublin, Ireland. gianluca.tamagno@ucd.ie
Celik
Yahya
Y
Simó
Rafael
R
Dihné
Marcel
M
Kimura
Kazumi
K
Gelosa
Giorgio
G
Lee
Byung I
BI
Hommet
Caroline
C
Murialdo
Giovanni
G
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000097:blood / Q000276:immunology; D002648:Child; D004569:Electroencephalography / Q000379:methods; D004660:Encephalitis / Q000097:blood / Q000150:complications* / Q000188:drug therapy; D005260:Female; D005500:Follow-Up Studies; D006111:Graves Disease / Q000150:complications* / Q000188:drug therapy; D006801:Humans; D008297:Male; D008875:Middle Aged; D013967:Thyroiditis, Autoimmune / Q000150:complications* / Q000188:drug therapy; D016896:Treatment Outcome; D055815:Young Adult
D000305:Adrenal Cortex Hormones; D001323:Autoantibodies