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Open Access Highly Accessed Research article

Encephalopathy associated with autoimmune thyroid disease in patients with Graves' disease: clinical manifestations, follow-up, and outcomes

Gianluca Tamagno1*, Yahya Celik2, Rafael Simó3, Marcel Dihné4, Kazumi Kimura5, Giorgio Gelosa6, Byung I Lee7, Caroline Hommet8 and Giovanni Murialdo9

Author affiliations

1 Department of Endocrinology and Diabetes Mellitus, St Vincent's University Hospital, University College Dublin, Dublin, Ireland

2 Department of Neurology, Trakya University School of Medicine, Edirne, Turkey

3 Division of Endocrinology, Vall d'Hebron University Hospital, Barcelona, Spain

4 Department of Neurology, University Hospital Düsseldorf, Düsseldorf, Germany

5 Department of Stroke Medicine, Kawasaki Medical School, Okayama, Japan

6 Department of Neurology, San Gerardo Hospital, University of Milano-Bicocca, Monza, Italy

7 Department of Neurology, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea

8 Service of Geriatric Internal Medicine, Regional University Hospital, Tours, France

9 Department of Endocrine and Medical Sciences, University of Genoa, Genoa, Italy

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Citation and License

BMC Neurology 2010, 10:27  doi:10.1186/1471-2377-10-27

Published: 28 April 2010

Abstract

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.