ESPE Abstracts (2014) 82 P-D-1-2-252

Autoimmune Encephalopathy in a Boy with Graves' Disease

Anna Kucharska, Katarzyna Kadziela & Beata Pyrzak

Department of Paediatrics and Endocrinology, Medical University of Warsaw, Warsaw, Poland

Background: Autoimmune encephalopathy is usually reported in patient with Hashimoto’s thyroiditis and in Graves’ disease is rather rare, especially in children.

Method: We report a boy of 15 years diagnosed with Graves’ disease and treated ineffectively with thyrostatics. After 2 years of the therapy he had recurrence of hyperthyroidism and underwent radical treatment with ablative dose of 131I. After 2 weeks the patient suffered from tachycardia and weakness, increasing sleepiness and progressive qualitative and quantitative disorders of consciousness. Body temperature was normal. Laboratory examinations revealed severe thyrotoxicosis and increase of antithyroid antibodies in blood. Thyroid storm was suspected and typical treatment was administered, including steroids. Symptoms of thyrotoxicosis and consciousness disorders completely withdrew. After 6 weeks hypothyroidism developed and substitution with left- thyroxine was introduced to receive euthyroidism. Three months later again disorders of consciousness appeared again similarly to the previous episode.

Results: Electroencephalography, CT and MR of the CNS were normal. The cerebro-spinal fluid lab evaluation was within normal reference values. In the serum high concentrations of antithyroid antibodies were found without hormonal dysfunction. Taking into account the encephalopathy associated with autoimmune thyroid disease, steroid therapy was administered (i.v. pulses and than orally). The fast improvement was observed with complete withdrawal of neurological symptoms.

Conclusion: Lack of thyroid dysfunction during the second episode, high levels of antithyroid antibodies, exclusion of CNS infectious diseases and very good response to steroids confirmed the diagnosis of autoimmune encephalopathy. Patient was treated with steroids for 8 months in decreasing doses without the recurrence of symptoms. During 3 years follow-up he remained in complete remission.

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