Endocrine Abstracts (2017) 49 EP1223 | DOI: 10.1530/endoabs.49.EP1223

An interesting etiology in childhood choreoathetosis; autoimmune hyperthyroiditis

Ayca Torel Ergur1, Sevinc Odabasi Günes1, Cihat Sanli2 & Rabia Gundogan2

1Kirikkale University Faculty of Medicine, Department of Pediatric Endocrinology, Kirikkale, Turkey; 2Kirikkale University Faculty of Medicine, Department of Pediatric Cardiology, Kirikkale, Turkey.

Introduction: Neurologic symptoms due to autoimmune encephalopathy related to autoimmune hyperthyroidism (AEAH) cases are very rare in childhood. The reason why hyperthyroidism causes this situation is still unknown. In this case we discussed the diagnosis, treatment and follow-up of a case with choreoathetosis in the review of literature.

Case: 15 years old female admitted to hospital with the complaint of nervousness, discomfort for last 3 weeks. There was not any particular feature in personal and family history. In physical examination, heart rate was 110/min, goiter, exophthalmos, dysarthria, involuntary hand-foot movements, and decrement of fine motor skills was observed. Laboratory results were sT3: 12.59 ng/dl, sT4:3.6 ng/dl, TSH:0.007 μU/ml anti-TPO 591.4 595 IU/ml, ani-Tg:357.9 IU/ml, TRAb: positive. In thyroid ultrasonography volume >+2S.D., and parenchymal heterogeneity were observed. History of beta streptococcus infection was negative, mitral valve prolapse and non-rheumatologic mitral regurgitation was seen on echocardiography so Sydenham’s chorea was not considered. Lupus antibodies were negative; C3, C4, cranial MR and MR spectrogram were normal. Anti-thyroid and beta-blocker treatment were started. On the 14th day of the treatment major recovery in patient’s walking and talking were observed. Recovery of the fine motor skills was evaluated comparatively with weekly video records. On the first month of the treatment fine motor skills were definitely normal.

Discussion: Cranial hypoperfusion due to cerebral vasculitis, autoimmunity specific to cerebral tissues and primary demyelination secondary to neuronal dysfunction were blamed in pathogenesis of AEAH. Plasmapheresis and corticosteroids are used in addition to anti-thyroid drugs in the treatment. The best aspect of is our patient responded anti-thyroid drugs well. Our case is very challenging since it reminds us the autoimmune thyroiditis in the etiology of major neurologic symptoms in children and adolescents.

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