Steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) in childhood

Steroid responsive encephalopathy with autoimmune thyroiditis (SREAT) is a clinically and electrographically heterogeneous steroid-responsive encephalopathy associated with thyroid autoantibodies. We report an adolescent with SREAT and review PubMed literature relating to childhood.

A 14-year-old boy, without any preceding history of trauma, meningoencephalitis or seizures, was admitted in a comatose state. A similar episode of loss of consciousness 2 months prior with normal neuroimaging and electroencephalogram (EEG) had been followed by behavioral alterations. A year previously, during evaluation for increased appetite and poor weight gain, he was noted to have small goitre with thyroid-stimulating hormone (TSH) 7.26 mIU/L, T3 1.232 nmol/L, and T4 117.63 nmol/L. Routine hemogram, blood biochemistry, thyroid function tests including free hormone levels, ultrasonography thyroid and magnetic resonance imaging were normal. EEG showed diffuse slowing of all waves. Cerebrospinal fluid showed no pleocytosis and electrophoresis showed oligoclonal band. Viral studies and serum N-methyl-D-aspartate receptor antibody levels were negative. Anti-thyroid peroxidase (Anti-TPO) antibodies were raised. Intervention was with intravenous dexamethasone 4 mg every 6 h for 1 week followed by tapering schedule of oral prednisolone over 6 months.

He regained consciousness after the second dose of dexamethasone and was discharged on day 7 in a fully conscious and ambulant state on a tapering course of low dose prednisolone for 6 months. He remains euthyroid with normal sensorium and behavior at 18 months follow-up. Only 50 cases below 18 years age were identified amongst 300 PubMed articles up to 31 July 2013.

Prompt steroid therapy following early recognition by high clinical suspicion and measurement of antithyroid antibody titers can lead to a favorable prognosis in SREAT.