Encefalopatía respondedora a corticoides asociada a tiroiditis autoinmune (SREAT). Una causa no habitual de compromiso de conciencia en pediatría, reporte de 2 casos / Steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT): an unusual cause of encephalopathy in children, report of two cases

ABSTRACT

Introduction: steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) is a rare condition in children. The pathogenesis and etiology of SREAT has not yet been clearly identified. Clinical features include acute or subacute encephalopathy with neuropsychiatric symptoms, associated with abnormally elevated thyroid antibodies (TA) and symptoms improvement with corticosteroid treatment. Methods and Patients: we present 2 clinical cases; the first a 6 years 8 months male with cephalic myoclonic seizures and behavioral changes, the second a 12 years 10 months female with 4 hospitalizations for encephatlopathy, dystonia and psychomotor agitation. In both patients thyroid function tests and TA were compatible with Hashimoto’s thyroiditis, this associated with neuropsychiatric symptoms did raise the diagnosis of SREAT. Glucocorticoid therapy was started, the first case showed remission of seizures and behavioral improvement, however the second patient had insufficient response, so second line therapy with intravenous immunoglobulin was introduced with good response. This therapy was supplemented by additional long-term glucocorticoids use but when suspended both patients presented relapsing symptoms. Conclusions: Although SREAT is rarely suspected at presentation, it is necessary to consider this diagnosis in patients with encephalopathy, neuropsychiatric symptoms and elevated TA. Further studies are required to elucidate the pathophysiology of this disease and follow-up work to assess comorbidities and long-term complications in pediatric patients.