Background: Hashimoto encephalopathy (HE) is a rare autoimmune disorder. It is defined as a ‘corticosteroid-responsive encephalopathy associated with thyroiditis’.
Clinical observation: We describe a boy who suffered from HE, responded only to intravenous immunoglobulin therapy. This is the first case report of immunoglobulin therapy in paediatric HE.
Conclusion: After review of the literature, we recommend that unexplained encephalopathy in children should lead to evaluation of thyroid autoantibody titres. Immunoglobulins should be considered.