Pediyatrik popülasyonda alışılmamış klinik bulgularla bulunan Hashimoto ensefalopatisi: tanı kriterlerinin gözden geçirilmesi

Abstract

Aim: Hashimoto's encephalopathy is a rare progressive and relapsing disease of presumed autoimmune origin associated with high titers of thyroid antibodies. Also named steroid-responsive encephalopathy, the disease can be cured with early treatment. Hashimoto's encephalopathy may occur with various clinical manifestations, but it often presents with symptoms such as seizures, confusion, hallucination, sleep abnormalities, and behavioural problems. Patients are mostly euthyroid or mildly hypothyroid. Hashimoto encephalopathy is a controversial diagnosis for misdiagnosis and overdiagnosis because of the high prevalence of thyroid antibodies in the population. Therefore, this diagnosis requires strict criteria that can highly indicate the diagnosis. We aimed to present the diagnosis and treatment processes from unusual symptoms of patients who are considered to have Hashimoto's encephalopathy with very strict criteria. Materials and Methods: Here we present five pediatric patients diagnosed with Hashimoto's encephalopathy between 2013 and 2023. The clinical signs and symptoms, laboratory findings, treatment options and response to treatment were obtained from patient records retrospectively. Results: On admission, patients had striking behavioural changes such as hallucinations, insomnia, purposeless laughing as well as signs of encephalopathy such as status epilepticus and confusion; neurological sequelae; history of surgery, and fever. All patients had high levels of thyroid antibodies and they responded perfectly to steroid treatment in a short period of time. Conclusion: Hashimoto’s encephalopathy should be considered in all patients presenting with encephalopathy in the pediatric age group. These patients may have unusual and clinical manifestations.