ESPE Abstracts

Background: Pediatric Hashimoto’s encephalopathy (HE) is a rare, progressive and relapsing steroid- responsive autoimmune encephalitis. Children usually present with subacute cognitive dysfunction, psychiatric symptoms, seizures, and movement disorders associated with elevated thyroid antibodies, specifically thyroid peroxidase (TPO) antibodies. We present a rare pediatric Hashimoto’s encephalopathy with super refractory status epilepticus

Case Report: A 13-year-old male patient, presented with a 3-day febrile illness to our hospital. He was initially treated with antimalarials but later experienced drowsiness, confusion, and short-term memory loss, followed by generalized tonic-clonic seizures that proved challenging to manage. Vital signs and systemic examination were normal. He was admitted in ICU and started on anesthetic and anti-convulsant medications to manage the seizures. Since the clinical suspicion of autoimmune encephalitis was very high, intravenous immunoglobulins was initiated with insignificant improvement. Laboratory tests done to rule out various neuro-infections, autoimmune encephalitis, and other autoimmune conditions were all negative (Table1). Brain MRI revealed bilateral cortical and subcortical oedema without infarction. EEG showed generalized slow wave activity. The thyroid ultrasound showed bilateral multi-cystic nodules. Thyroid antibodies were elevated. Diagnosis of Hashimoto’s encephalopathy (HE) was made and the patient was started on pulse methylprednisolone at 10 mg/kg/day for 3 days followed by tapered doses of prednisolone over 3-4 weeks. There was marked improvement at the end of 4 weeks.

Discussion: HE is a diagnosis of exclusion which is made by demonstration of elevated antithyroid antibodies in the serum. A non-specific slowing of background activity can be seen on EEG of most affected individuals. The MRI of affected patients is usually normal, however focal or diffuse white matter changes may be present. Like in the case of our patient most patients with HE are euthyroid. Corticosteroids are the mainstay treatment for HE and in this case, there was marked improvement following methylprednisolone treatment

Conclusion: Hashimoto’s encephalopathy is an underdiagnosed condition which can cause super refractory status epilepticus in children. A high index of suspicion aids in the diagnosis, management and subsequent better outcomes