Dramatic Improvement Followed by Rapid Deterioration after Glucocorticoid Pulse Therapy in a Case of Sporadic Creutzfeldt-Jakob Disease with Hashimoto's Encephalopathy Features: A Case Report

Abstract Creutzfeldt-Jakob disease (CJD) is a rare and fatal transmissible spongiform encephalopathy that results in rapidly progressive dementia. The diagnosis of CJD is often delayed due to the reliance on brain tissue biopsy, which is not a routine clinical examination. Therefore, the confirmation of CJD in patients is often delayed. We reported a case of patient presented with rapid-onset neurological symptoms, initially resembling Hashimoto's Encephalopathy but later diagnosed as CJD. The patient's serum thyroid antibodies were significantly elevated, which initially led us to consider a diagnosis of Hashimoto's encephalopathy (HE). After receiving steroid pulse therapy, the patient's clinical symptoms dramatically improved, but one week later, there was a rapid deterioration in cognitive function. The patient's cerebrospinal fluid (CSF) tested positive for 14-3-3 protein, and the RT-QuIC assay revealed the presence of prion protein in the CSF. The patient was ultimately diagnosed with CJD. We present the series of EEG and MRI abnormalities to raise awareness of the value of diagnosing early cerebral injury in patients with CJD.