An Unusual Case of Creutzfeldt- Jakob Disease Misdiagnosed as Status Epilepticus: A Case Study

Introduction : Creutzfeldt-Jakob disease (CJD) is a rare neurodegenerative disease worldwide, with approximately 1–1.5 cases per million people per year. It is caused by a type of abnormal protein known as a prion or scrapie PrP. Infectious prions are misfolded proteins that can induce misfolding in normally folded proteins, leading to clinical symptoms. Clinical symptoms vary, with early symptoms including memory problems, behavioral changes, poor coordination, and visual and auditory disturbances. Later symptoms include dementia, involuntary movements, blindness, deafness, weakness, and coma. The varied clinical presentations pose diagnostic challenges for clinicians, requiring multiple diagnostic modalities and a high index of suspicion. Case Presentation : We present a case of a 54-year-old man with a history of complex partial seizures, diabetes mellitus type 2, left temporal lobe surgery secondary to seizures, deep vein thrombosis and pulmonary embolism, left common to external iliac vein wall stent, hypertension, and hyperlipidemia, who was admitted to the Neuro ICU after a motor vehicle accident and was found to have seizures at the time of extrication. He was intubated at the site due to the inability to protect the airway with concern for status epilepticus. The patient had a protracted ICU course due to a lack of improvement despite being treated with multiple antiseizure medications and a negative workup. Furthermore, a rapidly progressive neurological decline marked by refractory encephalopathy, abnormal involuntary movements, and evolving focal-to-diffuse neuroimaging abnormalities developed. Ultimately, the patient tested positive for 14-3-3 in the CSF and was diagnosed with Creutzfeldt- Jakob Disease (CJD) as a delayed diagnosis. Conclusion : Creutzfeldt-Jakob disease (CJD) is a rare disease with varied presentations, posing diagnostic challenges for clinicians. We highlight an interesting case of Creutzfeldt- Jakob Disease (CJD), which was misdiagnosed as status epilepticus due to an unusual presentation causing a diagnostic dilemma. The case highlights features of the clinical examination and imaging findings that are atypical of CJD. Clinicians should be vigilant in the management of patients with status epilepticus who do not respond to conventional antiepileptic medications and may have an alternative diagnosis.