Introduction Creutzfeldt‐Jakob disease (CJD) is a rare, fatal neurodegenerative disorder caused by misfolded prion proteins that accumulate in the brain, leading to spongiform encephalopathy. It is characterized by rapidly progressive mental decline and other neurological symptoms, typically resulting in death within a year. Stroke like symptoms as vertigo with CJD was not reported in the literature and hence we present our case. Case Presentation 65‐year old female with past medical history of chronic hypertension presented to the emergency department with persistent dizziness that started a month prior. She described it as spinning. No reported change in mentation or memory issues. On exam she was alert and oriented, chorea‐athetoid type movements of the left hand, abnormal finger to nose worse on the left side, and horizontal nystagmus more prominent on the left side. MRI brain with and without contrast revealed subtle DWI and FLAIR changes over the bilateral caudate and Putamen, right temporal and occipital area. Symptoms were worsening with agitation, visual hallucinations, and cognitive impairments. EEG performed showed right temporal sharp waves and diffuse generalized Triphasic waves, did not respond to anti seizure medications. Autoimmune and Paraneoplastic encephalitis panel came back negative. CSF studies were unremarkable. She had positive CSF RT‐QulC confirmed diagnosis of Creutzfeldt‐Jakob Disease. Discussion CJD is extremely rare with an estimated global incidence of approximately 1 to 2 cases per million people per year. Despite its rarity, early recognition is crucial, as the disease progresses rapidly and can mimic other neurological conditions as Stroke. Clinicians should maintain a high index of suspicion for CJD in patients presenting with vertigo and cerebellar ataxia that quickly evolve into profound cognitive decline. The clinical presentation may initially appear nonspecific, as stroke like symptoms, with early symptoms including ataxia, develop visual disturbances, dysarthria, dysphagia, myoclonus, and marked impairments in coordination and mobility. Due to the atypical nature and rapid progression of symptoms, CJD poses significant diagnostic challenges and often requires a multidisciplinary approach. Conclusions This case highlights the critical importance of including Creutzfeldt‐Jakob Disease in the differential diagnosis of patients presenting with stroke like symptom as vertigo accompanied by rapidly progressive dementia and ataxia. The patient's swift neurological deterioration, along with characteristic EEG findings and positive RT‐QuIC assay, emphasizes the need for early recognition and a high index of clinical suspicion when evaluating unexplained stroke like symptoms, cognitive and motor symptoms. image
Kareem et al. (Sat,) studied this question.