Sporadic Creutzfeldt–Jakob disease


  • S. Zhelyazkova Clinic of Nervous Diseases, University Hospital “Alexandrovska”, Medical University – Sofia, Bulgaria, Department of Neurology, Medical University – Sofia, Bulgaria Author
  • S. Nachev Department of Neurosurgery, Laboratory of Neuropathology, Medical University Hospital “Sv. Ivan Rilski” – Sofia, Bulgaria Author
  • O. Kalev Institute of Pathology, Neuromed Campus, Kepler University Hospital, Johannes Kepler University – Linz, Austria Author
  • I. Tournev Clinic of Nervous Diseases, University Hospital “Alexandrovska”, Medical University – Sofia, Bulgaria, Department of Neurology, Medical University – Sofia, Bulgaria, Department of Cognitive Science and Psychology, New Bulgarian University – Sofia, Bulgaria Author




sporadic Creutzfeldt–Jakob disease, prion disease, brain autopsy, electroencephalogram


Sporadic Creutzfeldt–Jakob disease is a rare and fatal human prion disease characterized by a rapidly progressive dementia, myoclonus, cerebellar, pyramidal, extrapyramidal, visual, and psychiatric symptoms. These findings are all non-specific and making 
diagnosis is often difficult at the symptoms onset, especially in case of atypical clinical and radiological presentation. This case report describes a woman in her 60s, who presented with rapid cognitive decline, confusion, ataxia and electroencephalographic changes compatible with non-convulsive status epilepticus. Her symptoms progressively worsened and she died 8 weeks after the onset. The two cerebrospinal fluid analyses were normal and no 14-3-3 protein was detected. The brain MRIs revealed areas of cortical restricted diffusion involving the right frontal and parietal lobe. The electroencephalographic findings of continuous periodic generalized bi-triphasic complexes, typical for sporadic Creutzfeldt–Jakob disease, were detected 1 month after the onset. Sporadic Creutzfeldt–Jakob disease was neuropathologically confirmed. Although sporadic Creutzfeldt–Jakob disease is a rare neurodegenerative disease, it should be considered in the differential diagnosis of all cases with unexplained and rapid cognitive decline and confusion, along with ataxia, pyramidal/extrapyramidal signs, myoclonus and dysphagia.


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How to Cite

Zhelyazkova, S., Nachev, S., Kalev, O., & Tournev, I. (2024). Sporadic Creutzfeldt–Jakob disease. Acta Medica Bulgarica, 51(Suppl 1), 36-40. https://doi.org/10.2478/AMB-2024-0032