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Updated: Aug 22, 2026

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Published on: February 14, 2025
Sporadic Creutzfeldt-Jakob disease presenting with nonconvulsive status epilepticus
David Cohen1, Ekrem Kutluay, Jonathan Edwards
1Department of Neurology, University of Michigan Health System, UH1B300/0036 Ann Arbor, MI 48109, USA.
Abstract:
Creutzfeldt-Jakob disease (CJD) is a rare prion disease characterized by a spongiform encephalopathy in humans. Although the characteristic triad of myoclonus, dementia, and periodic EEG activity is easy to recognize, unusual manifestations of the disease may be challenging and create a diagnostic dilemma. We report a case of CJD that occurred in a 26-year-old patient who presented with a receptive (Wernicke's) aphasia secondary to nonconvulsive status epilepticus.
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