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Creutzfeldt-Jakob Disease Presenting as Stroke

A Case Report and Systematic Literature Review

Sharma, Divya K. MBBS; Boggild, Mike MBChB, MRCP, MD; van Heuven, Annemarie W. MD; White, Richard P. MD, FRCP, FRACP

doi: 10.1097/NRL.0000000000000107
Review Article

Background: Creutzfeldt-Jacob disease (CJD) is a human prion disease generally characterized by subacute changes in behavior and intellectual function, often followed by ataxia, vision changes, and myoclonus. Ten percent of cases may present atypically, both symptomatically and in respect to initial investigations.

Methods: We report a case of CJD mimicking acute stroke and review all similar cases in the magnetic resonance imaging era reported in English, identified through a PubMed and SCOPUS search.

Results: A 68-year-old woman presented with an acute left parietal syndrome, initially referred as a left middle cerebral artery territory stroke. Structural imaging was unremarkable and focal electroencephalogram changes suggested nonconvulsive status epilepticus. Subsequent clinical progression, with the development of cortical high signal on diffusion-weighted imaging and positive 14-3-3 protein in the cerebrospinal fluid, confirmed a diagnosis of CJD. Review of the literature identified 14 further cases mimicking both anterior and posterior stroke syndromes.

Conclusions: CJD develops primarily within a population in whom stroke risk factors are common and represents a rare but important stroke mimic. Negative vascular imaging in elderly patients with apparent acute stroke syndromes should prompt diagnostic review including consideration of prion diseases.

*Mater Health Services North Queensland

Department of Neurology, The Townsville Hospital, Townsville, Qld, Australia

The authors declare no conflict of interest.

Reprints: Divya K. Sharma, MBBS, Mater Health Services North Queensland, The Townsville Hospital, 100 Angus Smith Drive, Townsville 4814, Qld, Australia. E-mail:

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