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Case report
peer-reviewed

A Case Report of Probable Sporadic Creutzfeldt-Jakob Disease: How to Approach Early Diagnosis?



Abstract

Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare and fatal spongiform encephalopathy characterized by rapidly progressive dementia and myoclonus. The rarity of this disease and varied initial symptoms make the early diagnosis fairly challenging. Here, we present a case initially admitted for confusion and bizarre behaviors. She had acute deterioration of mental status, akinetic mutism, and myoclonus jerks four weeks later. Cerebrospinal fluid (CSF) analysis was positive for protein 14-3-3. Brain magnetic resonance imaging (MRI) showed hyperintensities in the bilateral cortex, basal ganglia, and thalami in diffusion-weighted imaging (DWI). Electroencephalogram (EEG) showed bihemispheric periodic lateralizing epileptiform discharges. The probable diagnosis of sCJD was reached based on the clinical features, characteristic findings in her MRI, the EEG, and a positive 14-3-3 CSF assay. The literature was also reviewed for early diagnosis of sCJD. 



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Case report
peer-reviewed

A Case Report of Probable Sporadic Creutzfeldt-Jakob Disease: How to Approach Early Diagnosis?


Author Information

Bowei Tan Corresponding Author

Department of Medicine, Brookdale University Hospital and Medical Center

Carlos Morales Mangual

Department of Medicine, Brookdale University Hospital and Medical Center

Iftekhar Mahmud

Department of Medicine, Brookdale University Hospital and Medical Center

Nosakhare D. Tongo

Department of Medicine, Brookdale University Hospital and Medical Center

Larisa Mararenko

Department of Medicine, Brookdale University Hospital and Medical Center

Arthur Kay

Department of Neurology, Brookdale University Hospital and Medical Center


Ethics Statement and Conflict of Interest Disclosures

Human subjects: Consent was obtained by all participants in this study. Brookdale University Hospital and Medical Center issued approval. This case report has been approved by the Ethics Board of Brookdale University Hospital and Medical Center. . Conflicts of interest: The authors have declared that no conflicts of interest exist.


Case report
peer-reviewed

A Case Report of Probable Sporadic Creutzfeldt-Jakob Disease: How to Approach Early Diagnosis?


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Case report
peer-reviewed

A Case Report of Probable Sporadic Creutzfeldt-Jakob Disease: How to Approach Early Diagnosis?

  • Author Information
    Bowei Tan Corresponding Author

    Department of Medicine, Brookdale University Hospital and Medical Center

    Carlos Morales Mangual

    Department of Medicine, Brookdale University Hospital and Medical Center

    Iftekhar Mahmud

    Department of Medicine, Brookdale University Hospital and Medical Center

    Nosakhare D. Tongo

    Department of Medicine, Brookdale University Hospital and Medical Center

    Larisa Mararenko

    Department of Medicine, Brookdale University Hospital and Medical Center

    Arthur Kay

    Department of Neurology, Brookdale University Hospital and Medical Center


    Ethics Statement and Conflict of Interest Disclosures

    Human subjects: Consent was obtained by all participants in this study. Brookdale University Hospital and Medical Center issued approval. This case report has been approved by the Ethics Board of Brookdale University Hospital and Medical Center. . Conflicts of interest: The authors have declared that no conflicts of interest exist.

    Acknowledgements


    Article Information

    Published: May 30, 2017

    DOI

    10.7759/cureus.1297

    Cite this article as:

    Tan B, Morales mangual C, Mahmud I, et al. (May 30, 2017) A Case Report of Probable Sporadic Creutzfeldt-Jakob Disease: How to Approach Early Diagnosis? . Cureus 9(5): e1297. doi:10.7759/cureus.1297

    Publication history

    Received by Cureus: April 23, 2017
    Peer review began: May 02, 2017
    Peer review concluded: May 20, 2017
    Published: May 30, 2017

    Copyright

    © Copyright 2017
    Tan et al. This is an open access article distributed under the terms of the Creative Commons Attribution License CC-BY 3.0., which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

    License

    This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Abstract

Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare and fatal spongiform encephalopathy characterized by rapidly progressive dementia and myoclonus. The rarity of this disease and varied initial symptoms make the early diagnosis fairly challenging. Here, we present a case initially admitted for confusion and bizarre behaviors. She had acute deterioration of mental status, akinetic mutism, and myoclonus jerks four weeks later. Cerebrospinal fluid (CSF) analysis was positive for protein 14-3-3. Brain magnetic resonance imaging (MRI) showed hyperintensities in the bilateral cortex, basal ganglia, and thalami in diffusion-weighted imaging (DWI). Electroencephalogram (EEG) showed bihemispheric periodic lateralizing epileptiform discharges. The probable diagnosis of sCJD was reached based on the clinical features, characteristic findings in her MRI, the EEG, and a positive 14-3-3 CSF assay. The literature was also reviewed for early diagnosis of sCJD. 



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Bowei Tan

Department of Medicine, Brookdale University Hospital and Medical Center

For correspondence:
tanboweiblue@gmail.com

Carlos Morales Mangual

Department of Medicine, Brookdale University Hospital and Medical Center

Iftekhar Mahmud

Department of Medicine, Brookdale University Hospital and Medical Center

Nosakhare D. Tongo

Department of Medicine, Brookdale University Hospital and Medical Center

Larisa Mararenko

Department of Medicine, Brookdale University Hospital and Medical Center

Arthur Kay

Department of Neurology, Brookdale University Hospital and Medical Center

Bowei Tan

Department of Medicine, Brookdale University Hospital and Medical Center

For correspondence:
tanboweiblue@gmail.com

Carlos Morales Mangual

Department of Medicine, Brookdale University Hospital and Medical Center

Iftekhar Mahmud

Department of Medicine, Brookdale University Hospital and Medical Center

Nosakhare D. Tongo

Department of Medicine, Brookdale University Hospital and Medical Center

Larisa Mararenko

Department of Medicine, Brookdale University Hospital and Medical Center

Arthur Kay

Department of Neurology, Brookdale University Hospital and Medical Center