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Sporadic CJD in association with HIV

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Abstract

Background

Creutzfeldt-Jakob disease (CJD) is a rapidly progressive fatal neurodegenerative disorder. We report an unusual case of pathologically confirmed sporadic CJD developing in a HIV-positive patient but presenting with clinical and radiological features suggestive of variant CJD.

Case presentation

A 63-year-old man with chronic stable HIV developed progressive difficulties with decision-making, obsessive compulsive disorder and visual hallucinations over 3 months. CSF examination detected a weakly positive 14-3-3 protein, elevated S-100 protein, and siginificantly elevated total-Tau protein. Brain MRI revealed bilateral abnormal signal within the posterolateral thalami compatible with pulvinar sign. Further investigations revealed a negative tonsillar biospy and positive blood test consistent with variant CJD. However, prion protein genotyping detected MV heterozygosity at codon 129 and post-mortem histopathological examination was consistent with sporadic CJD.

Conclusion

Although MRI findings were suggestive of variant CJD, the short residence in the UK and MV heterozygosity are aytpical, and the histopathological examination was consistent with sporadic CJD. With only two cases of HIV and sporadic CJD reported so far, the association of CJD with HIV remains unclear.

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Authors and Affiliations

  1. Brighton and Sussex University Hospitals Trust, Royal Sussex County Hospital, Eastern Road, Brighton, BN2 5BE, UK

    Nikolien S. van de Ven, Jaime Vera, John R. Jones, Sriram Vundavalli & Basil H. Ridha

  2. Department of Global Health and Infection, Brighton and Sussex Medical School, University of Sussex, Brighton, UK

    Jaime Vera

  3. King’s College Hospital, London, UK

    John R. Jones

Authors
  1. Nikolien S. van de Ven
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  2. Jaime Vera
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  3. John R. Jones
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  4. Sriram Vundavalli
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  5. Basil H. Ridha
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Corresponding author

Correspondence to Nikolien S. van de Ven.

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The authors report no disclosures relevant to this work.

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No requirement for ethics committee review. The patient’s wife has given approval for this case report to be submitted.

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van de Ven, N.S., Vera, J., Jones, J.R. et al. Sporadic CJD in association with HIV. J Neurol 266, 253–257 (2019). https://doi.org/10.1007/s00415-018-9116-0

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  • DOI: https://doi.org/10.1007/s00415-018-9116-0

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