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Sporadic CJD - a first case in association with HIV[2].pdf (261.89 kB)

Sporadic CJD in association with HIV

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posted on 2023-06-09, 16:00 authored by Nikolien van de Ven, Jaime Vera RojasJaime Vera Rojas, John R Jones, Sriram Vundavalli, Basil H Ridha
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.

History

Publication status

  • Published

File Version

  • Accepted version

Journal

Journal of Neurology

ISSN

0340-5354

Publisher

Springer Verlag

Issue

1

Volume

266

Page range

253-257

Department affiliated with

  • BSMS Publications

Full text available

  • Yes

Peer reviewed?

  • Yes

Legacy Posted Date

2018-11-26

First Open Access (FOA) Date

2019-11-16

First Compliant Deposit (FCD) Date

2018-11-26

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