Sporadic CJD - a first case in association with HIV[2].pdf (261.89 kB)
Sporadic CJD in association with HIV
journal contribution
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 RidhaBackground 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 NeurologyISSN
0340-5354Publisher
Springer VerlagExternal DOI
Issue
1Volume
266Page range
253-257Department affiliated with
- BSMS Publications
Full text available
- Yes
Peer reviewed?
- Yes
Legacy Posted Date
2018-11-26First Open Access (FOA) Date
2019-11-16First Compliant Deposit (FCD) Date
2018-11-26Usage metrics
Categories
No categories selectedKeywords
Licence
Exports
RefWorks
BibTeX
Ref. manager
Endnote
DataCite
NLM
DC