Sporadic CJD in association with HIV

van de Ven, Nikolien, Vera, Jaime, Jones, John R, Vundavalli, Sriram and Ridha, Basil H (2018) Sporadic CJD in association with HIV. Journal of Neurology. ISSN 0340-5354

[img] PDF - Accepted Version
Restricted to SRO admin only until 16 November 2019.

Download (268kB)
[img] PDF - Published Version
Restricted to SRO admin only

Download (1MB)

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.

Item Type: Article
Schools and Departments: Brighton and Sussex Medical School > Brighton and Sussex Medical School
Brighton and Sussex Medical School > Global Health and Infection
Subjects: R Medicine
R Medicine > R Medicine (General)
R Medicine > R Medicine (General) > R690 Medicine as a profession. Physicians
R Medicine > RC Internal medicine
R Medicine > RC Internal medicine > RC0031 Internal medicine. Practice of medicine
Depositing User: Jaime Vera Rojas
Date Deposited: 26 Nov 2018 12:42
Last Modified: 27 Nov 2018 09:18
URI: http://srodev.sussex.ac.uk/id/eprint/80396

View download statistics for this item

📧 Request an update