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Sporadic CJD in association with HIV.
Nikolien S van de Ven1, Jaime Vera2,3, John R Jones2,4
1Brighton and Sussex University Hospitals Trust, Royal Sussex County Hospital, Eastern Road, Brighton, BN2 5BE, UK. nikolien.vandeven@bsuh.nhs.uk.
Journal of Neurology
|November 18, 2018
Summary
This study details a rare case of sporadic Creutzfeldt-Jakob disease (CJD) in an HIV-positive patient. Despite initial variant CJD indicators, pathological findings confirmed sporadic CJD, highlighting diagnostic challenges.
Area of Science:
- Neurodegenerative Diseases
- Infectious Disease Co-morbidity
- Prion Diseases
Background:
- Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disorder.
- An unusual case of sporadic CJD in an HIV-positive patient is presented.
- The patient exhibited clinical and radiological features mimicking variant CJD.
Observation:
- A 63-year-old HIV-positive man presented with cognitive and visual disturbances.
- Cerebrospinal fluid (CSF) analysis showed elevated Tau and S-100 proteins, with weakly positive 14-3-3.
- Brain MRI revealed a pulvinar sign, suggestive of variant CJD.
Findings:
- Despite MRI findings and a positive blood test for variant CJD, tonsillar biopsy was negative.
- Prion protein genotyping indicated MV heterozygosity at codon 129.
- Post-mortem histopathology confirmed sporadic CJD.
Implications:
- The association between HIV and CJD remains unclear, with few reported cases.
- Diagnostic challenges arise when sporadic CJD mimics variant CJD, especially in immunocompromised patients.
- Further research is needed to understand the interplay between HIV and prion diseases.
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