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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
PubMed

Insights

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.
Abstract

Keywords:
Creutzfeldt–Jakob diseaseHuman immunodeficiency virus

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