Creutzfeldt-Jakob disease (CJD)

Discussion:

This patient went on to have confirmatory CSF and EEG. 

Cerebrospinal Fluid

  • Csf Protein 0.49H g/L 0.15-0.45

  • Csf Glucose 4.8 mmol/L 2.5-5.0

  • CELL COUNT: x10^6/L

    • Erythrocytes 0

    • Polymorphs 0

    • Lymphocytes 0

  • ​​​HERPES VIRUS MULTIPLEX PCR Result: VIRUS DNA NOT DETECTED

    • COMMENT: Viruses screened with this PCR are: HSV-1, HSV-2, CMV & VZV.

  • Polyomavirus DNA by PCR: Not detected 

  • CJD Protein (14-3-3): POSITIVE

    • ​COMMENT: The 14-3-3 protein is a non-specific marker of central nervous system neuronal injury or death. Based on international experience in carefully selected patients, a positive result has approximately 90% sensitivity and specificiy for sporadic Creutzfeldt-Jacob disease. "False" positives are frequently recognized in encephalitis (especially Herpes simplex) & recent cerebral infarcts. Based on 120 definite (pathologically proven) sporadic patients, total CSF protein >1.0g/L is rarely seen (<1%) in uncomplicated CJD, while total CSF white cell count is never >10 cells/uL

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