Related Experiment Video
Updated: Jun 25, 2026

Prion Safety Laboratory Swipe Test
Published on: February 14, 2025
Inflammatory cerebrospinal fluid in sporadic Creutzfeldt-Jakob disease
Esther Bui1, Eric Ehrensperger, Demetrios J Sahlas
1Division of Neurology, Department of Medicine, Sunnybrook Health Sciences Centre, Toronto, Ontario, Canada.
Background:
Sporadic Creutzfeldt-Jakob disease (CJD) is a fatal, transmissible spongiform encephalopathy characterized by rapidly progressive dementia, myoclonus, ataxia and akinetic mutism. The underlying mechanism is believed to be a conformational change of a native prion protein which characteristically fails to provoke an immune response. Commensurate with the latter, cerebrospinal fluid (CSF) classically exhibits a non-inflammatory profile.
Cases:
We report two patients with pathologically-proven sporadic CJD presenting with a significant CSF pleocytosis.
Conclusion:
Although uncommon, the presence of an inflammatory CSF profile should not exclude the diagnosis of sporadic CJD.
Related Concept Videos
Cerebral Edema ll: Pathophysiology
Cerebrospinal Fluid
CSF Production
CSF is produced mainly in the choroid plexus, a network of capillaries and ependymal cells located within the ventricular system of the brain.
Viral Meningitis
Cryptococcal Meningitis
Increased Intracranial Pressure ll: Pathophysiology
Cerebral Edema l: Introduction

