To the Editor

Sporadic Creutzfeldt-Jakob disease (sCJD), the most common human prion disease, is rare and universally fatal. Its causative agent resists conventional sterilization, persists on instrument surfaces, and has been transmitted iatrogenically through contaminated neurosurgical instruments. ^{Reference Rutala and Weber1–Reference Brown, Brandel and Sato3} International guidance therefore mandates enhanced, prion-specific decontamination of devices that contact high-infectivity neural tissues. ^{Reference Rutala and Weber1} Early recognition is frequently delayed by nonspecific neuropsychiatric onset, and in settings without real-time quaking-induced conversion (RT-QuIC) assays, clinicians lack a rapid, objective trigger for precautionary infection prevention and control (IPC) action. We describe an institutional response in which early diffusion-weighted imaging (DWI) findings on magnetic resonance imaging (MRI) triggered instrument recall and risk-stratified decontamination before biomarker confirmation.

A 56-year-old woman with congenital deafness was admitted in April 2026 with a 1-week history of acute behavioral changes, confusion, and progressive right hemiparesis after a 1-month prodrome of gait instability; noncontrast head CT was nonspecific. Brain MRI on hospital day 3 showed bilateral cortical ribboning on DWI involving the temporal, parietal, and frontal cortices—meeting the CDC criterion of at least two cortical regions (temporal, parietal, or occipital)—with corresponding apparent diffusion coefficient hypointensity and basal ganglia restriction, suggesting sCJD (Figure 1). ^{2,Reference Eisenmenger, Porter and Carswell4} Recognizing the theoretical prion transmission risk, the hospital IPC office activated an emergency containment protocol consistent with national and international guidance. ^{Reference Rutala and Weber1,Reference Ge, Jia and Li5} Using a centralized instrument tracking system, the index lumbar puncture needle was traced to the sterile processing department, and all 102 stainless steel instrument packs processed through the same automated washer-disinfector within a ±2-hour window were recalled and secured within 2 hours of clinical suspicion. Standard steel instruments underwent precleaning with enzymatic detergent, immersion in 1 mol/L sodium hydroxide for ≥60 minutes at room temperature, and prevacuum steam sterilization at 134°C for ≥18 minutes. ^{Reference Rutala and Weber1,6} Three precision endoscopic devices incompatible with concentrated alkali were processed with deconex 28 ALKA ONE-x (Borer Chemie AG, Zuchwil, Switzerland), a caustic-free, high-alkaline chemistry with documented prion-inactivating activity, at 1% concentration and 70°C for ≥10 minutes. ^{Reference Heinzer, Avar and Pfammatter7} Enhanced local exhaust ventilation and N95 respirators were used during sodium hydroxide handling; standard personal protective equipment sufficed for the alkaline cycle. Sterilization efficacy was validated using Class 6 emulating chemical indicators calibrated for extended prion inactivation cycles (134°C, ≥18 min) and Geobacillus stearothermophilus biological indicators; all chemical indicators achieved their end point and all biological indicators were negative. No occupational exposures were reported, and a retrospective audit confirmed strict aseptic technique during the original procedure; the procedure room and patient ward underwent terminal cleaning with 1 mol/L sodium hydroxide.

Figure 1.

Brain MRI findings in probable sporadic Creutzfeldt-Jakob disease. Axial diffusion-weighted imaging (DWI, b = 1,000 s/mm ² ; A–C) shows bilateral cortical ribboning with lace-like hyperintensities in the temporal, parietal, and frontal lobes (arrows), with restricted diffusion in the bilateral caudate nuclei and putamen (horizontal arrows, (B). Corresponding axial T2-weighted images (D–F) show symmetric gyral hyperintensities without mass effect or edema. These imaging patterns prompted immediate activation of prion-specific infection prevention and control protocols before biomarker confirmation. DWI, diffusion-weighted imaging; MRI, magnetic resonance imaging.

Figure 1. Long description

Panel A: An axial diffusion-weighted imaging (DWI) scan of the brain showing bilateral cortical ribboning with lace-like hyperintensities in the temporal, parietal, and frontal lobes, indicated by arrows. Panel B: An axial DWI scan showing restricted diffusion in the bilateral caudate nuclei and putamen, marked by horizontal arrows. Panel C: An axial DWI scan highlighting bilateral cortical ribboning with lace-like hyperintensities in the temporal, parietal, and frontal lobes, indicated by arrows. Panel D: An axial T2-weighted image showing symmetric gyral hyperintensities without mass effect or edema. Panel E: An axial T2-weighted image showing symmetric gyral hyperintensities without mass effect or edema. Panel F: An axial T2-weighted image showing symmetric gyral hyperintensities without mass effect or edema.

Cerebrospinal fluid (CSF) obtained before imaging was acellular with a negative Pandy test and negative Gram stain, bacterial culture, and viral polymerase chain reaction. Quantitative ELISA showed markedly elevated CSF 14-3-3 protein gamma isoform (>80,000 AU/mL; the assay cut-off is 2,000 AU/mL). ^{Reference Matsui, Satoh and Miyazaki8} PRNP gene sequencing revealed a wild-type sequence with no pathogenic variants and methionine homozygosity (MM) at codon 129; together with the clinical, MRI, and CSF findings, this fulfilled current criteria for probable sCJD. ^{Reference Hermann, Appleby and Brandel9} RT-QuIC was not available at the study site during this admission. The patient was transitioned to palliative home care and died approximately 2 weeks after discharge.

This case illustrates that early DWI-MRI findings can serve as an objective trigger for prion-specific IPC in settings where RT-QuIC is inaccessible, ^{Reference Eisenmenger, Porter and Carswell4,Reference McGuire, Peden and Orrú10} enabling containment to begin before biomarker confirmation. Two features of our response merit emphasis. First, instrument traceability permitted complete, time-bound recall: all at-risk packs were secured within 2 hours of suspicion, whereas log-based tracing without digital tracking often yields only probabilistic identification of exposed devices and patients. Second, material-compatible, risk-stratified decontamination—sodium hydroxide for standard steel and a validated caustic-free alkaline chemistry for delicate endoscopes—preserved device integrity while adhering to recognized prion inactivation parameters, ^{Reference Rutala and Weber1,6,Reference Heinzer, Avar and Pfammatter7} with engineered ventilation mitigating occupational exposure to alkaline aerosol. The response also catalyzed durable institutional improvements, including electronic medical record alerts for suspected prion disease, mandatory preprocedure risk assessments for invasive neurodiagnostic procedures, updated standard operating procedures requiring material-compatibility verification, and quarterly simulation drills for prion exposure response. The patient’s prelingual deafness complicated early symptom reporting, underscoring the value of objective imaging-based triggers for equitable diagnosis and timely IPC in patients with communication barriers.

Our observations are limited by their single-case nature: transmission risk was theoretical rather than confirmed, no device or environmental sampling was performed, and the ±2-hour recall window was a locally determined precautionary threshold. Nevertheless, the framework is reproducible and low-technology, and it directly addresses a recognized gap in resource-variable settings. We encourage other institutions to evaluate imaging-triggered, traceability-based prion containment and to report process outcomes so that the approach can be refined with stronger evidence.