Conservative Infection Prevention and Control Practices in a Patient With Confirmed Creutzfeldt-Jakob Disease

Abstract

Creutzfeldt-Jakob disease (CJD) presents unique infection prevention and control (IPC) challenges because prions resist conventional disinfection and sterilization methods, and evidence guiding reusable endoscope reprocessing remains limited. We describe the management of a 61-year-old woman with confirmed sporadic CJD who required percutaneous endoscopic gastrostomy (PEG) tube placement as part of her care plan. Following multidisciplinary risk assessment and consultation of available literature, device manufacturers, and sterile processing, the IPC team recommended an alternative approach to avoid potential prion contamination of reusable endoscopes. Percutaneous endoscopic gastrostomy placement was successfully performed through interventional radiology using single-use instrumentation, and associated medical waste was managed according to institutional policy. This case highlights the importance of early IPC involvement, multidisciplinary collaboration, and proactive risk assessment when evidence and guidelines are insufficient. Alternative procedural approaches may provide practical, conservative strategies to balance patient-centered care with infection prevention priorities, particularly in resource-constrained healthcare settings.

This case describes the infection prevention and control challenges of performing a common procedure in a patient with confirmed Creutzfeldt-Jakob disease (CJD), where guidance for reusable endoscope reprocessing remains limited. A conservative, multidisciplinary, tiered approach prioritizes alternative procedures that avoid reusable endoscopes when feasible, while providing mitigation strategies such as prion-specific reprocessing or quarantine when reusable equipment must be used, demonstrating how proactive decision-making can help mitigate potential iatrogenic transmission risk.

Clinically, Creutzfeldt-Jakob disease (CJD), a prion disease, presents with rapidly worsening dementia, progressing to myoclonic jerks, akinetic mutism, and inevitably death with a median survival of 5 months [1, 2]. Approximately 85% of CJD cases occur sporadically, while other forms may be inherited, iatrogenic, or zoonotic [3]. Routine person-to-person transmission does not occur through standard health care interactions; however, iatrogenic CJD has been associated with exposure to prion-contaminated tissue or medical equipment, including neurosurgical instruments and transplanted or extracted tissue from affected individuals [3, 4]. Relatively recent advances in our abilities to detect prions through assays such as real-time quaking-induced conversion have demonstrated prion seeding activity and infectivity in non–central nervous system tissues such as the skin [5]. We present a patient with confirmed CJD, not to highlight the diagnostic characteristics of the disease, but to illustrate real-world infection prevention and control (IPC) decision making for a common procedure performed under conditions of uncertainty and limited guidance. This is complicated by the resistance of prion diseases, including CJD, to conventional sterilization methods [6, 7].

CLINICAL CASE DESCRIPTION

Our patient was a previously independent 61-year-old Caribbean female who initially presented with visual disturbances, prompting cessation of driving and multiple medical evaluations. Findings from her initial imaging and optometry evaluation were unremarkable, but over a 4- to 5-week period, she developed progressive cognitive decline, including memory loss, mild disorientation, and rare perseverations. Despite extensive early evaluations and multiple diagnostic tests, her results remained largely benign.

DIAGNOSTIC CONFIRMATION

A month after the onset of the patient's initial symptoms, her memory and cognitive decline progressed. Early in her hospitalization, she would exhibit rare, abrupt rhythmic unilateral hemibody jerking movements that often presented with tactile stimuli, which initially prompted continuous electroencephalogram that did not note epileptic activity, helping lower seizures on the differential. Magnetic resonance imaging of her brain showed no signs of stroke or tumor but instead demonstrated stereotypical patterns of CJD, such as bilateral caudate hyperintensities with cortical ribboning on diffusion-weighted imaging sequencing, as shown in Figure 1 [1]. Hemibody jerking movements during diagnostics resulted in suboptimal image clarity.

Figure 1.

Apparent diffusion coefficient in brain magnetic resonance imaging. A, Orange arrows represent bilateral caudate hyperintensities; green arrows show cortical ribboning, more prominent in the left hemisphere. B, Subtle hyperintensity in the left lobe and caudate.

Figure 1 illustrates diagnostic imaging findings characteristic of sporadic CJD, supporting clinical confirmation of the diagnosis.

Her new presentation raised substantial concerns for CJD due to its unique presentation. She underwent extensive laboratory evaluation for infectious and autoimmune mimickers, including viral, bacterial, and autoimmune encephalitis. Findings from cerebrospinal fluid testing by real-time quaking-induced conversion were positive, supporting the diagnosis of CJD. Through discussions with the family, the patient was determined to have no risk factors for familial, iatrogenic, or zoonotic CJD and was subsequently diagnosed with sporadic CJD. Due to the family's religious beliefs and patient's previously expressed wishes, we pursued percutaneous endoscopic gastrostomy (PEG) tube placement and medical optimization of symptoms for the duration of her life.

INFECTION PREVENTION AND CONTROL

Disinfection and Sterilization Practices for Instruments Used in Prion Diseases

While CJD is not transmitted through routine person-to-person contact, prions can persist on medical instruments and pose a risk of iatrogenic transmission. Prions have demonstrated resistance to traditional disinfection and sterilization practices, leading the Centers for Disease Control and Prevention (CDC) and the World Health Organization (WHO) to recommend different modalities for reprocessing reusable instruments used on patients with suspected or confirmed CJD [4, 7]. Much of the understanding of decontamination comes from studies on transmissible spongiform encephalopathies in animals, particularly scrapie in mice and hamsters, which have been used to test various inactivation methods such as chemical exposure and steam sterilization [8]. However, inconsistencies in tissue type, prion strain stability, and inoculum size have produced conflicting results, making it difficult to determine whether these findings reflect real hospital conditions and complicating the application of animal model data to human infection control practices [8]. Literature reviews and meta-analyses on best practices and references from the CDC and WHO provide recommendations that differ in approach, with no prospective studies validating endoscope decontamination protocols in prion cases. Similarly, studies have not evaluated the true effectiveness of cleaning and reprocessing methods in health care settings; rather, recommendations from the CDC have come from in vitro studies. Antiquated publications remain a primary reference for prion resistance characterization due to the limited newness of research and policy implementations. The updated 2026 CDC guidance primarily focuses on surgical instrumentation protocols with a brief statement on devices sensitive to heat and moisture that is nonspecific to endoscopes. The guidance recommends soaking in a 2-molar (2 M) aqueous solution of sodium hydroxide (NaOH) or undiluted sodium hypochlorite for 1 hour while acknowledging potential damage to devices, with no clear indication that this effectively inactivates prions [4]. The WHO similarly relies on national and international policy that underlines IPC guidelines for transmissible spongiform encephalopathy that are ambiguous to the use of flexible endoscopes and favor either single-use scopes or the destruction of a reusable scope after use on a confirmed case. Based on recommendations from the WHO, heat-sensitive instruments exposed to CJD should be immersed in sodium hypochlorite (NaClO) for 1 hour prior to being reprocessed [9]. Recommendations from outside the United States have notable differences on how to handle flexible gastroenterology endoscopes used on patients with confirmed CJD, with Switzerland's SwissNoso recommendations from 2016 advising that the scope be incinerated [10]. Unlike the CDC and WHO, SwissNoso provides stratified standards based on prion classification and tissue infectivity. The global guidelines published by the World Gastroenterology Organisation and the World Endoscopy Organization characterize the risk of transmission as extremely low when careful attention is paid to the decontamination process [6]. A domestic infection prevention organization, the Association for Professionals in Infection Control and Epidemiology suggests that chlorine is the only compound to inactivate these prions; however, this chemical is incompatible with some endoscopes, as their manufacturer states that it can compromise the integrity of the device [11]. Kampf et al proposed that it is “not possible to evaluate the real risk of prion protein transmission via flexible endoscopes . . . [as] no studies were found measuring prion proteins . . . either after use in a patient with proven or suspected prion disease without processing or . . . after reprocessing” [2]. Recent research has explored novel sterilization technologies for prion inactivation, including vaporized hydrogen peroxide, peracetic acid, and gas plasma systems, which show promise for heat-sensitive instruments while maintaining device integrity [12, 13].

IPC Protocols in Our Health Facility for Patients With Prion Diseases

Given the rarity of CJD transmission through patient contact, neither isolation nor transmission-based precautions are routinely indicated. Figure 2 visualizes our institutional workflow of a suspected or confirmed case of CJD. When a 14-3-3 protein test is ordered at our facility, the IPC team is automatically alerted by the microbiology laboratory and confirms with the ordering provider if CJD is included in the differential diagnosis. If CJD is suspected, our hospital CJD policy is initiated. This includes adding an alert notification (“rule-out transmissible spongiform encephalopathy”) in the electronic medical record viewable to all health care personnel caring for the patient. The notification includes the following statement: “Patient is being ruled out for Creutzfeldt Jakob Disease (prion disease) you must notify the on-call specialist and surgical leadership team prior to scheduling any surgical procedures.” The notification is pinned on the side panel of the suspected patient's electronic medical record, which remains easily viewable until a result is determined. This process allows for timely notification and helps reduce the potential for iatrogenic transmission. At the time of ordering, the laboratory also initiates its own CJD protocol, completing a tracking form, notifying management, and maintaining chain-of-custody documentation for all personnel handling the specimen. Cerebrospinal fluid samples are collected through standard precautions, including hand hygiene, gloves, eye protection, and disposable equipment. These specimens are double-bagged, labeled “possible CJD,” and promptly hand-delivered to the laboratory. During specimen processing, laboratory personnel follow enhanced precautions, wearing a disposable laboratory coat, gloves, and face/eye protection as samples are processed in a biological safety cabinet. After use, all personal protective equipment and materials are disposed of, and work surfaces are disinfected with a bleach-based solution. These IPC measures ensure early notification, containment, staff safety, and compliance with the specific required protocols.

Figure 2.

Institutional workflow for suspected or confirmed Creutzfeldt-Jakob disease (CJD). EMR, electronic medical record; IFU, instructions for use.

At our facility, reusable surgical instruments are reprocessed via steam or hydrogen peroxide gas plasma sterilization in accordance with the manufacturer's instructions for use. If a patient with suspected or confirmed CJD must have a procedure using non–single-use instruments, our policies dictate that instruments first be placed in a leak-proof biohazard container and, after a successful steam sterilization cycle, be sent for incineration per the policy of our medical waste vendor. It is preferred that reusable, non–single-use instruments be discarded. If instruments are to be reprocessed for reuse, approval must be obtained from sterile processing, infection prevention, and perioperative services leadership. Our flexible endoscopes undergo high-level disinfection (HLD) after use with a minimum concentration of 0.1% peracetic acid (Rapicide PA; Medivators, Inc) in an automatic endoscope reprocessor. Our IPC department was consulted to provide guidance, which identified an oversight in our existing policy regarding the use of reusable endoscopes for procedures involving potentially prion-contaminated tissue.

Case-Specific IPC Actions

Table 1 illustrates the clinical timeline detailing key events of this case, from symptom onset to confirmed CJD diagnosis and subsequent management decisions.

Table 1.

Clinical Course Timeline

Day	Clinical Event
0	Symptom onset: cognitive and behavioral changes
1	Hospital admission to neurology service with full evaluation; brain MRI ordered and completed; CSF testing ordered and collected (14-3-3 protein, RT-QuIC); low suspicion of CJD
4	“Rule-out CJD infection” banner added; EMR encounter alert activated
15	Confirmatory testing resulted; CJD diagnosis confirmed
17	Family meeting; transition to palliative care
21	Clinical team requested PEG tube placement; IPC team consulted; institutional risk assessment conducted
25	Decision made to defer use of endoscope; PEG placed percutaneously
80	Patient discharged to home with end-of-life care resources

Table 1 presents a timeline of key events in the clinical course, including symptom onset, diagnostic workup, infection prevention actions, and outcomes, to illustrate the rationale for clinical decision making and the overall process.

Abbreviations: CJD, Creutzfeldt-Jakob disease; CSF, cerebrospinal fluid; EMR, electronic medical record; IPC, infection prevention and control; MRI, magnetic resonance imaging; PEG, percutaneous endoscopic gastrostomy; RT-QuIC, real-time quaking-induced conversion.

In this case, the patient with confirmed sporadic CJD required PEG tube placement, which would ordinarily involve the use of a flexible endoscope. The infectivity risk associated with gastric tissue and flexible endoscopy in patients with sporadic CJD remains theoretical and is not well characterized. Gastric tissue is not considered a high-infectivity tissue for CJD, and there are no well-documented cases establishing transmission of sporadic CJD through flexible endoscopy. Therefore, the decision to avoid a reusable endoscope in this case was not based on documented transmission through gastric tissue or endoscopy but rather on the potential for prion contamination combined with uncertainty regarding the effectiveness of conventional reprocessing methods. This distinction is important, as the absence of documented transmission does not establish the absence of risk, particularly when the consequences of iatrogenic transmission would be severe.

However, peracetic acid at concentrations ranging from 0.2% to 19% has been shown to be ineffective in inactivating prions at room temperature, with efficacy demonstrated at only elevated temperatures (eg, 55 °C) [7]. Therefore, standard HLD protocols are considered insufficient for mitigating prion transmission risk in this case.

Our IPC department conducted literature reviews, consulted manufacturers, and the sterile processing department to gather evidence on the proper reprocessing of reusable endoscopes used in procedures for patients with suspected or confirmed CJD, recognizing that an HLD cycle would not be sufficient in eliminating prions. The safest option would be to use disposable, single-use flexible endoscopes for the procedure; however, these are not stocked by our facility due to their significant costs and low demand. We consulted with the manufacturer of our flexible endoscopes (Olympus Cooperation of America), which recommended that we submerge the endoscope for 1 hour in NaClO; 2% available chlorine prior to reprocessing normally and continue routine use [14]. Olympus conducted compatibility testing and determined that its scopes can withstand 1-hour immersions in NaClO up to 25 times without compromising their integrity [14].

Although manufacturer compatibility testing supports device integrity following NaClO exposure, this does not confirm prion inactivation. Given this and the differing literature and recommendations within vs outside the United States, we decided to maintain a conservative approach. The risk-based decision made was to have the PEG tube placed via interventional radiology (IR) without the use of an endoscope by performing the insertion percutaneously with all instrumentation being single use. IR-guided PEG placement was considered a clinically acceptable alternative to the standard because it allowed enteral access to be established without use of a reusable endoscope while maintaining the intended clinical goal of the procedure. The decision balanced patient-centered care with institutional infection prevention and safety obligations. Despite the patient's terminal prognosis, PEG placement was pursued in accordance with her previously expressed wishes and goals of care. The multidisciplinary team therefore modified the procedural approach to respect patient autonomy and avoid unnecessary delay while minimizing potential prion exposure and the associated uncertainty surrounding reprocessing of reusable equipment. IR guidance led to a successful procedure, and subsequently, all medical waste generated during the case was packaged per our medical waste vendor's policy and sent for incineration. After the procedure, the patient was discharged and followed by end-of-life care resources.

POLICY INSIGHTS AND RECOMMENDATIONS

In this case, performing the procedure without a reusable endoscope provided the most conservative approach to minimizing potential transmission risk while maintaining patient safety. This approach reflects the balance between acting cautiously under conditions of uncertainty and adhering to evidence-based infection prevention practices. Our organization is classified as a safety-net acute care hospital that provides medical care to low-income, uninsured, and otherwise vulnerable populations. The approach adopted within our facility demonstrates one feasible strategy for resource-conscious care. However, access to IR and single-use procedural equipment may be limited in low- and middle-income countries and other resource-constrained settings. As such, alternative mitigation strategies may be necessary and should be developed per available resources and institutional capabilities. Coordination with IR may introduce procedural delays, resource reallocation, and increased operational complexity. These trade-offs highlight the real-world impact of conservative IPC decision making in rare but high-risk scenarios.

Leading public health organizations should promote the use of single-use endoscopes in patients with suspected or confirmed CJD to prevent iatrogenic transmission. These agencies should revise their guidance to also provide clear, unified direction addressing surgical instrumentation and the management of endoscopes.

We recommend avoiding the use of reusable endoscopes in patients with suspected or confirmed CJD to minimize the potential for iatrogenic transmission. A hierarchical approach to procedural management is recommended. The preferred approach is to use a single-use endoscope that will be discarded after use. An acceptable alternative is IR-guided PEG placement without the use of an endoscope, when clinically feasible. As a last resort, if a reusable endoscope is used, the scope should be considered high risk and managed in consultation with infection prevention, sterile processing, and applicable manufacturer recommendations, with consideration given to prion-specific remediation or quarantine based on available guidance. Given the differing literature on cleaning, disinfection, and sterilization of medical equipment for patients with suspected or confirmed CJD and the fatal nature of the disease, it is better to adopt a precautionary stance.

LESSONS LEARNED

A key takeaway from this case is the importance of being operationally proactive in high-stakes, rare situations that require timely decision making. Novel situations such as this CJD case can drive innovation and better prepare health care systems for future cases requiring efficient solutions. This can be implemented by establishing a process that includes early notification of suspected CJD cases with clear, prompt communication with all stakeholders to ensure policy adherence and prevent iatrogenic transmission. Essential insights include effective interdepartmental communication in collaboration with exhausting all resources such as applicable manufacturers. This case was limited by its single-case nature, lack of prospective validation, absence of environmental contamination testing, uncertainty regarding true endoscopic transmission risk, and inability to empirically compare decontamination strategies. This case affirms the need for proactive institutional preparedness and clear decision making when scientific evidence gaps persist. Conservative IPC measures should be integrated into institutional policies across health care settings, ensuring safety without compromising patient care.