Case of CJD Disease in New Hampshire

Bill Rutala

by Bill Rutala, Ph.D.

In September 2013, health officials confirmed that a patient who underwent neurosurgery at a New Hampshire hospital earlier in the year had Creutzfeldt-Jacob disease.  The death, and suspicions that the patient may have had the devastating brain ailment, prompted authorities in two states to warn that as many as 13 patients may have been exposed to surgical equipment used during the patient's surgery, thus to the same disease. The now-deceased patient had undergone neurosurgery at a New Hampshire hospital and the patient was later suspected of having sporadic Creutzfeldt-Jakob disease, a rare, rapidly progressing and always-fatal degenerative brain disease. But by the time this diagnosis was suspected, equipment used in the patient's surgery had been used several other operations. This raised the possibility that the equipment might have been contaminated -- especially since normal sterilization procedures are not enough to get rid of the disease proteins, known as prions, tied to Creutzfeldt-Jakob disease -- thus potentially exposing the other patients to infection (Botelho, CNN, September 2013).  This exposure scenario could happen in any hospital and this is why we must remain vigilant and implement practices that minimize its occurrence in our hospitals.

           Creutzfeldt-Jakob disease (CJD) is a degenerative neurologic disorder of humans with an incidence in the United States of approximately 1 case per million population per year. CJD is caused by a proteinaceous infectious agent, or prion.  Prion diseases elicit no immune response, result in a noninflammatory pathologic process confined to the central nervous system, have an incubation period of years, and usually are fatal within 1 year after diagnosis. 

Iatrogenic CJD has been rarely described in patients for whom contaminated medical equipment was used during intracranial placement of contaminated electroencephalography electrodes (2 cases in Switzerland) or neurosurgical procedures (4 suspected cases: 3 cases in the United Kingdom and 1 case in France).  Transmission to 2 patients via stereotactic electrodes is the only proven example of transmission by way of a medical device. None of the instruments used in these procedures were cleaned and sterilized using currently accepted methods for standard instrument reprocessing.  All known instances of iatrogenic CJD have resulted from exposure to infectious brain, pituitary, or eye tissue. The prions that cause CJD exhibit an unusual resistance to conventional chemical and physical decontamination methods. Because the CJD agent is not readily inactivated by means of conventional disinfection and sterilization procedures and because of the invariably fatal outcome of CJD, the procedures for disinfection and sterilization of the CJD prion have been both cautious and controversial for many years.

            The high resistance of prions to standard sterilization methods warrants special procedures in the reprocessing of surgical instruments. Special prion reprocessing is necessary when reprocessing critical or semicritical medical devices that have had contact with high-risk tissues (i.e., brain, spinal cord, pituitary tissue and eyes) from high-risk patients (e.g., known or suspected CJD, rapidly progressive dementia).  After the device is clean, it should be sterilized by either autoclaving (i.e., steam sterilization) or using a combination of sodium hydroxide and autoclaving using 1 of the 4 options below (Rutala WA, Weber DJ, ICHE 2010; 31:107-117).

• Option 1. Autoclave at 134^oC for 18 minutes in a prevacuum sterilizer.

• Option 2. Autoclave at 132^oC for 1 hour in a gravity displacement sterilizer.

• Option 3. Immerse in 1 N NaOH (1 N NaOH is a solution of 40 g NaOH in 1 L water) for 1 hour; remove and rinse in water, then transfer to an open pan and autoclave (121^oC gravity displacement sterilizer or 134^oC porous or prevacuum sterilizer) for 1 hour.

• Option 4. Immerse in 1 N NaOH for 1 hour and heat in a gravity displacement sterilizer at 121^oC for 30 minutes, then clean and subject to routine sterilization.

            To minimize the possibility of using neurosurgical instruments that potentially were contaminated during procedures performed on patients in whom CJD was later diagnosed, healthcare facilities should consider using the sterilization guidelines outlined above for neurosurgical instruments used during brain biopsy performed on patients in whom a specific lesion (e.g., a suspected tumor or abscess) has not been demonstrated (e.g., by magnetic resonance imaging, computed tomography scans, or positron emission scans). Alternatively, disposable neurosurgical instruments should be used for such patients, or the instruments could be quarantined until the pathologic findings of the brain biopsy are reviewed and the diagnosis of CJD is excluded. If disposable instruments are used, they should be of the same quality as reusable devices.