Described only in cases of direct contact with infected organs, tissues, or surgical instruments. Has been reported with: corneal transplants, intracerebral EEG electrodes sterilized with 70% alcohol and formaldehyde vapor after use on a CJD patient, operations in neurosurgical ORs after procedures on CJD patients, in recipients of pituitary-derived human growth hormone (hGH) (most cases have occurred in France; there is no longer a risk of CJD with growth hormone in the U.S. since distribution of pituitary derived hGH was halted in 1985 and current hGH is obtained from recombinant DNA technology), and dural graft with cadaveric dura mater (Lyodura®) (most cases have occurred in Japan). Ethylene oxide, autoclaving, formalin, and ionizing radiation do not inactivate the CJD agent2
Four patients who received dural grafts of cadaveric origin in the course of posterior fossa procedures subsequently developed Creutzfeldt-Jakob disease (CJD). The interval from dural placement to clinical onset of CJD ranged from 16 months to nine years. Initial clinical presentation consisted of cerebellar symptoms, with dementia and myoclonus developing in later stages of the disease. EEGs showed diffuse slowing that evolved to a periodic activity pattern. CT and MRI were unremarkable in the early stages but pronounced cerebral and cerebellar atrophy with widened sulci and collections of fluid over the convexities were seen in the late stages of disease. The diagnosis was histologically proved by brain biopsy in all four cases. Molecular genetic analysis showed that the four patients were homozygous for methionine at codon 129 of the PrP gene. From this experience, and from six previous descriptions of this occurrence in the literature, it is manifest that awareness of the means of iatrogenic transmission of CJD, and the adoption of preventive measures, constitute the only effective way to stop the spread of CJD among patients who have neurosurgery 1)
Fujioka H, Soejima Y, Izumihara A, Yamashita K. [A Case of Creutzfeldt-Jakob Disease before Trepanation Presenting as a Chronic Subdural Hematoma]. No Shinkei Geka. 2017 Nov;45(11):1011-1014. doi: 10.11477/mf.1436203637. Japanese. PubMed PMID: 29172208 2).
A 10-year-old boy underwent a posterior fossa craniectomy for removal of a grade 2 cerebellar astrocytoma. Dural closure was achieved by the placement of a dural graft. Eight years later the patient developed dementia and myoclonus. Electroencephalography demonstrated generalized slow activity that evolved into a pattern of periodic triphasic waves. Computed tomography scan and magnetic resonance imaging were unremarkable. Brain biopsy confirmed spongiform encephalopathy of the Creutzfeldt-Jakob type. In the light of previous reports of four similar occurrences, and of our own experience with two further cases of this disease, we believe that the cadaveric dura was the source of transmission of Creutzfeld-Jakob disease in our patient. The authors remark the importance of the awareness of this late complication of dural substitutes, both for the diagnosis of possible future cases and for taking preventive measures to stop the spread of the disease 3)