Special Needs Populations: Perioperative Care of the Patient With Creutzfeldt-Jakob Disease

Abstract

Creutzfeldt-Jakob disease (CJD) is a rare but lethal prion disease. The worldwide mortality rate of CJD is 1.67 per one million people, although 90% of cases will lead to death within one year of symptom onset. Rapid, progressive dementia is the cardinal sign of CJD. Other early symptoms include deterioration of muscle coordination, memory, judgment, and vision, as well as personality changes, insomnia, and depression. It occurs in two types, classic and new variant, and classic CJD can be further divided into three subtypes: sporadic, familial (ie, genetic), and iatrogenic (ie, health care associated). Surgery often requires using instruments that come in contact with high-infectivity tissue, especially during neurosurgical procedures and diagnostic biopsies. To reduce the risk of disease transmission, infection control practices should include identification of the risk of infection, implementation of safety protocols (eg, use of personal protective equipment), proper selection and use of instrumentation, and adequate disinfection and sterilization practices. Silent carriers, risks of iatrogenic infection, and the possibility of inadvertent exposure of healthy patients may carry great liability to public health and hospital stability.