Barriers to Autopsy: Creutzfeldt-Jakob Disease in New York State

Abstract

Surveillance of Creutzfeldt-Jakob disease (CJD) monitors trends and ensures timely identification of variant CJD and other emergent prion diseases. Brain tissue is needed to definitively diagnose these diseases. A survey of neurologists and pathologists in New York State was conducted to understand neurologists’ and pathologists’ views on autopsy and CJD. Neurologists reported using autopsy rarely or never. Over half of the pathologists worked in facilities that did not perform autopsies when CJD was suspected. Barriers to autopsy included family reluctance, infection control concerns, and local facilities unable to perform brain autopsy. More accurate, complete recognition of CJD and variant forms depends on physician awareness of the manifestations of CJD and its diagnosis, access to pathologists and facilities willing and able to perform brain biopsies and autopsies, and family acceptance of such procedures.

Description and analysis of 12 years of surveillance for Creutzfeldt–Jakob disease in Denmark, 1997 to 2008

“…The number of autopsies performed on patients with a probable and possible diagnosis declined, leading to a lower number of definite diagnoses and a larger uncertainty in the surveillance. An American study showed that reluctance of the family to give consent was one of the most important barriers to performing an autopsy [36]. This does not, however, explain why the number of autopsies decreased over those 12 years.…”

Creutzfeldt-Jakob Disease in Austria: An Autopsy-Controlled Study

“…In Austria, definite TSEs are notifiable diseases since 1996, and postmortem analysis of brain tissue is mandatory in all suspect cases to establish the definite diagnosis. Thus, Austrian epidemiological data may serve as an autopsy-controlled baseline for comparison with countries that have a lower autopsy rate [19] .…”

Human prion diseases and the risk of their transmission during anatomical dissection

“…However, hospital autopsy rates have been declining in the UK (Ayoub and Chow, ), US (Hoyert, ), and Japan (Maeda et al, ) and are at an all‐time low (<6% of the US Hospital deaths). Concerns in the US about inadequate infection control and facilities to perform brain autopsy on suspected CJD patients have been suggested as factors discouraging autopsy (Lillquist et al, ). In practice within the UK this is not the case and autopsy rates for legal reasons are high, and for suspected cases of human prion disease are exemplary.…”