Barriers to Creutzfeldt-Jakob disease autopsies, California.Creutzfeldt-Jakob disease Creutzfeldt-Jakob disease: see prion. Creutzfeldt-Jakob disease or CJD Rare fatal disease of the central nervous system. It destroys brain tissue, making it spongy and causing progressive loss of mental functioning and motor control. (CJD CJD abbr. Creutzfeldt-Jakob disease CJD Creutzfeldt-Jakob disease, see there ) surveillance relies on autopsy and neuropathologic evaluation. The 1990-2000 CJD autopsy rate in California was 21%. Most neurologists This is a list of the most important neurologists, with their dates of birth and death and nationality.
********** Transmissible spongiform encephalopathies Transmissible spongiform encephalopathies (TSEs, also known as prion diseases) are a group of progressive conditions that affect the brain and nervous system of humans and animals and are transmitted by prions. (TSEs) are rare, progressively fatal, neurodegenerative illnesses. Human TSEs include classic Creutzfeldt-Jakob disease (CJD) and the recently described variant CJD associated with eating bovine spongiform spongiform /spon·gi·form/ (spun´ji-form) resembling a sponge. spon·gi·form adj. Resembling a sponge, as in appearance or porosity. spongiform resembling a sponge. encephalopathy--infected cattle products in Europe (1). The recent identification of bovine spongiform encephalopathy bovine spongiform encephalopathy: see prion. in the United States United States, officially United States of America, republic (2005 est. pop. 295,734,000), 3,539,227 sq mi (9,166,598 sq km), North America. The United States is the world's third largest country in population and the fourth largest country in area. underscores the importance of maintaining enhanced surveillance to monitor for the possible occurrence of variant CJD in this country (2,3). In California, CJD is not reportable. Since 1999, the California CJD Surveillance Project of the California Emerging Infections Program, a collaboration of the California Department of Health Services Department of Health Services may refer to:
Currently, pathologic review of brain tissue obtained by biopsy or autopsy is the only means of confirming a diagnosis of CJD. Autopsy remains the preferred method for obtaining tissue, as brain biopsy Brain Biopsy Definition A brain biopsy is the removal of a small piece of brain tissue for the diagnosis of abnormalities of the brain, such as Alzheimer's disease, tumors, infection, or inflammation. can result in serious complications (e.g., brain hemorrhage hemorrhage (hĕm`ərĭj), escape of blood from the circulation (arteries, veins, capillaries) to the internal or external tissues. The term is usually applied to a loss of blood that is copious enough to threaten health or life. or abscess abscess, localized inflamation associated with tissue necrosis. Abscesses are characterized by inflamation, which is due to the accumulation of pus in the local tissues, and often painful swelling. formation) and may not yield adequate amounts of tissue for analysis. The main role of brain biopsy is to exclude other, potentially treatable conditions (4). In this article, we describe results from analysis of California mortality data from 1990 through 2000. We also summarize responses generated from a statewide survey of neurologists and pathologists regarding the challenges to diagnosing CJD and variant CJD, including obtaining autopsy in suspected cases. The Study Data from the 1990-2000 Death Public Use File (underlying cause of death only) and 1990-1999 Multiple Cause-of-Death Data (underlying or contributing causes of death) were obtained from the Center for Health Statistics, California Department of Health Services (5). Deaths among California residents with an International Classification of Diseases, 9th Revision, code 046.1 or 10th Revision, code A81.0 listed anywhere on the death record were included in our analysis. Both data files included report of autopsy as a variable, with the exception of the Multiple Cause-of-Death Data for 1997 to 1999, when autopsy performance was not recorded. Statistical analysis was performed by using SAS (1) (SAS Institute Inc., Cary, NC, www.sas.com) A software company that specializes in data warehousing and decision support software based on the SAS System. Founded in 1976, SAS is one of the world's largest privately held software companies. See SAS System. software (SAS Institute SAS Institute Inc., headquartered in Cary, North Carolina, USA, has been a major producer of software since it was founded in 1976 by Anthony Barr, James Goodnight, John Sall and Jane Helwig. , Cary, NC). From July to December 2002, questionnaires regarding experience with diagnosing CJD were sent to 1,241 California neurologists identified as members of the American Academy of Neurology The American Academy of Neurology (AAN) is a professional society for neurologists and neuroscientists. As a medical specialty society it was established in 1949 by A.B. Baker of the University of Minnesota to advance the art and science of neurology, and thereby promote the best and 574 pathologists identified as members of the California Society of Pathologists and the American Association of Neuropathologists American Association of Neuropathologists, Inc. was established in the 1930's as a professional and educational organization representing American neuropathologists. It was incorporated in the State of Pennsylvania in May 1960. . Approval was obtained from the Committee for the Protection of Human Subjects of the State of California. Review of mortality data identified 263 CJD-related deaths in California from 1990 through 2000. Of these, 244 were identified from the 19901999 Multiple Cause-of-Death Data, and an additional 19 deaths were identified from the 1990 2000 Death Public Use File. A total of 42 (16%) cases identified by the Multiple Cause-of-Death Data were not detected in the Death Public Use File. Overall, 26 (10%) of the 263 CJD-related deaths were in persons <55 years of age. Only two deaths occurred in persons <30 years of age. The overall autopsy rate, which for 1997 to 2000 only includes autopsies performed on persons for whom CJD was recorded as the underlying cause of death, was 53 (21%) of 251 persons: 11 (44%) of 25 persons <55 years of age, and 42 (19%) of 226 persons [greater than or equal to] 55 years of age. For two deaths, autopsy performance was not recorded. Of 1,241 questionnaires mailed to neurologists, 428 (34%) were completed, including 310 (25%) from respondents involved in patient care. Responses regarding the neurologists' experience with diagnosing CJD and performing autopsy are summarized in Tables 1 and 2. Most neurologists (83%, 255/307) felt comfortable clinically recognizing classic CJD. More than one third (36%, 74/207) had not considered arranging for autopsy in their CJD patients, although most reported access to histopathologic services (75%, 223/297). The most commonly cited barrier to obtaining autopsy was family reluctance to give consent (79%, 192/242). Of 574 questionnaires mailed to pathologists, 284 (49%) were completed. Tables 1 and 2 summarize the responses. Thirty-five percent (96/273) and 15% (40/274) of pathologists were comfortable recognizing the neuropathologic features of classic CJD and variant CJD, respectively. Infection control concerns (77%, 143/185), lack of experience (62%, 69/111), and institutional limitations (53%, 111/210) were cited as major obstacles to autopsy performance, and less than half of respondents reported that confirming the diagnosis of CJD (47%, 92/197) or ruling out variant CJD (45%, 87/193) was an important reason to pursue autopsy. Conclusions Our analysis suggests that autopsy rates for CJD in California are low. The results of our surveys, which attempted to discern the reasons for this low rate, imply that both neurologists and pathologists have similar perceptions of the value of obtaining histopathologic evaluation for CJD but for different reasons. Most neurologists appeared to be comfortable clinically diagnosing CJD, with more than one third reporting they had never considered pursuing autopsy for CJD cases. In contrast, pathologists appeared to be less comfortable making a histopathologic diagnosis, indicating that autopsy performance was limited by infection control concerns, lack of experience with CJD cases, and institutional restrictions. Our results have some limitations. Approximately 10% of CJD cases may have atypical atypical /atyp·i·cal/ (-i-k'l) irregular; not conformable to the type; in microbiology, applied specifically to strains of unusual type. a·typ·i·cal adj. signs and symptoms that can obscure the diagnosis. To the extent that these cases are misdiagnosed and not autopsied, they could contribute to overestimation o·ver·es·ti·mate tr.v. o·ver·es·ti·mat·ed, o·ver·es·ti·mat·ing, o·ver·es·ti·mates 1. To estimate too highly. 2. To esteem too greatly. of the autopsy rate. On the other hand, death certificate analysis can be an insensitive indicator of the true rate of autopsy, and autopsy performance information was unavailable for 1997 to 2000 from the Multiple Cause-of-Death Data. Both factors could lead to possible underestimation of the true autopsy rate. Given that some CJD cases will have had confirmatory brain biopsy or strongly suggestive clinical features and diagnostic studies, the autopsy rates cited may apply mostly to patients for whom a satisfactory antemortem antemortem /an·te·mor·tem/ (an?te-mor´tem) [L.] occurring before death. an·te·mor·tem adj. Before death. antemortem performed or occurring before death. diagnosis could not be made. Interpreting survey results is limited by the low response rate; neurologists and pathologists who are experienced in diagnosing CJD may be more likely to respond, which would introduce bias. The public health benefits of performing autopsy on patients with suspected CJD should not be underestimated. Autopsy and histopathologic analysis remain important ways to confirm a diagnosis of CJD and help define the usual occurrence of subtypes of classic CJD, thereby facilitating the recognition of emerging TSEs (1,6,7). Autopsy rates for nonforensic deaths have declined dramatically during the past 40 years, with national hospital rates currently <5%, possibly resulting in missed diagnoses of the actual cause of death in 8% to 25% of cases (8-11). The reasons for the decline are multifaceted mul·ti·fac·et·ed adj. Having many facets or aspects. See Synonyms at versatile. Adj. 1. multifaceted - having many aspects; "a many-sided subject"; "a multifaceted undertaking"; "multifarious interests"; "the multifarious and include escalating cost of autopsy borne by hospitals and county medical examiners A public official charged with investigating all sudden, suspicious, unexplained, or unnatural deaths within the area of his or her appointed jurisdiction. A medical examiner differs from a Coroner in that a medical examiner is a physician. , lack of direct reimbursement Reimbursement Payment made to someone for out-of-pocket expenses has incurred. , fear of litigation An action brought in court to enforce a particular right. The act or process of bringing a lawsuit in and of itself; a judicial contest; any dispute. When a person begins a civil lawsuit, the person enters into a process called litigation. , and increasing reliance on modern technology to determine a diagnosis antemortem (10). Our survey results suggest that infection control concerns play a role in low autopsy rates for CJD, whether because of fears about the risk of acquiring CJD from handling contaminated contaminated, v 1. made radioactive by the addition of small quantities of radioactive material. 2. made contaminated by adding infective or radiographic materials. 3. an infective surface or object. tissue or because of liability considerations at the institutional level. More realistically, brain autopsy can be performed safely as long as CJD-specific infection control guidelines are strictly followed (12-13). Nonetheless, concerns about potentially acquiring CJD through autopsy procedures should be acknowledged and recognized as an opportunity to address proper infection control techniques. Enhancing surveillance for variant CJD and other emerging prion diseases Prion disease Transmissible spongiform encephalopathies in both humans and animals. Scrapie is the most common form in animals, while in humans the most prevalent form is Creutzfeldt-Jakob disease. will require educating neurologists and pathologists, addressing the perceived obstacles to obtaining autopsy, and encouraging the use of available resources that provide expertise and technical assistance in evaluating CJD. For example, brain tissue can be submitted to the National Priori Disease Pathology Surveillance Center (NPDPSC NPDPSC National Prion Disease Pathology Surveillance Center (Case Western Reserve University, Cleveland, OH) ) in Cleveland, Ohio "Cleveland" redirects here. For the Cleveland metropolitan area, see . For other uses, see Cleveland (disambiguation). Cleveland is a city in the U.S. state of Ohio and the county seat of Cuyahoga County, the most populous county in the state. , for free state-of-the-art diagnostic testing Diagnostic testing Testing performed to determine if someone is affected with a particular disease. Mentioned in: Von Willebrand Disease (14). The availability of a national center of expertise may facilitate obtaining tissue evaluation; since the inception of NPDPSC, the number of referrals to the facility has more than doubled, from 104 in 1997 to 265 in 2002, and the number of TSE See Tokyo Stock Exchange. TSE 1. See Tokyo Stock Exchange (TSE). 2. See Toronto Stock Exchange (TSE). cases confirmed from those referrals increased from 60 in 1997 to 151 in 2002 (14). Regional academic institutions, such as the University of California, San Francisco , Memory and Aging The references in this article would be clearer with a different and/or consistent style of citation, footnoting or external linking. One of the key concerns of older adults is the experience of memory loss, especially as it is one of the hallmark symptoms of Center, can also provide expertise and assistance with diagnostic testing. Such resources are vital to maintaining vigilance for cases of CJD and potentially emerging human TSEs, such as variant CJD or possibly a human form of chronic wasting disease Noun 1. chronic wasting disease - a wildlife disease (akin to bovine spongiform encephalitis) that affects deer and elk animal disease - a disease that typically does not affect human beings in the United States.
Table 1. Knowledge and experience of California neurologists,
pathologists, and neuropathologists in diagnosing
Creutzfeldt-Jakob disease (CJD)
Neurologists
Characteristic n/N (%)
Have evaluated a case of CJD 212/310 (68)
Median no. (range) of CJD cases evaluated 3 (0-30)
Type of practice
Private practice/private hospital 144/308 (47)
Outpatient HMO (a)/managed care 55/308 (18)
Community hospital/clinic 1/308 (<1)
University affiliated 82/308 (27)
Veterans hospital 13/308 (4)
County medical examiner or coroner --
Other 15/308 (5)
Can recognize the clinical or pathologic features of 255/307 (83)
classic CJD
Can recognize the clinical or pathologic features of 120/305 (39)
variant CJD
Have not considered arranging for an autopsy for CJD 74/207 (36)
patients under their care
Pathology group available at facility to perform 223/297 (75)
autopsy on suspect CJD cases
Pathology group available at facility to confirm 223/297 (75)
diagnosis of suspect CJD with histopathologic analysis
Pathologists
Characteristic n/N (%)
Have evaluated a case of CJD 56/259 (22)
Median no. (range) of CJD cases evaluated 2 (0-30)
Type of practice
Private practice/private hospital 122/278 (44)
Outpatient HMO (a)/managed care --
Community hospital/clinic 68/278 (24)
University affiliated 37/278 (13)
Veterans hospital 3/278 (1)
County medical examiner or coroner 7/278 (3)
Other 41/278 (15)
Can recognize the clinical or pathologic features of 96/273 (35)
classic CJD
Can recognize the clinical or pathologic features of 40/274 (15)
variant CJD
Have not considered arranging for an autopsy for CJD --
patients under their care
Pathology group available at facility to perform 74/259(29)
autopsy on suspect CJD cases
Pathology group available at facility to confirm 91/254(36)
diagnosis of suspect CJD with histopathologic analysis
Neuropatho-
logists
Characteristic n/N (%)
Have evaluated a case of CJD 18/33 (55)
Median no. (range) of CJD cases evaluated 10 (0-50)
Type of practice
Private practice/private hospital 8/33 (25)
Outpatient HMO (a)/managed care --
Community hospital/clinic 4/33 (12)
University affiliated 10/33 (30)
Veterans hospital 1/33 (3)
County medical examiner or coroner 2/33 (6)
Other 5/33 (15)
Can recognize the clinical or pathologic features of 25/28 (89)
classic CJD
Can recognize the clinical or pathologic features of 18/28 (64)
variant CJD
Have not considered arranging for an autopsy for CJD --
patients under their care
Pathology group available at facility to perform 17/28(61)
autopsy on suspect CJD cases
Pathology group available at facility to confirm 18/27(67)
diagnosis of suspect CJD with histopathologic analysis
(a) HMO, health maintenance organization.
Table 2. Perceptions of California neurologists, pathologists, and
neuropathologists regarding performance of autopsy in
Creutzfeldt-Jakob disease (CJD)
Neurologists
Characteristic n/N (%)
Important reasons to obtain autopsy for CJD patients
Autopsy is needed to confirm CJD diagnosis --
Autopsy is needed to rule out variant CJD or other 168/231 (73)
TSE (a) forms
Barriers to performing autopsy and histopathologic
analysis for CJD
Clinicians do not feel autopsy is required for 94/221 (43)
diagnosis
Facilities not able/willing to perform autopsies on 75/234 (32)
CJD patients
Families are reluctant to give consent for autopsy 192/242 (79)
Cost of autopsy is a concern to patient's family 113/234 (48)
Cost of autopsy is a concern to hospital/institution 78/234 (34)
Infection control is a concern regarding autopsy 102/235 (44)
Facilities are inadequate to perform autopsy --
Infection control is a concern regarding --
histopathologic evaluation
No available pathologists experienced in recognizing --
histopathologic features of CJD
Pathologists
Characteristic n/N (%)
Important reasons to obtain autopsy for CJD patients
Autopsy is needed to confirm CJD diagnosis 92/197 (47)
Autopsy is needed to rule out variant CJD or other 87/193 (45)
TSE (a) forms
Barriers to performing autopsy and histopathologic
analysis for CJD
Clinicians do not feel autopsy is required for 72/198 (36)
diagnosis
Facilities not able/willing to perform autopsies on 111/210 (53)
CJD patients
Families are reluctant to give consent for autopsy 57/202 (28)
Cost of autopsy is a concern to patient's family 34/202 (17)
Cost of autopsy is a concern to hospital/institution 40/199 (20)
Infection control is a concern regarding autopsy 143/185 (77)
Facilities are inadequate to perform autopsy 24/185 (13)
Infection control is a concern regarding 62/111 (56)
histopathologic evaluation
No available pathologists experienced in recognizing 69/111 (62)
histopathologic features of CJD
Neuropatho-
logists
Characteristic n/N (%)
Important reasons to obtain autopsy for CJD patients
Autopsy is needed to confirm CJD diagnosis 11/21 (52)
Autopsy is needed to rule out variant CJD or other 12/20 (60)
TSE (a) forms
Barriers to performing autopsy and histopathologic
analysis for CJD
Clinicians do not feel autopsy is required for 7/21 (33)
diagnosis
Facilities not able/willing to perform autopsies on 8/22 (36)
CJD patients
Families are reluctant to give consent for autopsy 6/22 (27)
Cost of autopsy is a concern to patient's family 8/20 (40)
Cost of autopsy is a concern to hospital/institution 8/21 (38)
Infection control is a concern regarding autopsy 9/11 (82)
Facilities are inadequate to perform autopsy 5/11 (45)
Infection control is a concern regarding 4/8 (50)
histopathologic evaluation
No available pathologists experienced in recognizing 1/8 (13)
histopathologic features of CJD
(a) TSE, transmissible spongiform encephalopathy.
Acknowledgments We gratefully acknowledge Laura Dalla Betta, Stephen DeArmond, Michael Geschwind, Ryan Maddox, Jennifer Martindale, Bruce Miller Bruce Miller is an American attorney born in 1945. He is known for arguing a legal case claiming welfare to be a constitutional right. Early life Miller was born in 1945 in California, where he spent his formative years. , Gretchen Rothrock, James Sejvar, and Mark Starr for their invaluable advice and support. References (1.) Belay ED be·lay v. be·layed, be·lay·ing, be·lays v.tr. 1. Nautical To secure or make fast (a rope, for example) by winding on a cleat or pin. 2. , Maddox RA, Gambetti P, Schonberger LB. Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States. Neurology neurology (n  rŏl`əjē, ny–), study of the morphology, physiology, and pathology of the human nervous system. . 2003;60:176-81.(2.) Tan L, Williams MA, Khan MK, Champion HC, Nielsen NH. Risk of transmission of bovine spongiform encephalopathy to humans in the United States. JAMA JAMA abbr. Journal of the American Medical Association . 1999;281:2330-9. (3.) Centers for Disease Control and Prevention. Preliminary investigation suggests BSE-infected cow in Washington state was likely imported from Canada [monograph on the Internet]. 2003 Dec 29 [cited 2004 Jul 10]. Available from: http://www.cdc.gov/ncidod/diseases/cjd/bse_washington.htm (4.) Will RB, Alpers MP, Dormont D, Schonberger LB, Tateishi J. Infectious and sporadic prion diseases. In: Prusiner SB, editor. Prion prion (prī`ŏn), infectious agent thought to cause a group of diseases known as prion diseases or transmissible spongiform encephalopathies. biology and disease. Cold Spring Harbor (NY): Cold Spring Harbor Laboratory  The Cold Spring Harbor Laboratory Press; 1999. p. 465-507.(5.) Center for Health Statistics. California's death public use tape documentation. Sacramento (CA): California Department of Health Services; 1997. (6.) U.K. Creutzfeldt-Jakob Disease Surveillance Unit. Investigations undertaken in possible CJD cases [monograph on the Internet]. 2002 Oct 15 [cited 2004 Jul 10]. Available from: http://www.cjd. ed.ac.uk/investigations.htm (7.) Fatal degenerative de·gen·er·a·tive adj. Of, relating to, causing, or characterized by degeneration. Degenerative Degenerative disorders involve progressive impairment of both the structure and function of part of the body. neurologic neurologic /neu·ro·log·ic/ (-loj´ik) pertaining to neurology or to the nervous system. Neurologic Having to do with the nervous system. illnesses in men who participated in wild game feasts--Wisconsin, 2002. MMWR MMWR Morbidity & Mortality Weekly Report Epidemiology A news bulletin published by the CDC, which provides epidemiologic data–eg, statistics on the incidence of AIDS, rabies, rubella, STDs and other communicable diseases, causes of mortality–eg, Morb Mortal Wkly Rep. 2003;52:125-7. (8.) Burton EC. The autopsy: a professional responsibility in assuring quality of care. Am J Med Qual. 2002;17:56-60. (9.) The autopsy as an outcome and performance measure [monograph on the Internet]. File inventory, evidence report/technology assessment no. 58. AHRQ AHRQ, n.pr See Agency for Healthcare Research and Quality. publication no. 03-E002. Rockville (MD): Agency for Healthcare Research and Quality Agency for Healthcare Research and Quality, n.pr formerly known as the Agency for Health Care Policy and Research, this agency researches the quality of medical care and health services. ; 2002 Oct [cited 2004 Jul 10]. Available from: http://www.ahrq.gov/clinic/autopinv.htm (10.) Brooks JP, Dempsey J. How can hospital autopsy rates be increased? Arch Pathol Lab Med. 1991;115:1107-11 (11.) Hasson J, Schneiderman H. Autopsy training programs: to right a wrong. Arch Pathol Lab Med. 1995;119:289-91. (12.) WHO infection control guidelines for transmissible spongiform encephalopathies: report of a WHO consultation [monograph on the Internet]. Geneva Geneva, canton and city, Switzerland Geneva (jənē`və), Fr. Genève, canton (1990 pop. 373,019), 109 sq mi (282 sq km), SW Switzerland, surrounding the southwest tip of the Lake of Geneva. : World Health Organization; 1999 Mar 26 [cited 21104 Jul 10]. Available from: http://www.who.int/emcdocuments/tse/whocdscsraph2003c.html (13.) Questions and answers regarding Creutzfeldt-Jakob disease infection control practices [monograph on the Internet]. Atlanta; Centers for Disease Control and Prevention: 2003 May 21 [cited 2004 Ju1 10]. Available from: http://www.cdc.gov/ncidod/diseases/cjd/cjd_inf_ctrl_qa.htm (14.) National Prion Disease Pathology Surveillance Center [homepage on the Internet], [cited 2004 Jul 10]. Available from: www.cjdsurveillance.com Dr. Louie served as project clinician clinician /cli·ni·cian/ (kli-nish´in) an expert clinical physician and teacher. cli·ni·cian n. for the California Creutzfeldt-Jakob Disease Surveillance Project, a joint collaborative project of the California Department of Health Services and the Centers for Disease Control and Prevention. Her research interests include the study of emerging infectious diseases An emerging infectious disease (EID) is an infectious disease whose incidence has increased in the past 20 years and threatens to increase in the near future. EIDs include diseases caused by a newly identified microorganism or newly identified strain of a known microorganism (e.g. . Janice K. Louie, * ([dagger]) Shilpa S. Gavali, * Ermias D. Belay, ([double dagger double dagger n. A reference mark (  ) used in printing and writing. Also called diesis.Noun 1. ]) Rosalie Trevejo, ([dagger]) Lucinda H. Hammond, * Lawrence B. Schonberger, ([double dagger]) and Duc J. Vugia * ([dagger]) * California Emerging Infections Program, Richmond, California, USA; ([dagger]) California Department of Health Services, Berkeley, California Berkeley is a city on the east shore of San Francisco Bay in Northern California, in the United States. Its neighbors to the south are the cities of Oakland and Emeryville. To the north is the city of Albany and the unincorporated community of Kensington. , USA; and ([double dagger]) Centers for Disease Control and Prevention, Atlanta, Georgia, USA Address for correspondence: Janice K. Louie, California Emerging Infections Program, California Department of Health Services, 2151 Berkeley Way, Room 716, Berkeley, CA 94704, USA; fax: 510-883-6015; email: jlouie@dhs.ca.gov |
|
||||||||||||||||||||
Printer friendly
Cite/link
Email
Feedback
Reader Opinion