Barriers to Creutzfeldt-Jakob Disease Autopsies, California

Creutzfeldt-Jakob disease (CJD) surveillance relies on autopsy and neuropathologic evaluation.The 1990-2000 CJD autopsy rate in California was 21%.Most neurologists were comfortable diagnosing CJD (83%), but few pathologists felt comfortable diagnosing CJD (35%) or performing autopsy (29%).Addressing obstacles to autopsy is necessary to improve CJD surveillance.T ransmissible spongiform encephalopathies (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 encephalopathy-infected cattle products in Europe (1).The recent identification of bovine spongiform encephalopathy in the United States 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 and the U.S. Centers for Disease Control and Prevention, has conducted enhanced surveillance for classic and variant CJD.Methods include review of state mortality data and followup investigation of CJD-related deaths that occur in persons <55 years of age, since >98% of cases of variant CJD in the United Kingdom have occurred in this age group.As part of this enhanced surveillance, medical records for 33 deceased California residents <55 years old from 1996 through 2003 have been investigated with criteria for CJD developed by the World Health Organization and Centers for Disease Control and Prevention; none met the criteria for variant CJD.