Creutzfeldt-Jakob Disease (CJD) is a rare, invariably fatal neurodegenerative disease caused by misfolded proteins called prions. The disease can originate in three different ways (sporadic, genetic and infectious) and has four forms (sporadic, variant, iatrogenic, and familial). Known infectious routes include ingestion of contaminated beef (mad cow disease or vCJD), and certain surgical procedures and contaminated surgical instruments (iCJD). CJD is a difficult disease to diagnose; ultimately requiring neuropathological examination of brain tissue for confirmation. Clinical history, MRI, electroencephalogram and cerebrospinal fluid testing assessing 14-3-3 and Tau proteins levels are all tools that are used to assess the likelihood of CJD. Unlike most reportable conditions, CJD can only be confirmed after death. Because of the complexity of the disease, surveillance and case investigation can be a challenge. The average rate of CJD in the US is 1 case per million. Historically, Texas has averaged 16 cases per year (0.7 cases per million). Last year, 2010, was an exception. Not only did Texas have over 25 cases, 7 of these cases had an uncharacteristic clinical course. This presentation will take a historical look at CJD surveillance in Texas from 2000 to 2010 and will highlight the distinctiveness of 2010.