Creutzfeldt-Jakob Disease (CJD)
Creutzfeldt-Jakob disease (CJD) is a rare disease of the brain cause by infectious proteins called prions. People with CJD have rapid mental deterioration resulting in death within one year of illness onset. The disease almost always affects adults 50 years of age and older. From 1990-2016, there were 36 CJD-related deaths in West Virginia.
Most cases (85%) of CJD cases are "sporadic," and about 5-15% of cases are hereditary, meaning that they inherited mutations of the prion protein gene. Very rarely, CJD has been transmitted from one person to another by a medical procedure such as a corneal transplant or a skin transplant.
Variant CJD (vCJD) is different from CJD. vCJD is linked to eating beef infected with "mad cow disease” or bovine spongioform encephalopathy (BSE). vCJD cases either lived in or traveled to countries where BSE was occurring in cattle. There have been no cases of vCJD acquired in the United States and no cases of vCJD in West Virginia.
CJD is reportable to the state health department within one week.
Age-adjusted death of CJD -- United States, 1979-2016. Rates are adjusted to the US standard 2000 projected population. Source: CDC.