Creutzfeldt-Jakob Disease (CJD)

Creutzfeldt-Jakob disease (CJD) is a rare disease of the brain. People with CJD have rapid mental deterioration resulting in death. CJD occurs at a rate of 1-2 cases per 1,000,000 people per year worldwide. CJD almost always affects adults 50 years of age and older. In 2013, 478 CJD deaths were reported in the United States; four of those cases were from West Virginia. From 1990-2016, there were 36 CJD-related deaths in the state. CJD is not a reportable disease in West Virginia. Because the disease is 100% fatal the occurrence of CJD is tracked through death certificates.

Most cases of CJD occur for no apparent reason. These cases are called ‘sporadic’ or ‘spontaneous’ CJD. CJD can also be hereditary. 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. The two diseases are sometimes confused. 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. vCJD is even more rare than sporadic CJD with about 220 known cases to have occurred worldwide. There have been no cases of vCJD acquired in the United States and no cases of vCJD in West Virginia.

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