Laboratory tests have confirmed a diagnosis of variant CJD (a fatal brain disorder) in a patient who recently died in Texas. The confirmation was made when laboratory results from an autopsy of the patient’s brain tested positive for variant CJD, according to a Centers for Disease Control and Prevention news release today.

Image/CDC
Image/CDC

First described in 1996 in the United Kingdom, variant CJD is a rare, degenerative, fatal brain disorder in humans. It is believed to be caused by consumption of products from cows with the disease bovine spongiform encephalopathy (BSE, or “mad cow” disease).

Worldwide, more than 220 variant CJD patients have been reported, with a majority of them in the United Kingdom (177 cases) and France (27 cases). This case is the fourth to be reported in the United States. In each of the three previous cases, infection likely occurred outside the United States, including the United Kingdom (2 cases) and Saudi Arabia (1 case). The history of this fourth patient, including extensive travel to Europe and the Middle East, supports the likelihood that infection occurred outside the United States.

The last case confirmed in the US was in 2007  in a US resident in Virginia who was born and raised in Saudi Arabia and has lived in the United States since late 2005.

CDC assisted the Texas Department of State Health Services (DSHS)’s investigation of this case and will continue to help confirm further details of the patient’s history, including the potential source of infection. There are no Texas public health concerns or threats associated with this case.

A classic form of CJD, which is not caused by the BSE agent, occurs worldwide, including in the United States. Annually, for every 1 million people in the United States, 1 to 2 develops classic CJD. More information about variant CJD, including how it differs from classic CJD, is available in the Variant Creutzfeldt-Jakob Disease Fact Sheet.

Variant CJD  has different clinical and pathologic characteristics from classic CJD. Each disease also has a particular genetic profile of the prion protein gene.  The median age at death for vCJD patients is 28 years, compared with 68 years for patients with classic CJD. The median duration of illness for vCJD is 14 months, compared to 5 months for classic CJD.