Federal health officials say a patient in Texas is the fourth person in the United States to die of a mad cow-related disease.
The Centers for Disease Control and Prevention says in the three previous cases, infection likely occurred outside the U.S. and the Texas patient had traveled extensively to Europe and the Middle East.
The CDC says worldwide, more than 229 cases of variant Creutzfeldt-Jakob disease have been reported.
Most of them are in Great Britain and France.