Biomarker found for human form of mad cow disease

Creutzfeldt-Jakob disease (sCJD), or the human form of mad cow disease, is just as frightening as both names sound. It's a degenerative brain disease that's always fatal within a year of onset and has no cure. And, if that isn't grim enough, there is currently no way to even diagnose the disease until after brain tissue is obtained by biopsy after death. 

Enter Neena Singh and colleagues at Case Western Reserve University School of Medicine. They think they've found a basis for developing a test to diagnosis sCJD while patients are still alive. Singh and her team found that levels of the iron-transport protein transferrin (Tf) are significantly decreased in the cerebrospinal fluid of patients with sCJD well before the end stage of the disease, potentially allowing for earlier diagnosis.

"The decrease in Tf is significant enough to distinguish sCJD from dementia of non-CJD origin with an accuracy of 80 percent," Singh said in a release. "When combined with the currently used non-disease-specific biomarker T-tau, the diagnostic accuracy increases to 86 percent. This suggests that the two biomarkers represent separate disease processes, and complement each other as diagnostic biomarkers."

The ability to accurately diagnose patients while they are still living is necessary to prevent accidental spread of sCJD, reduce misdiagnosis of treatable causes of dementia and, Singh hopes, eventually develop therapies for sCJD.

- read the release from Case Western Reserve University