Ances Creutzfeldt-Jakob Disease Study

ICTS Just-In-Time Award

ICTS investigator Beau M. Ances, MD, PhD, Assistant Professor of Neurology at Washington University in St. Louis, received an ICTS Just-In-Time (JIT) core usage funding award in 2009. Dr. Ances’ JIT award, co-funded by the ICTS and the Mallinckrodt Institute of Radiology, supported services in the Human Imaging Unit to study Creutzfeldt-Jakob Disease (CJD), a rapidly progressive dementia (RPD). Their results confirmed that cerebrospinal fluid abnormalities and magnetic resonance imaging (MRI) can assist in distinguishing CJD patients from non-prion RPD patients, calling for future longitudinal studies to evaluate pathological changes seen in CJD patients. Dr. Ances was the senior author of the resulting publication [PMCID PMC3651672] in the Journal of Neurology.

In the fall of 2012, Dr. Ances also hosted a symposium at Washington University on the “Evaluation of CJD & other Rapidly Progressive Dementias.” The symposium focused on the diagnosis of RPDs, and in particular, on the basic biology and new diagnostic methods for identification. One speaker whose husband had been diagnosed with CJD also talked about her experiences.

Dr. Ances summarized the impact of this work and his collaboration with the ICTS:

“The JIT award has been instrumental in getting our research off the ground. It has led to our characterization of patients with RPDs, and has allowed us to provide additional information to families with loved ones who are diagnosed. In addition, our work has led our site to be recognized by the CJD Foundation as a site that can assist families who are concerned about CJD. We have had an increase in the number of referrals both internally and externally due to the 2012 conference.

While we do not have cure for CJD, we are constantly working on new methods for early diagnosis of CJD (and other RPDs). The JIT award and conference have helped us spread the word concerning this disorder. Barnes-Jewish Hospital has the necessary resources (i.e. MRI scanners and ability to perform lumbar puncture for cerebrospinal fluid) to help make a probable diagnosis of CJD. In addition, we can also provide autopsies that are coordinated with the National Prion Center and help give a more complete closure to families who are unsure about the final diagnosis of a loved one.”

Dr. Ances also noted that CJD is an orphan disease and few groups study this disorder as it affects roughly one in a million people. He and his team hope to apply to the CJD Foundation in the near future to build on their previous research.

Dr. Ances was also the senior author of “one of the top articles in Alzheimer disease in the Annals of Neurology in 2012” – "Alzheimer disease family history impacts resting state functional connectivity" [PMCID PMC3490438]