Report Focuses on Two Cases of Sporadic CJD in the UK

Medscape Education recently launched a new activity, the focus of which is a report from the journal Emerging Infectious Diseases (EID) on surveillance as it relates to two unique cases of patients deceased and diagnosed with sporadic type Creutzfeldt-Jakob Disease (sCJD) in the United Kingdom (UK). sCJD is the most common type in a group of rare neurodegenerative diseases characterized by abnormal prion proteins that form sponge like holes in the brain tissue, resulting in death. sCJD arises in patients with no known risk factors for the disease.

The report in EID, which is published by the U.S. Centers for Disease Control and Prevention (CDC), looks at two deceased patients, both of whom had been receiving human-derived plasma products sourced and fractionated from UK-based donors for an extended period. One patient had hemophilia B and the other had von Willebrand Disease. Both individuals were women who died in 2014 at the age of 64, after suffering a steady neurological decline over the course of several months. After reviewing their patient history, the investigators noted that both individuals had clinical and investigative features “suggestive of sCJD,” a form of the disease not associated with the use of plasma-derived therapies. While these cases are unusual, the authors made sure to explain that no connection to the use of plasma products has been established.

“It is clearly of concern that there have been two such cases in a relatively short peri­od in the UK, where many plasma product recipients have been informed that they are at increased risk for vCJD, explained the authors. “However, a causal link between the treatment with plasma products and the onset of sCJD has not been established, and the occurrence of these cases may simply reflect a chance event in the context of systematic surveillance of CJD in large populations.”

No additional cases have been reported since the two in 2014. The authors concluded that continued monitoring of at-risk patient populations through existing surveillance programs such as those found in Europe is essential.

Access the full report synopsis on the CDC website.

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