Health officials in Hood River County of Oregon are now investigating the three suspected cases of a rare and a fatal brain disorder. It is called the Creutzfeldt-Jakob disease (CJD). What is even more concerning is that two residents of the small county have already been reported dead and a third case is currently under review. While CJD is extremely rare, with a cluster of cases in the community of just 24,000 which prompt its serious concern, a deeper look at this mysterious disease could help people understand more about it.What Is Creutzfeldt-Jakob disease (CJD)?Before we delve into how it happens or what causes, let us first understand what is it. CJD is degenerative brain disorder that is caused by abnormal or infectious proteins, also known as prions. Unlike bacteria or viruses, prions are misfolded protein which can induce normal protein in the bodies to misfold itself too. It can also lead to severe and irreversible brain damage. These proteins accumulate and cause the brain to develop sponge-like holes, which disrupt normal function and lead to a rapid mental and physical decline.As per the Centers for Disease Control and Prevention (CDC), most patients diet within a year of symptom onset and there is currently no cure of that same.Is The Disease Common?We have already established the fact that it is a rare disease. In fact, as per the National Institutes of Health (NIH) only 350 cases of CJD is diagnosed in the US each year. This translates into 1 to 2 cases per million people, annually. Most cases—about 85%—are considered sporadic, meaning they arise without any known cause or risk factors.Other types include:Familial CJD (about 10–15% of cases), caused by inherited mutations in the prion protein gene.Acquired CJD, which is extremely rare, can result from exposure to infected brain or nervous system tissue, often during certain medical procedures or, in even rarer cases, through the consumption of infected cattle (known as variant CJD).Health officials in Hood River have stated that the current cases do not appear to be linked to cattle consumption, and it’s unclear whether the individuals were related or had any shared genetic factors.What Are The Common Symptoms Of CJD?CJD progresses rapidly. According to the World Health Organization (WHO) and CDC, early symptoms can include:Rapid memory loss and confusionChanges in behavior and personalityVisual disturbancesPoor coordination and balanceMuscle stiffness or jerkingDifficulty speaking or swallowingAs the disease advances, patients often lose the ability to move or speak and may enter a coma. The rapid progression makes early diagnosis difficult and complicates treatment, especially in communities with limited access to specialized neurological care.How Can It Be Diagnosed?In the Hood River cases, one of the diagnoses was done through an autopsy. This is also one of the most definitive way to identify CJD by analyzing the brain tissue under a microscope. Testing it for prion proteins then reveals the result. Two other cases are considered likely, however, the results are pending.Because CJD is so difficult to confirm during life, doctors often rely on clinical symptoms, advanced imaging like MRI, and spinal fluid tests to support a diagnosis. But confirmation usually requires a postmortem examination.The National Prion Disease Pathology Surveillance Center at Case Western Reserve University plays a key role in diagnosing and studying cases in the U.S. Dr. Brian Appleby, the center’s director, notes that genetic testing can also help determine whether a case is inherited.
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