Creutzfeldt-Jakob Disease: All About The Rare Disorder That Causes Holes In Brain – News18


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Three individuals have been identified with Creutzfeldt-Jakob disease in Oregon’s Hood River County.

Creutzfeldt-Jakob disease (CJD) is a rare and rapidly progressing brain disorder belonging to the family of diseases known as prion diseases, which are also known as transmissible spongiform encephalopathies (TSEs). (Shutterstock)

In an alarming health crisis, two people have died of a rare, incurable brain disorder, Creutzfeldt-Jakob disease, within a few months of each other in Hood River County, a region in the northwestern state of Oregon. The deaths have been reported at a time when the county health officials have claimed to identify three cases of Creutzfeldt-Jakob disease in the past eight months.

As per The Oregonian, two of the three cases have resulted in death. While one of them was confirmed by autopsy, two others are presumptive diagnoses. It is yet to be known if the cases are linked; however, the risk to the public still remains “extremely low,” as the Hood River County Health Department monitors the situation. “We’re trying to look at any common risk factors that might link these cases. But it’s pretty hard in some cases to come up with what the real cause is,” Hood River County Health Department director Trish Elliot told the outlet.

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With the health crisis being monitored, here’s all you need to know about the rare brain disease.

What Is Creutzfeldt-Jakob Disease?

Creutzfeldt-Jakob disease (CJD) is a rare and rapidly progressing brain disorder belonging to the family of diseases known as prion diseases, which are also known as transmissible spongiform encephalopathies (TSEs).

According to the National Institute of Neurological Disorders and Stroke, spongiform refers to the way affected brains look, and with prion diseases, the brain is filled with holes and looks like a sponge when examined. CJD causes problems with muscle coordination, thinking, and memory. With about 350 cases reported every year in the US, CJD tends to progress rapidly, while about 70 per cent of people die within a year of getting the disease.

Causes Of Creutzfeldt-Jakob Disease

An individual can develop CJD in various ways, including sporadically, hereditarily, or through transmission.

While the majority of cases of CJD are sporadic, where the prion diseases may develop when a person’s normal prion protein suddenly turns abnormal, it can also be hereditary when the gene mutations affecting normal prion protein production run in families.

Creutzfeldt-Jakob Disease (CJD) is not spread through the air or casual contact. However, it can be transmitted through direct exposure to infected brain or nervous system tissue, particularly during certain medical procedures.

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Symptoms Of CJD

With cognitive decline being the main symptom of CJD, it often leads to dementia, involuntary muscle jerks, and a lack of coordination in movements. Among other early symptoms are poor coordination, walking and balance problems, speech difficulty, dizziness, insomnia, vision changes, hallucinations, and more. As the condition progresses, individuals can witness a few other symptoms like weakness of the arms and legs, blindness, inability to move or speak, problems swallowing, and coma.

Treatment Of CJD

There is currently no cure for the brain disorder; however, treatment can help relieve symptoms and make the affected person feel comfortable and at ease. At later stages, patients may need IV fluids and machine feeding.

News explainers Creutzfeldt-Jakob Disease: All About The Rare Disorder That Causes Holes In Brain



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