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What Is Creutzfeldt–Jakob Disease?

Creutzfeldt–Jakob Disease (CJD) is a rare neurodegenerative disorder that damages your brain. The disease is very rare, with an occurrence of about one to two cases in a million people per year. However, it’s rapidly progressive and fatal. It causes the affected person to die within a year of its onset.

The disease can be classified into two types – Classic Creutzfeldt–Jakob Disease and Variant Creutzfeldt–Jakob Disease.

CJD first became widely known in the 1990s when some people contracted a variant of this disease called variant CJD by consuming the meat of affected animals.

What Causes CJD?

Creutzfeldt–Jakob Disease is caused by the formation of an abnormal isoform of prion protein in the brain. This, in turn, causes the brain proteins to fold abnormally. CJD is the most common form of prion disease in humans.[1]

Theoretically, prion diseases can occur in three ways – genetic, acquired, and sporadic. However, as reported by the Centers for Disease Control and Prevention (CDC), CJD has been sporadic in a majority (about 85%) of identified cases.[2] Only a very small percentage of patients have been found to develop brain disease by inheriting the prion protein’s mutated genes or acquiring it by being exposed to infected human tissue.

Symptoms of the CJDisease

Creutzfeldt–Jakob Disease is primarily characterized by the deterioration of mental capabilities. Since the disease progresses rapidly, patients begin to experience visible signs and symptoms of mental deterioration and brain damage within a few months. However, the earliest signs and symptoms of the CJ disease are similar to some other diseases and can be confused with dementia. Therefore, proper diagnosis is essential.

Some of the early signs and symptoms of the CJ disease include:

· Anxiety

· Depression

· Impaired thinking

· Memory loss

· Personality changes

· Difficulty in swallowing

· Insomnia

· Blurred vision or blindness

· Difficulty in speaking

· Sudden jerky movements of arms and legs

The symptoms continue to worsen as the disease progresses. In the majority of the cases, the patient eventually lapses into a coma. However, heart failure, pneumonia, respiratory failure, and other serious infections cause death.

How Is the CJ Brain Disease Diagnosed?

Doctors typically make a diagnosis of CJD based on a patient’s personal and family medical history, a bunch of diagnostic tests, and a neurological examination. A doctor may prescribe an electroencephalogram (EEG), MRI, and Spinal Fluid tests to reach a diagnosis.

While all these tests help doctors make an accurate diagnosis in most cases, only a brain biopsy or autopsy can confirm the presence of Creutzfeldt–Jakob Disease.

Is There a Treatment Available For Creutzfeldt–Jakob Disease?

Unfortunately, there is no effective treatment available for Creutzfeldt–Jakob Disease. Researchers have tested several drugs over the years, and none have shown any benefits.

Treatment for Creutzfeldt–Jakob Disease only focuses on alleviating pain and other symptoms to make life as comfortable as possible for the patients.

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