Creutzfeldt-Jakob disease (CJD) also known as subacute spongiform encephalopathy is a degenerative disorder and a rare fatal condition that affects the brain. The onset of the problem and symptoms occur at about the age of sixty. The signs can be like those of dementia or Alzheimer's disease, but it usually appears in the later part of life and mostly leads to memory loss and untimely death. CJD is primarily of three types. One of them is sporadic CJD which is when the disease appears even though the person has no known risk factors for the disease. This is the most common type of CJD and accounts for at least 80 percent of cases.
The second is hereditary CJD, when the person may have a family history of the disease and test positive for a genetic mutation associated with CJD. The rarest one is acquired CJD, the disease is transmitted by exposure to brain or nervous system tissue, usually through certain medical procedures. Over time, people with CJD withdraw from friends and family and eventually lose the ability to recognize or relate to them. They also lose the ability to care for themselves and many eventually slip into a coma. The disease is always fatal and does not have a specific treatment per se, but the spread of this ailment can certainly be prevented.
Symptoms Of CJD
Early signs and symptoms of this disease typically include:
- Memory loss
- Impaired thinking
- Blurred vision
- Insomnia
- Difficulty speaking
- Difficulty swallowing
- Jerky movements
- Behavioral changes
How is CJD Transmitted?
CJD cannot be transmitted through the air or other forms of contact, however, exposure to brain tissue and spinal cord fluid from infected persons should be avoided to prevent transmission of the disease through these materials. In some cases, CJD has spread to other people from grafts of dura mater, transplanted corneas, implantation of inadequately sterilized electrodes in the brain, and injections of contaminated pituitary growth hormone derived from human pituitary glands.
How is CJD Diagnosed?
The only way to confirm a diagnosis of CJD is by brain biopsy or an autopsy. In a brain biopsy, the neurosurgeon removes a piece of tissue from the person’s brain for further investigation. Electroencephalography (EEG) is another technique that records the brain’s electrical pattern and can help in identification since it shows a specific type of abnormality in major types of CJD. Cerebrospinal fluid-based tests of a person can also demonstrate the specificity of the disease. Magnetic Resonance Imaging (MRI) has recently been found to be accurate in identifying about 90 percent of CJD cases. In some cases where none of the above tests confirm the diagnosis, an autopsy is the only resort whereby the whole brain is examined after death and the exact cause of CJD is studied.
Risk Factors Of CJD
Mostly, the causes of Creutzfeldt-Jakob Disease are unknown, however, a few factors seem to be associated with different kinds of CJD such as:
Age: Sporadic CJD develops later in life, but the onset of familial CJD is slightly earlier and has affected people at a much younger age such as in their late twenties.
Genetics: People with familial CJD have a genetic mutation that causes the ailment to progress. To develop familial CJD, a child must have one copy of the mutated gene inherited from either parent. If the parent has the mutated gene, the chance of passing it on to the child is nearly 50%.
Also Read: Genetic Testing: What Is It, How It Is Done, And What To Expect?
Exposure To Contaminated Tissues. People who receive transplants of the infected tissues that cover the brain (dura mater) may be at risk of getting a CJD.
How To Avoid the Spread Of CJD?
To reduce the risk of CJD transmission from one person to another, people should never donate blood, tissues, or organs if they have suspected or confirmed CJD or if they are at increased risk of the disorder. Medical practitioners, and healthcare workers, should take precautions when they are working with a person with CJD. They must wear surgical gloves when handling the patient's fluids and cover their cuts and abrasions with waterproof dressings besides using disposable clothing. They must also use face protection if there is a risk of splashing contaminated material such as blood or cerebrospinal fluid which can be a carrier of the disease-laden tissues and cells. There's no known way to prevent sporadic CJD. The only remedy is to delay the progression and prevent the spread. In the case of genetically inherited CJD, talking with a genetics counselor may help sort through the risks associated with the situation.
