Creutzfeldt-Jakob Disease

What is Creutzfeldt-Jakob Disease?

CJD is a rare, degenerative, invariably a fatal brain disorder. It affects only about one person in every one million people per year worldwide. CJD usually appears in later life. Typically, the onset of symptoms occurs about age 60, and about 90 per cent of individuals die within 1 year. In the early stages of this disease, people may have behavioural changes, failing memory, visual disturbances and lack of coordination. As the illness progresses, blindness, mental deterioration becomes pronounced and involuntary movements, weakness and in worst cases coma may occur. It can be of 3 types i: e; sporadic CJD, hereditary CJD, acquired CJD.

Symptoms

CJD is characterized by rapidly progressive dementia. Initially, individuals experience problems with personality changes, muscular coordination, including impaired memory, judgment, thinking and impaired vision. People with the disease also may experience depression, insomnia, or unusual sensations. As the illness progresses, mental impairment becomes severe. Individuals often develop involuntary muscle jerks called myoclonus and they may go blind. They eventually lose the ability to move and speak and finally enter a coma.

How is CJD Diagnosed?

There is currently no diagnostic test for CJD. When a doctor suspects CJD, the first concern is to rule out treatable forms of dementia such as encephalitis or chronic meningitis. A neurological examination will be performed. Standard diagnostic tests will include a spinal tap to rule out more common causes of dementia and an EEG to record the brain's electrical pattern, which will show a specific type of abnormality in CJD. Computerized tomography of the brain can help rule out the possibility that the symptoms result from other problems such as stroke or a brain tumour. MRI brain scans can also reveal characteristic patterns of brain degeneration that can help diagnose CJD. 

Treatment

There is no specific treatment that can cure or control Creutzfeldt-Jakob Disease. Researchers have tested many drugs, including steroids, amantadine, acyclovir, interferon, antibiotics and antiviral agents. Studies of a number of other drugs are now in progress. However, so far none of these treatments has shown any consistent benefit in humans.

How is CJD Transmitted?

CJD cannot be transmitted through the air or touching or other forms of casual contact. Spouses and other household members of sporadic CJD patients have no higher risk of contracting the disease than the general population. However, exposure to spinal cord fluid and brain tissue from infected individuals should be avoided to prevent transmission of the disease.

How Can People Avoid Spreading the Disease?

To reduce very low risk of CJD transmission from one person to another, people should never donate tissues, blood, or organs. Normal sterilization procedures such as washing, cooking, and boiling do not destroy prions. Caregivers, healthcare workers, and undertakers should take the following precautions when they are working with a person with CJD:

• Cover cuts and abrasions with waterproof dressings.
• Avoid cutting themselves with instruments contaminated by the patient's blood or other tissues.
• Wear surgical gloves when handling a patient's fluids and tissues or while dressing the patient's wounds.
• Use face protection if there is a risk of splashing contaminated materials such as cerebrospinal fluid or blood.
• Use disposable bedclothes for contact with the patient. If disposable materials are not available, the regular cloth should be soaked in undiluted with chlorine bleach for an hour or more.
• Soak instruments that have come in contact with the patient in undiluted chlorine bleach for an hour or more, then use an autoclave to sterilize them in distilled water for at least one hour at 132-134 degrees Centigrade.