Reutzfeldt-Jakob disease (CJD) is a degenerative fatal brain disorder.

Creutzfeldt-Jakob Disease – Definition

Creutzfeldt-Jakob disease (CJD) is a degenerative fatal brain disorder, which gradually destroys brain cells, and it causes tiny holes in the brain. CJD is caused by a protein called a prion. A prion causes normal proteins to fold abnormally. This affects other protein’s ability to function. People with CJD will have ataxia, or difficult in controlling body movements, abnormal gait, speech, and dementia.

Types

There are three major categories of CJD:

Sporadic CJD – It is the most common type disease appears even though the person has no known risk factors for the disease.

Hereditary CJD – In this the person has a family history of the disease and/or tests positive for a genetic mutation associated with CJD.

Acquired CJD – This disease is transmitted by exposure to brain or nervous system tissue, usually through certain medical procedures such as surgical treatments.

History

CJD captured public attention in the1990’s when some people in the United Kingdom developed a form of the disease called variant CJD (vCJD), after eating meat from diseased cattle and beef. Although serious CJD is rare, vCJD is the least common form. Worldwide, there is an estimated one case of CJD diagnosed per million people each year but most often in older adults.

The brain affected by CJD and Healthy brain

Epidemiology of Creutzfeldt-Jakob disease

Before the identification of vCJD, Creutzfeldt-Jakob disease (CJD), the most common of the known human TSEs, was thought to exist in only three forms:

Sporadic CJD, which occurs throughout the world at the rate of about one per million people, and accounts for about 85% of CJD cases;

Familial CJD, which is associated with a gene mutation and makes up 5–15% of CJD cases; and

Iatrogenic CJD, which results from accidental transmission via contaminated surgical equipment or as a result of corneal or meningeal (dura mater) transplants or the administration of human-derived pituitary growth hormones; this accounts for less than 5% of CJD cases.

Risk factors

People who consume contaminated beef may be at increased risk for developing an acquired form of Creutzfeldt-Jakob disease called variant CJD (vCJD)

Age and genetics

Exposure to contaminated tissue

Causes

CJD happens when a prion protein, an abnormal kind of amyloid protein, causes abnormalities in other proteins

The buildup and malformation of prions on the brain cells ultimately lead to brain damage and death. It may be sporadic, inherited, or acquired

Symptoms of Creutzfeldt-Jakob Disease

Depression

Agitation, apathy and mood swings

Rapidly worsening confusion, disorientation, and problems with memory, thinking, planning and judgment

Difficulty walking

Muscle stiffness, twitches and involuntary jerky movements

Diagnosis and Test

Physical examination will look for muscle spasms and check the patient’s reflexes

Vision or eye test may detect partial blindness

Electroencephalogram (EEG) can reveal abnormal electrical impulses

CT scan or MRI can rule out stroke as a cause of symptoms

Lumbar puncture or spinal tap, can test spinal fluid to rule out other causes of dementia

There is no cure for CJD, and no medications can help to control it or slow disease progression

Treatment aims to relieve symptoms and make the patient as comfortable as possible

Treatment and Medication

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

Current treatment for CJD is aimed at alleviating symptoms and making the individual as comfortable as possible

Opiate drugs can help relieve pain if it occurs, and the drugs clonazepam and sodium valproate may help relieve myoclonus

Prevention of Creutzfeldt-Jakob Disease

Preventive measures include sterilization of all medical equipment to kill any organisms that may cause the disease, and not accepting cornea donations from people with a history of diagnosed or possible CJD

Wear surgical gloves when handling a CJD patient’s tissues and fluids or dressing the patient’s wounds

Avoid cutting or sticking with instruments contaminated by the CJD patient’s blood or other tissues

Research is underway into the role of prions in CJD, to find out how the disease affects the brain, and to find an effective treatment

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