Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob Disease:

Creutzfeldt-Jakob Disease: Introduction

Creutzfeldt-Jakob disease is a rare neurodegenerative disease that rapidly, progressively and severely affects the brain. Creutzfeldt-Jakob disease is one cause of dementia and results in the destruction of brain cells in the brain. Creutzfeldt-Jakob disease eventually leads to the development of many tiny holes in the brain.

Creutzfeldt-Jakob disease is a seriously disabling and devastating disorder that progressively damages and destroys a person's memory, vision, and ability to think and move voluntarily, and function independently. Creutzfeldt-Jakob disease is one of a variety of transmissible spongiform encephalopathies (TSEs). Creutzfeldt-Jakob disease is not curable and is eventually fatal.

In addition to Creutzfeldt-Jakob disease, another type of transmissible spongiform encephalopathy is bovine spongiform encephalopathy (BSE), which is found in cows and is commonly known as "mad cow" disease.

The cause of Creutzfeldt-Jakob disease is not yet well understood, but it is believed that Creutzfeldt-Jakob disease may be caused by an abnormal protein called an infectious prion protein.

There are three types of Creutzfeldt-Jakob disease. Sporadic Creutzfeldt-Jakob disease is the most common form of the disease and has no known cause. Far more rare is hereditary Creutzfeldt-Jakob disease, which runs in families and/or occurs due to a specific genetic mutation that may affect normal prion proteins in the body.

Acquired Creutzfeldt-Jakob disease is extremely rare and caused by the exposure to the brain or nervous system tissue to the tissues of a person who has Creutzfeldt-Jakob disease. This generally occurs during a medical procedure, such as certain transplant surgeries and other neurological procedures.

Symptoms of Creutzfeldt-Jakob disease occur most often in the elderly, after the age of 60. Symptoms are due to the destruction of brain cells and include neurological symptoms, such as memory loss, blindness, and poor coordination. Symptoms of Creutzfeldt-Jakob disease eventually progress to become severely disabling, and coma and death eventually occur. For more details on symptoms and complications, refer to symptoms of Creutzfeldt-Jakob disease.

Making a diagnosis of Creutzfeldt-Jakob disease includes taking a thorough personal and family history, including symptoms, and completing a physical examination. This includes a neurological and psychological exam.

There is no specific diagnostic test to test for Creutzfeldt-Jakob disease, although scientists are currently researching a possible diagnostic test. Diagnosis is dependent on ruling out other diseases of the brain and nervous system, such as meningitis or encephalitis. Testing includes lumbar puncture, electroencephalogram (EEG), and CT or MRI is also performed to look for abnormal changes in the brain.

A definitive diagnosis can only be made upon examination of the brain during an autopsy or through a brain biopsy. In a brain biopsy, a small amount of tissue is removed from a living brain to examine for changes under the microscope. However, a brain biopsy is dangerous and may not always be accurate, so it is generally not done.

It is possible that a diagnosis of Creutzfeldt-Jakob disease can be missed or delayed because symptoms can mimic symptoms of other diseases and for a variety of other reasons. For more information on misdiagnosis, refer to misdiagnosis of Creutzfeldt-Jakob disease.

Creutzfeldt-Jakob disease is not curable, and at this time there are no treatments that can slow the advancement of the disease. However, there are some medications that may help to reduce some symptoms and increase comfort and quality of life. There are also clinical trials taking place to research a variety of potential treatments. For more information on treatment, refer to treatment of Creutzfeldt-Jakob disease. ...more »

Creutzfeldt-Jakob Disease: Creutzfeldt-Jakob Disease (CJD) is a rare 1-in-a-million disease that occurs spontaneously, with 10-15% inheritance, and unrelated to animal diseases. It usually leads to dementia.

CJD is a rare brain disease in humans caused by abnormal brain proteins (prions). It is similar to the various transmissible spongiform encephalopathies that afflict animals including "mad cow disease". Unfortunately, prognosis for CJD is poor as there are no effective treatments against prions. CJD is not usually contagious, except rarely by direct exposure to the brain (such as brain surgery).

Because CJD is rare it is often misdiagnosed as other brain-related diseases. Common misdiagnoses include Alzheimer's disease, motor neurone disease, ALS, Picks disease, Multi infarct Disease, and metabolic encephalopathies. ...more »