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Creutzfeldt-Jakob disease - Symptoms, Causes and Treatment

Definition

Creutzfeldt-Jakob disease (CJD) is a rare. Actually it is a degenerative, invariably fatal brain disorder. Typically, its symptoms occurs at about age 60. There is currently no single diagnostic test for CJD. While CJD can be transmitted to other people, the risk of this happening is extremely small.

In contrast to the traditional forms of CJD, vCJD has affected younger patients (average age 29 years, as opposed to 65 years), has a relatively longer duration of illness (median of 14 months as opposed to 4.5 months) and is strongly linked to exposure, probably through food, to a TSE of cattle called Bovine Spongiform Encephalopathy (BSE). There are three major categories of CJD are :-

1) sporadic CJD
2) Hereditary CJD, and
3) Acquired CJD

Causes

There are several types of Creutzfeldt-Jakob disease (CJD). The disorder itself is rare, occurring in about 1 out of 1 million people. Prion diseases are a unique form of infectious diseases. The disease is not produced by a bacterial or viral infection; instead, the illness is related to progressive accumulation of prion proteins.

It is clear that Creutzfeldt-Jakob disease is caused by an infectious agent, but it is not yet clear what type of agent that is. Originally assumed to be a virus, evidence is accumulating that, instead, CJD is caused by a protein called a prion transmitted from victim to victim. The other spongiform encephalopathies also are hypothesized to be due to prion infection.

Both classic and variant CJD belong to a broad group of human and animal diseases known as transmissible spongiform encephalopathies (TSEs). . It usually first appears in midlife, beginning between ages 20 and 70, with average age at onset of symptoms in the late 50s.

Symptoms

The most characteristic symptom of CJD is rapidly progressing dementia, or loss of mental function. Dementia is marked by :-

memory losses
impaired abstraction and planning
language and comprehension disturbances
poor judgment
disorientation
decreased attention and increased restlessness
personality changes and psychosis
hallucinations

Treatment

The only way to confirm a diagnosis of CJD is by brain biopsy or autopsy. In a brain biopsy, a neurosurgeon removes a small piece of tissue from the patient's brain so that it can be examined by a neurologist.

There is no known cure for Creutzfeldt-Jakob disease. Custodial care may be required early in the course of the disease. Medications may be needed to control aggressive behaviors. These include sedatives, antipsychotics, and others.
Currently, clinical studies include tests of antiviral drugs, such as amantadine, a compound useful in parkinsonism.