Creutzfeldt-Jakob Disease

Creutzfeldt - Jakob disease – A Rare & Fatal Ailment

Creutzfeldt-Jakob disease – CJD is a rare and fatal ailment which affects the brain causing brain damage which tends to get worse over a period of time. It is a degenerative neurological disorder which is incurable. CJD at times is called a human form of mad cow disease – bovine spongiform encephalopathy or BSE, though it is believed to be the cause of variant Creutzfeldt-Jakob disease in human and the two often tend to get confused.

CJD is caused due to an agent known as a prion which are misfolded proteins that are replicated by converting their properly folded counterpart to the same misfolded structure they tend to possess that leads to rapid neuro-degeneration resulting in the brain tissue developing holes, taking the form of a more sponge like texture.

These prions accumulate at high levels in the brain causing irreversible damage to the nerve cells resulting in the disease. The abnormal prions are infectious technically, and are very different to viruses and bacteria. Prions are not destroyed by extremes of heat and radiation which is used to kill bacteria and viruses while antibiotics or antiviral medication is not of much help.

The symptoms of CJD include loss of balance and co-ordination, change in personality, loss of intellect and memory, vision problems and blindness, slurred speech, abnormal jerking movements, progressive loss of mobility and brain function.

Four Types of CJD

Most of the people who suffer from this disease die within a year of the ailment, beginning usually from infection which is due to the immobility that makes the victim of CJD prone to infection.There are four forms of CJD namely:

Sporadic CJD which is the most common kind and the cause of it is not known, though it is suggested that in some cases, the normal brain protein goes through an abnormal process and turns into a prion. These cases usually occurs in older people between the age of 45 and 75 years of age and the average age of the symptoms progresses around 60 -65 years.

Variant CJD is probably caused on consuming meat from cow which could have been infected with a similar prion disease known as bovine spongiform encephalopathy – BSE or mad cow disease. The average time taken for the symptoms of variant CJD to take place after initial infection is yet unknown and the incubation period may be longer in some cases. Moreover the prion which may cause variant CJD could also be transmitted by blood transfusion though it is very rare.

Familial or inherited CJD is a rare genetic ailment wherein one of the genes of the person inherited from their parent carries a mutation causing the formation of prions in the brain during adulthood and triggers the symptoms of this disease which develops first in people in their early 50s.

Iatrogenic CJD is when the infection accidentally spreads from someone with CJD through surgical or medical treatment.

Presently there is no cure for CJD and treatment is given to relieve the symptoms making the person as comfortable as possible with medication to improve anxiety, depression together with painkillers to reduce the pain.