It is a neurological in which the brain cells become inactive and results in dementia and impaired cognitive functions. It is also known as subacute form spongiform encephalopathy. It results in fatal condition.
It accounts for the 60-70 % of the dementia cases and it is uncommon under the age of 45 yrs.
Types of CJD:
It is caused by the abnormal build up of proteins called prions(misfolded proteins) in the brain and also the conformational change of prions in the brains. The buildup of protein called amyloid protein and tau protein leads to the death of the brain cells. Amyloid proteins build up in the surface of the brain forming larger masses of cells called plaques.Twisted protein of another protein which is termed as tau results in tangled structure of protein.These plaque and tangled blocks the synaptic communication between the nerve cells and results is loss of brain function and prevents the brain from carrying out its action.This leads to the programmed cell death in the indivduals with deposition of amyloid fibrils in their brain.The slow and ongoing death of the cells starting in one area of the brain eventually sprading to other areas usually starts in the area which controls the memory and leading to the cause of the Creudtzfeldt jacob disease’s disease.
These defective proteins can be transmitted by the contamination of the harvested human brain products such as corneal grafts, dural grafts or electrode or through implants and human growth hormone.
It also can be contacted from the animals by eating the food from animals infected with bovine spongiform encephalopathy (BSE) the bovine form of TSE also known as Mad Cow Disease.
Cannibalism ( eating the same species for food) might also result in development of the disease
Inability to recall the past events
Difficulty to recognize the family members
Difficult in finding the words and reading
Spatial or vision issues
Impaired reasoning /judgement skills
Movement difficulties and problems linked to smell perception is also seen in some cases
Involuntary muscle movements which are known as Myoclonus.
Psychosis
Obessessive compulsive disorder.
STAGES OF CREUDTZFELDT JACOB DISEASE DISEASES:
Mild stage:
greater memory loss and impaired cognitive functions.Wandering and getting lost,repeating the questions,difficulty in paying bills and collecting money,feeling difficult to do day to day activities,personality and behavioural changes.Patients are often diagnosed in this stage
Moderate stage:
In this stage the damage occurs to the brain cell that controls the language,reasoning,sensory processing and consciousness.Memory loss and confusion grows and the patients loss the ability to recognize the family and friends.The person find difficulty in dressing and to cope up with the new situations,inability to do multistep task.The patient might experience hallucinations,delusions and paranoia.
Severe stage:
The amyloid and tau tangles covers the brain and leads to shrinkage of the brain.Loss of communication and the patient is completely depend on the other for their survival.the patient is mostly bed ridden at this stage and he/she is nearer to death.
It is usually diagnosed with the history of the patient and behavioural changes and impaired cognitive functions.
Medical imaging:
Magnetic resonance imaging-MRI
Computed tomography-CT
Single photon emission computed tomography
Positron emission tomography : It is used in early diagnosis of Creudtzfeldt jacob disease disease.
Biomarker tests. A biomarker is a biological factor that can be measured to indicate the presence or absence of disease or the risk of developing a disease. The biomarker for Creudtzfeldt jacob disease disease is the beta amyloid,tau proteins and phsopho tau which are detected in the CSF
Neuropsychological test mainly to observe cognitive functions is done with MINI MENTAL STATE EXAMINATION
Prevention:
