The initial symptom of Creutzfeldt-Jakob Disease (CJD) is rapidly progressive dementia, resulting in loss of memory, alterations in personality and hallucinations. Physical problems, such as jerky movements, defective speech, ataxia (coordination and balance problems), alterations in gait, rigid postures and seizures usually accompany the cognitive impairments. In some patients signs and symptoms can persist for years, while in many cases of non-inherited (sporadic) CJD, death occurs not long after the first symptoms emerge. Meaning of signs and symptoms - the patient feels and describes a symptom, while other people, including the physician or nurse detect a sign. For example, headache may be a symptoms while dilated pupils may be a sign. CJD signs and symptoms and the disease's rates of progression vary depending on the type of CJD and the person.
Creutzfeldt-Jakob disease (CJD) is caused by a prion - an infectious protein in the brain. The disease belongs to a broad group of animal and human diseases known as TSEs (transmissible spongiform encephalopathies). When the brain of an affected person is viewed under a microscope, it has many spongy holes, hence the use of the word "spongiform". Proteins are made up of strings of amino acids and then fold themselves into a three-dimensional shape, which help our body cells function. The protein folding helps them perform vital functions inside the cells of our body. Prion proteins (not the same as the infection-causing CJD ones) exist in our brains and other tissues within our nervous system. Experts believe they are key players in long-term memory, however nobody is really sure what they do.
Researchers have identified three basic ways that Creutzfeldt-Jakob disease (CJD) may develop: Spontaneously. Most people with classic CJD develop the disease for no apparent reason. CJD cases occurring without explanation are called spontaneous CJD or sporadic CJD. This is the great majority of all cases. Normally, sCJD affects people over 40 years of age. By genetic mutation. About 5 percent to 10 percent of people with CJD have a family history of the disease or test positive for a genetic mutation associated with CJD. This type is referred to as familial CJD (fCJD). What Is Creutzfeldt-Jakob Disease (CJD)? What Is Mad Cow Disease? What Are The Signs And Symptoms Of Creutzfeldt-Jakob Disease (CDJ)? What Are The Complications Of CJD? What Are The Causes Of Creutzfeldt-Jakob Disease (CJD)?
In the majority of cases, doctors do not know what caused an individual to develop CJD (Creutzfeldt-Jakob disease). Although several factors may be linked to some extent to CJD risk, none have been specifically and compellingly identified. The following risk factors appear to be linked to different kinds of CJD to some extent: Age - if someone develops Sporadic CJD, they will do so when they are about 60 years old. Familial CJD symptoms usually start at a younger age. Some individuals, though, can get vCJD (variant CJD) much earlier in life. Your genes - familial CJD occurs among people who have a genetic mutation that causes the disease to develop. A susceptible individual only needs to inherit one copy of the faulty gene (from mother or father) to have a higher risk of developing familial CJD.
Only a brain biopsy or an examination of brain tissue after death during autopsy can confirm (diagnose) the presence of Creutzfeldt-Jakob disease ( CJD ). However, doctors often can make an accurate diagnosis based on the patient's medical and personal history, a neurological exam, and certain diagnostic tests. A neurologist will carry out the tests to rule out other conditions with similar symptoms, such as Alzheimer's disease, Parkinson's disease or a brain tumor. The neurological exam is likely to reveal characteristic symptoms as: muscle twitching and spasms abnormal reflexes What Is Creutzfeldt-Jakob Disease (CJD)? What Is Mad Cow Disease? What Are The Signs And Symptoms Of Creutzfeldt-Jakob Disease (CDJ)? What Are The Complications Of CJD? What Are The Causes Of Creutzfeldt-Jakob Disease (CJD)?