Creutzfeldt-Jakob disease is an uncommon, but a serious and inevitably fatal disorder of the brain. There are close to 200 cases of CJD in the United States of America. This disease generally starts in late adult life, but progresses very rapidly. Generally, the symptom onset is approximately at 60 years of age with the affected individual passing away within a year of the symptom onset in most of the cases. The early symptoms of Creutzfeldt-Jakob disease or CJD is memory loss, changes in the behavioral pattern, coordination abnormalities, etc. With the progression of the disease, there is deterioration of the mental condition of the patient along with extremity weakness, blindness, and eventually the patient may go into coma.
Categories of Creutzfeldt-Jakob disease or CJD
There Are Three Major Categories of Creutzfeldt-Jakob Disease or CJD:
- Sporadic Creutzfeldt-Jakob Disease or CJD: This is the most common category of Creutzfeldt-Jakob disease or CJD. It may affect a person who may have no risk factors associated with Creutzfeldt-Jakob disease or CJD. This type of CJD is present in approximately 80% of the cases.
- Hereditary CJD or Hereditary Creutzfeldt-Jakob Disease: In this type of Creutzfeldt-Jakob disease or CJD, the patient may have a family history of this disease or may have a positive mutation of gene associated with the disease. This type of CJD accounts for approximately 10% of the cases in USA.
- Acquired Creutzfeldt-Jakob Disease or Acquired CJD: In this type of Creutzfeldt-Jakob disease or CJD, the disease is transmitted to the brain generally from medical procedures. This type of CJD is pretty rare.
Signs and Symptoms of Creutzfeldt-Jakob Disease or CJD
Creutzfeldt-Jakob disease or CJD is described by dementia, which progresses very quickly. During the early stages of the disease, people may have difficulties with muscle coordination. They may undergo personality changes. They may have memory disorders. They may have impaired judgment and thinking. Some people may also experience visual disturbances. Insomnia, depression, or other uncommon sensations are also some of the symptoms. As the disease advances, the mental disorder becomes more pronounced. People begin to have involuntary jerking movements. They may also become blind. Eventually, they become so debilitated that they are not able to move or speak and finally go into coma.
There are some symptoms of this disease which are quite similar to other neurological disorders like Alzheimer disease etc., but dementia caused by Creutzfeldt-Jakob disease or CJD is more rapid and quick than any other forms of dementia.
Diagnosis of Creutzfeldt-Jakob Disease or CJD
At present, there is no clear-cut way to diagnose this disease. When there is a suspicion, the first thing the physician does is rule out other causes of dementia. The physician may perform a thorough neurological evaluation. He may conduct tests such as a spinal tap or an EEG to rule out other causes of the symptoms. He may also order imaging studies such as a CT scan or an MRI of the brain. Brain biopsy by far is the best way to confirm the diagnosis of Creutzfeldt-Jakob disease or CJD.
Causes of Creutzfeldt-Jakob disease or CJD
Some people involved in the research of this disease are of the belief that there is some form of virus or some other organism that causes Creutzfeldt-Jakob disease or CJD, but they have till date not been able to pinpoint a virus or any other organism. Moreover, the characteristics of the agent responsible for CJD are not known to be present in viruses or bacteria. As of today, the principal belief is that CJD is caused by a type of protein called as Prion. There are two types of Prions found in the body, one which is normal which is pretty benign and harmless, but there is an infected one which is the root cause of this disease.
Prognosis of Creutzfeldt-Jakob Disease or CJD
As stated above, the prognosis of this disease is very poor.
- New England Journal of Medicine (NEJM): Article: “Creutzfeldt-Jakob Disease” DOI: 10.1056/NEJMra0804619
- Journal of Neurology, Neurosurgery, and Psychiatry (JNNP): Article: “Creutzfeldt-Jakob disease” DOI: 10.1136/jnnp-2011-301400
- Journal of Alzheimer’s Disease: Article: “Creutzfeldt-Jakob Disease and Alzheimer Disease Overlap: A Case Report” DOI: 10.3233/JAD-2011-111055
- Alzheimer’s Research & Therapy: Article: “Rapidly progressive Alzheimer’s disease presenting as Creutzfeldt-Jakob disease: A report of three cases” DOI: 10.1186/s13195-020-00721-3