CREUTZFELDT-JAKOB DISEASE: AN OVERVIEW

Summary Creutzfeldt–Jakob disease or CJD is characterized by rapidly progressive dementia. It also tends to cause more rapid deterioration of a person’s abilities than Alzheimer’s disease or most other types of dementia. The disease is caused by prions which are defective proteins in the brain tissue.Initially, patients experience problem with muscular coordination; personality changes, including impaired memory, judgment, and thinking; and impaired vision. As the illness progresses, the patient’s mental impairment becomes severe. They often develop involuntary muscle jerks called myoclonus, and they may go blind. They eventually lose the ability to move and speak and enter a coma. Pneumonia and other infections often occur in these patients and can lead to death. Some symptoms of CJD can be similar to symptoms of other progressive neurological disorders, such as Alzheimer’s or Huntington’s disease. However, CJD causes unique changes in brain tissue which can be seen at autopsy. There is typical spongiform appearance of brain tissue. Electroencephalography, CSF studies and MRI scan confirms the diagnosis. Many drugs including antibiotics are found to be useless for treatment except quinacrine which has shown some promising results.