The National Institutes of Health (NIH) states “Creutzfeldt-Jakob disease (CJD) is a form of brain damage that leads to a rapid decrease of mental function and movement.” A rare disorder, it affects one out of one million people between the ages of 20 and 70. The Mayo Clinic states that 85 to 90 percent have sporadic CJD, where there is no known cause or risk factors. The other forms of CJD are familial CJD, which is an inherited form, and new variant CJD, which is caused by mad cow disease.
The mental deterioration from CJD is noticeable. It results in cognitive problems, emotional changes and movement problems. At first, CJD resembles depression. The patient can have personality changes, such as unusual sadness or irritability. Social withdrawal can occur, where the patient pulls away from friends and family; this can also be seen with the patient's loss of interest in usual activities. In addition, the patient can also have problems concentrating when working on a task, and can have memory lapses.
As CJD progresses, the patient develops dementia, which impairs cognitive functions like memory, learning, judgment and problem solving. Tschampa et al. in the Journal of Neurology article “Patients with Alzheimer's disease and dementia with Lewy bodies mistaken for Creutzfeldt-Jakob disease” note the similarities in the cognitive deficits of all three disorders. Their study focuses on a large number of patients who were diagnosed with CJD, but after a postmortem examination, they were found to have had Alzheimer's disease or dementia with Lewy bodies.
In the discussion of the paper, the authors note the clinical overlap. Patients with all three disorders exhibited dementia, muscle rigidity and myoclonus, which is an involuntary muscle jerk. Visual hallucinations are also seen in both CJD and dementia with Lewy bodies. However, the NIH notes that the confusion between these three types of dementia is not common. Alzheimer's disease and dementia with Lewy bodies are slow developing neurological disorders, and are more common that CJD. CJD, on the other hand, has a rapid development of dementia and delirium, with severe symptoms present within a few weeks or months after onset.
Prognosis for CJD is poor. The NIH states that most patients die within eight months of diagnosis from a complication, such as respiratory failure, infection or heart failure; however, some CJD patients live up to two years. By the time of death, the patient can no longer take care of herself. She can experience hallucinations, incontinence and severe memory loss. Vision can also be affected, resulting in blurred vision or blindness. The patient also loses her ability to speak and swallow, and can also fall into a coma.
Elizabeth Stannard Gromisch received her bachelor’s of science degree in neuroscience from Trinity College in Hartford, CT in May 2009. She is the Hartford Women's Health Examiner and she writes about abuse on Suite 101.