A new type of disease has baffled experts with its dementia like symptoms, on a more severe scale, which has taken the lives of 10 of its 16 sufferers from America.
Experts opine that the symptoms portrayed by these patients are close to Creutzfeldt-Jakob disease, which gradually incapacitates patients' ability to perform the basic functions of speech, thought and action. Scientists are still searching for its origins and mode of transmission and are unable to put a figure on the exact number of sufferers of this disease in the world.
Pierluigi Gambetti, director of the US National Prion Disease Pathology Surveillance Center at Case Western Reserve University in Cleveland, Ohio, feels the symptoms portrayed in earlier patients could have been mistaken for other forms of dementia. "I believe the disease has been around for many years, unnoticed," he said.
In victims suffering this rare type of dementia, the brain tissue appears to have tiny holes caused by the collection of a brain protein called PrP. It is also thought to be hereditary, triggered by gene mutations.
Infact, the paper presented by Gambetti makes a mention about six cases of dementia which were found to be familial. However, mutations in the PrP genes did not occur in these six cases. "Maybe there are other genes that have an influence on the disease," suggests James Ironside of the UK's National CJD Surveillance Unit in Edinburgh.
It is believed that CJD and all its other forms develop on its own. Yet, it can also be transmitted after exposure to brain material from victims of the disease during surgeries, and in the absence of proper sterilization.
Consumption of diseased cattle meat, especially from cattle suffering mad cow disease, is behind one type of CJD. It is yet unclear if triggers for this disease exist in the environment or in the diet. Gambetti has begun doing experiments on mice to understand how the disease is transmitted. "I believe the disease occurs naturally, and is not due to environmental causes," he said.