In July 2008, the Indiana Departmer cluster of deaths of persons reportedly link

Question

In July 2008, the Indiana Departmer cluster of deaths of persons reportedly linked to the local baseball park and the local famous restaurant "Lisa's dogs" that served hotdogs and burgers. Available clinical gs and burgers. Available clinical and neuropathological findings were reviewed for 17 suspected deaths referred to IDH and CDC. To investigate the deaths of these 17 persons, all of whom were reportedly linked to Lisa's and the ballpark, health-care providers were contacted and relevant medical records obtained by IDH, other state health departments, and CDC. Concerns were raised that these deaths might have resulted from consumption of meat contaminated with some agent served at the ballpark restaurant during 1999.-2000. Providers were asked to submit available brain autopsy tissue to the disease diagnostic referral laboratory established by CDC and the American Association of Neuropathologists. Because the exact etiology of the disease was unknown, sufficient demographic and clinical information was available to classify 11 of the 17 deaths as resulting from a definite or probable cause on the basis of World Health Organization criteria. Of the remaining six deaths, neuropathological analyses documented that three deaths resulted from causes unrelated and the three deaths reported remain under investigation. Excluding the three deaths, the 14 remaining deaths occurred over a period of approximately 9.25 years (1999 2007); eleven of the 14 decedents were male; median age was 69.5 years (range: 50-83 years) Six of the decedents resided in Indiana, four in Pennsylvania, and one each in Connecticut Delaware, Maryland, and Virginia. Family member reported the disease is marked by rapid mental deterioration, usually within the span of a few months. Initial signs and symptoms of their loved ones were symptoms similar to Alzheimers, personality changes, anxiety depression, memory loss, impaired thinking, impaired muscle coordination and speech impairment. In 2001, the Indiana ballpark and Lisa's was closed permanently. The number and ages of all persons visiting or dining at the ballpark restaurant is unknown, and records, indicate attendance however, during 1995--2000 was approximately 4.1 million. The findings underscore the need for physicians to arrange for brain autopsies of all patients with clinically suspected or diagnosed of this particular disease with these particular symptoms

Explanation / Answer

a) The disease is vCJD. This is a varisntbform of Creutzfeldt Jacob disease. The microorganism causing this disease is prion.

b) Prions are made entirely of protein molecules. They do not belong to bacteria, fungi, viruses or parasites. They are unique group of organisms.

c) Prion particles are entirely made of proteins. It causes musfolded protein response.

d) Prion protein can trigger abnormal folding of proteins. The human disease caused by prions are rare. Creutzfeldt Jacob disease occurs as a result of consumption of meat associated with its consumption. Prions affect the brain mainly causing dementia, altered sensorium, hallucinations, muscle stiffness and speech impairment.

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