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Creutzfeldt-Jakob Disease Essay

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Background Since about 1700, transmissible spongiform encephalopathies were untreated brain conditions that only affected sheep and goats. Known as “scrapie,” the disease was only found in sheep. After 200 years, a human discovery of transmissible spongiform encephalopathies, now known as Creutzfeldt-Jakob disease was found in a human by German scientists in 1920. Creutzfeldt-Jakob disease, also known as the human transmissible spongiform encephalopathy, is a degenerative disease in humans that affects the nervous system and results in rapidly progressive dementia, loss of motor control, paralysis, and death. Creutzfeldt-Jakob disease is caused by a protein called a prion, which causes the normal proteins to fold irregularly and affects the prions’ ability to function. Creutzfeldt–Jakob disease occurs in about one in a million people.
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The patient died after thirteen months. The clinical symptoms degenerated steadily to myoclonus [unusual movements] and akinetic mutism [neither tending to move or speak] on the fourteenth week.The initial MRI showed posterior reversible encephalopathy syndrome [PRES] on the ninth week after the onset of symptoms. Following MRI tests’ suggested symmetrical lesions in the basal ganglia on the thirteenth week and an EEG with periodic sharp wave complexes in front regions on the eighteenth week which allowed them to diagnose patient with Sporadic Creutzfeldt-Jakob Disease.

“The histopathological findings after brain biopsy on the fourteenth week demonstrated the presence of the abnormal prion protein deposits in the grey matter by immunohistochemistry with ICSM35, KG9 and 12 F10 antibodies and confirmed the diagnosis of sCJD––” (BMC

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