Question

Choose one prion disease, and find an interesting article about that disease online. Briefly summarize the disease listing symptoms, etc, and explain why that article was of interest to you. (250 words)

Answer 1

Hello,

1) Prions : Misfolded proteins which are causative for many neuronal system degenerative disorders.
2) One of the disorders caused by Prions is Creutzfeldt-Jakob disease (CJD).
4) Symptoms of disease : Failure of induction of immune response, Neuronal loss, characteristic changes.
5) Disease is fatal and is caused by an abnormally forming isoform a particular cellular glycoprotein.
6) This disease is classified under TSE - Transmissible Spongiform Encephalopathy.
7) Typical EEG patterns are diagnostic for the disease. Confirmation is done by immunological testing of brain.
8) Disease is extremely rare and is found in only one individual per million individuals. The risk of disease increases with age.
9) Occurence - Mostly as a sporadic disease. Rarely, due to mutations.
10) Patient comes with complaint of Dementia and shows some early neurological signs.
11) EEG shows periodic sharp waves and there is accumulation seen in immunohistochemical examination of brain.
12) Iatrogenic source of infection is due to usage of contaminated neurosurgical equipments.
13) The special thing about this disease as asked in question is the implementation of new rules by WHO for instrument sterilisation to reduce the risk of such diseases.
14) Immersion of instruments in Sodium hypochlorite is now made mandatory. However, thid id not strictly done as instruments get damaged due to this practice
15) No therapy of treatment has yet been discovered. The disease is invariably fatal.

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