Question

1. A seventy year-old male patient presented with complaints of weakness and involuntary movements in his left arm, changes in behavior, and forgetfulness. He also developed mutism after nearly three months. Although no microbes were recovered from his cerebral spinal fluid, high levels of Protein 14-3-3 were found in this fluid. Following his death, examination of his brain showed extensive degeneration such that his brain resembled a sponge.

1. What disease is it likely that this man has?

2. What is the causative agent involved in this disease?

3. How does this infectious entity cause the neurodegeneration?

Answer 1

a) High levels of Protein 14-3-3 are a diagnosis for the Creutzfeldt-Jakob disease (CJD). The man was most likely affected by this disease.

b) The disease is caused by an abnormal form of a cellular glycoprotein. The abnormal form is calles as a prion.Prions are misfolded or abnormally folded proteins with the ability to spread the misfolded shape to the nomral proteins.

c) The prions destroy neurons by destroying the signal transmission between neurons. They affect the dendritic spines, which receive signal from other neurons, thus eventually leading to neurodegeneration.