Prion Disease: Comparison of vCJD and RPD Mechanisms and Symptoms

This report provides a comparative analysis of two prion diseases: variant Creutzfeldt-Jakob disease (vCJD) and rapidly progressive dementia (RPD). The report begins by contrasting the occurrence of vCJD, a rare neurological disease caused by misfolded prion protein (PrPSc), with RPD, often associated with mutations in the prion protein gene. It then compares the major symptoms, highlighting psychiatric symptoms and neurological abnormalities in vCJD patients versus the initial apathy, ataxia, and headaches associated with RPD. The diagnosis section discusses the challenges in diagnosing vCJD, often requiring post-mortem examination, while RPD diagnosis involves MRI, blood, urine, and cerebrospinal fluid tests. The report further delves into the biological mechanisms and disease pathology of both conditions, explaining how misfolded PrPSc leads to brain damage in vCJD and how various causative agents, including antibodies and prion proteins, cause progressive nerve cell loss in RPD. The report references several studies to support its findings.

Running head: PRION DISEASE
Prion disease
Name of the Student
Name of the University
Author note

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Table of Contents
Compare and contrast RPD and vCJD:...........................................................................................3
Occurrence:..................................................................................................................................3
Major symptoms:.........................................................................................................................3
Diagnosis:........................................................................................................................................3
Description of vCJD biological mechanisms and disease pathology:.............................................4
Description of RPD biological mechanisms and disease pathology:..............................................4

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Compare and contrast RPD and vCJD:
Occurrence:
Variant Creutzfeldt-Jakob disease is relativity rare and new neurological disease, which
classified as the transmissible spongy form encephalitis. The causative agent of vCJD is the
misfolded prion protein (PrPSc). On the other hand, variant Creutzfeldt-Jakob often called as the
prototype of rapidly progressive dementia. According to Verity et al. (2018), rapidly progressive
dementia syndrome is associated with four extra repeat mutations in the gene of misfolded prion
protein (Asante et al. 2015).
Major symptoms:
Patient with variant Creutzfeldt-Jakob has predominant psychiatric symptoms such as
frequent depression. Patients suffer often-experienced neurological abnormalities such ataxia
within months and dementia in late illness (Sigurdson, Bartz and Glatzel, 2018). On the hand,
since variant Creutzfeldt-Jakob is considered as rapidly progressive dementia, the patient initially
experience apathy, mild ataxia, and headache, seizures that will lead to death.
Diagnosis:
In terms of sensitivity, it is difficult to diagnosis variant Creutzfeldt-Jakob. The definite
diagnosis for vCJD is post-mortem examination of brain tissue (Chiesa 2015). On the other hand,
vCJD is the prototype RPD the diagnosis, postmortem can detect the disease. However, other
diagnosis involves MRI, laboratory testing such as blood, urine, and cerebrospinal fluid. Cell
counts of the different protein, IgG index are used for the RPD (Sigurdson, Bartz and Glatzel,
2018).

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Description of vCJD biological mechanisms and disease pathology:
The study of prion disease has become an area of intense interest since experimental
evidence emerged for transmission of phenotypic variation. According to Goedert (2015) , the
underlying mechanism of the disease is misfolded Prion protein (Prp Sc) which produces the
disease by molecular conversion from normal cellular protein (PrpC). PrpC normally found in
different organs as normal cellular protein and conversion of the abnormal protein from normal
one proceeds as the chain reaction when the alpha form of amino acid converted to the Beta form
(Goedert 2015). The aggregates of PrPSc lead to brain damage and characteristics symptoms.
Figure: normal cellular protein to the misfolded prion protein
Source :(Goedert 2015).
Description of RPD biological mechanisms and disease pathology:

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In contrast to the more common dementia, rapidly progression dementia can develop
subacutely over months and can be fatal. The causative agents of rapidly progressive dementia
are antibodies, Lewy bodies, metabolic and toxic agents and prion protein (Bennett and Thomas
2014). These causative agents lead to the progressive loss of nerve cells that further caused the
loss of brain function due to serious stroke (Asante et al. 2015). Neurofibrillary tangles are the
primary marker of dementia which is caused by Alzheimer’s disease (Pope et al. 2017)

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References:
Asante, E.A., Smidak, M., Grimshaw, A., Houghton, R., Tomlinson, A., Jeelani, A., Jakubcova,
T., Hamdan, S., Richard-Londt, A., Linehan, J.M. and Brandner, S., 2015. A naturally occurring
variant of the human prion protein completely prevents prion disease. Nature, 522(7557), p.478.
Bennett, S. and Thomas, A.J., 2014. Depression and dementia: cause, consequence or
coincidence?. Maturitas, 79(2), pp.184-190.
Chiesa, R., 2015. The elusive role of the prion protein and the mechanism of toxicity in prion
disease. PLoS pathogens, 11(5), p.e1004745.
Goedert, M., 2015. Alzheimer’s and Parkinson’s diseases: the prion concept in relation to
assembled Aβ, tau, and α-synuclein. Science, 349(6248), p.125.
Sigurdson, C.J., Bartz, J.C. and Glatzel, M., 2018. Cellular and Molecular Mechanisms of Prion
Disease. Annual Review of Pathology: Mechanisms of Disease.
Pope, H., Kanayama, G., Hudson, J., Brennan, B., Jensen, E. and Kaufman, M., 2017. Long-term
anabolic-androgenic steroid (AAS) Use: A possible new risk factor for early dementia. Journal
of the Neurological Sciences, 381, p.772.
Xiong, L., Boulouis, G., Charidimou, A., Roongpiboonsopit, D., Jessel, M.J., Pasi, M., Reijmer,
Y.D., Fotiadis, P., Ayres, A., Merrill, E. and Schwab, K., 2018. Dementia incidence and
predictors in cerebral amyloid angiopathy patients without intracerebral hemorrhage. Journal of
Cerebral Blood Flow & Metabolism, 38(2), pp.241-249.

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