Variant Creutzfeldt-Jakob Disease: a rapid progressive dementias case study

Running head: RAPIDLY PROGRESSIVE DEMENTIAS (RPDs)
Rapidly Progressive Dementias (RPDs)
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1 RAPIDLY PROGRESSIVE DEMENTIAS (RPDs)
Introduction
The following paper aims to analyse variant Creutzfeldt-Jakob Disease (vCJD) which
is a rapid progressive dementias (RPD). In doing so, the paper will relate the case study with
prior disease symptoms and resease behind occurrence and will also compare VCJD with
non-prion RPDs.
Variant Creutzfeldt-Jakob disease (vCJD)
According to the World Health Organisation, vCJD is a progressive neuropsychiatric
disorder. The duration of the illness mainly range is greater than 6-month and the early onset
is guided by painful sensory symptoms (Liberski & Ironside, 2015).
Figure: classification of prion disease
(Source: Ironside et al. 2017)
Symptoms
The major symptom of this vCJD which belongs to the class of RPDs is guided by
behavioural and psychiatric features and this includes depression and agitation. The common
unusual sensory symptoms are pain and dysesthesia in face or limbs. Other abnormalities
associated with vCJD surface after 6 months of disease propagation. These abnormalities
include cerebellar ataxia, dystonia, chorea, cognitive impairment and visual abnormalities.

2 RAPIDLY PROGRESSIVE DEMENTIAS (RPDs)
The final stage of the disease is marked by the symptoms like akinetic mutism (Haïk &
Brandel, 2014).
Disease pathology
Apart from the stereotypic clinical features, the pathological phenotype of vCJD is
uniform in comparison to the other subtypes of CJD. However, Haïk and Brandel (2014)
highlighted that variations in the severity of the pathological lesions occurs with an increase
in the age of the individuals. The main causative agent of vCJD is the prior protein (PrP).
PrPc is the normal isoform of the disease while PrPd is the isoforrm of the misfolded (d
signifies for disease). The gene encoding PrP (PrPNP) is present in the polymphic nature in at
codon 129 (Val or Met). The Mutation of Met (methionine) is mutated to Valine (Val) at the
polymorphic codon leading to the disease development. The mutated isoform of the protein
(PrP) is abnormally mutated and thus leads to the accumulation of these abnormally folded
proteins in the brain leading to the disease development (Haïk & Brandel, 2014).