Creutzfeldt-Jakob Disease (CJD)

Creutzfeldt-Jakob disease (CJD)—a fatal neurodegenerative illness, is one form of transmissible spongiform encephalopathies (TSE) affecting humans. The suspected causal agent of these diseases is the prion—a proteinaceous infectious particle. Designated as PrPSC, this infectious protein is unique in that it does not contain nucleic acid, which is different from a virus, yet has the capability of replication and being transmitted to other hosts. This capability of transmission poses a major problem in that the detection of the disease caused by this prion while the individual is in the incubation phase is not currently possible. Hence the disease may be accidentally transmitted to another individual through medical procedures—as seen in the case study presented.

Throughout the 20th century, various case studies evolved and were compiled to form a sketchy descriptive pattern for Creutzfeldt-Jakob disease. However, as time progressed, new forms of CJD emerged—usually as an epidemic, presenting similar yet distinct prodromal and clinical patterns. Accumulation of data has led to a finer delineation of symptomology and sub-type classification of the disease. Yet a test to definitively show that the individual has the disease during incubation is not currently available.

With different methods of transmission and completion of the incubation periods, various epidemics have erupted. Those points in time have been reflected within the literature, e.g. iatrogenic CJD through growth hormone (HgH). Current known forms of CJD are sub-typed as sporadic (spontaneous), familial (genetic), new variant and iatrogenic (acquired by accidental medical introduction).

Documentation of the transmission of Creutzfeldt-Jakob disease through blood products and other similar tissues is very limited. Currently, there are no cases found in the literature reflecting the onset of the disease via immune gamma globulin (IgG) treatment. One major reason for this may be due to the fact that even if the disease is transmitted, the incubation period may span beyond an individual’s lifespan. Therefore, the individual will die incubating the disease, never entering the clinical phase.

Each of the different categories of the disease presents various clinical and neuropathological symptoms and patterns at onset and throughout the course of the disease. The prion strain, codon 129 and method of i...

... middle of paper ...

...e the information is rich in detail and may be an index case;

· The interaction of factors at play within the disease which included incubation period, method of transmission, codon 129 and the prodromal, clinical and differential diagnostic characteristics of the disease; and

· An awareness as to the risk factors of transmission which may have bearing on public health policies.

Summary

The objective of the research was to determine if the peripheral transmission of Creutzfeldt-Jakob disease via contaminated IgG would follow the same or similar disease course characteristics as other types of peripheral iatrogenic CJD cases. Research focused on the behavioral, cognitive and motor functions in relation to those seen in various forms of CJD at various stages of the disease—whether typical or atypical.

Medical records, literature reviews and interviews/consultations with the individual and medical personnel were made to gather and analyze data. All of the data was compiled to determine whether the disease characteristics displayed indeed followed the distinct patterns of the disease or if surprises waited around the corner.