Creutzfeldt-Jakob Disease: A Prion-Related Neurodegenerative Disorder

Creutzfeldt-Jakob Disease: A Prion-Related Neurodegenerative Disorder

Sadeeq Muhammad Sheshe (COMSATS Institute of Information Technology, Pakistan)
DOI: 10.4018/978-1-5225-5282-6.ch017

Abstract

Creutzfeldt-Jakob disease (CJD) is a rare disease associated with neurodegeneration mostly characterized by damage to the neurons. CJD is caused by aggregation of misfolded proteins known as prions; thus, CJD is said to be a prion-related illness. CJD and other prion-related illnesses such as Kuru and Gerstmann-Sträussler-Scheinker disease (GSS) have been reported to have complex mechanisms due to their association with the brain and the nervous system in general. A lot of questions have been raised about the mechanism, diagnosis, and pathogenesis of this disease. The complexity of prion proteins themselves have contributed to more questions about the complications of CJD, whether misfolding of the prions are responsible for neurodegeneration or the misfolding are mere symptoms of the disease. This chapter attempts to explore some details about CJD and answers most related questions about the disease's mechanism. The author finally attempts to explore recent development in pathogenesis, diagnosis, and treatment of CJD.
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Background

Neurons being the major cells of the nervous system could be damaged progressively by one cause or another leading to neurodegeneration and consequently diseases, such diseases are known as NDs (Aguzzi & Zhu, 2012). NDs are diseases associated with damage in structure and functions of the neurons, one of the important cells of the nervous system (Aguzzi & Zhu, 2012). In neurobiology, neurons form the complex network of communication around the central nervous system. Damage to neurons due to a number of factors has been reported to be lethal and associated with a number of diseases and disorders (Murray & Davis, 2003).

CJD was discovered by the German neurologist, Hans Gerhard Creutzfeldt and Alfons Maria Jakob in 1921 (Cordery et al., 2003). Creutzfeldt was the first to describe the disease characterized with neurodegeneration and Jakob proposed the disease to be associated with prions (Holman et al., 2010). However some of descriptions of the disease made by Jakob and Creutzfeldt did not match the current description of the disease (Creutzfeldt, 2002). This including being transmissible as well as currently being observed to be part of a class of both human and animal diseases called TSEs.

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