A Neurocognitive Rarity: Explaining Creutzfeldt-Jakob Disease


When thinking about neurocognitive diseases, the most common examples that arise are usually dementia, Alzheimer’s disease, and cognitive dysfunction brought upon by traumatic brain injury. A more unknown neurocognitive affliction is Creutzfeldt-Jakob Disease, a disease in which there are only about 350 known cases in the United States per year, and affects about one in a million people per year worldwide, making it pretty rare (National Institute of Neurological Disorders and Stroke, n.d). Though rare, the disease works rapidly and degenerates brain capability to be fatal in a relatively short amount of time (National Institute of Neurological Disorders and Stroke, n.d). 

Creutzfeldt-Jakob Disease is more common in the age range of sixty years old and above and is so severe that almost seventy percent of those afflicted die within the span of a year (National Institute of Neurological Disorders and Stroke, n.d). This destruction is caused by “prions which are misfolded prion proteins that build up in the brain and cause other prion proteins to misfold as well,” (NHS Choices, n.d.). In an article in Global Genes, a woman writes of her husband who was at first misdiagnosed with severe onset Alzheimer’s, and later found out that he was in fact battling Creutzfeldt-Jakob Disease. She says “I do believe there is hope, hope for all affected by these rare diseases but we must be diligent, passionate and willing to do anything we can to help those affected and their families,” after sharing her family’s story of caring for her husband.

With stories like that and with the quick statistics one can find on the disease, Creutzfeldt-Jakob Disease seems daunting and catastrophic. And while research on the disease is still in its infancy and there is no cure to date, scientists are working on how to combat and learn more about this disastrous disease. In News for Medical and Life Sciences, it was reported from a scientific journal that “National Institutes of Health scientists have used human skin cells to create what they believe is the first cerebral organoid system, or “mini-brain,” for studying sporadic Creutzfeldt-Jakob disease,” and with research like this, they expect to be able to find more ways to examine and create new therapeutics to treat Creutzfeldt-Jakob disease (Ives, 2019). The NIH also shared that “Researchers are examining and characterizing the prions associated with CJD and trying to discover factors that influence prion infectivity and transmission, and how the disorder damages the brain” (National Institute of Neurological Disorders and Stroke, n.d). New scientific strategies like this could open up many more methods of learning about neurodegenerative diseases. 

 

References

“Creutzfeldt-Jakob Disease Fact Sheet.” National Institute of Neurological Disorders and Stroke, U.S. Department of Health and Human Services, www.ninds.nih.gov/disorders/patient-caregiver-education/fact-sheets/creutzfeldt-jakob-disease-fact-sheet. 

NHS Choices, NHS, www.nhs.uk/conditions/creutzfeldt-jakob-disease-cjd/causes/. “A Shattered Life: The Last Days With Creutzfeldt-Jakob Disease.” Global Genes, 16 June 2016, globalgenes.org/2016/06/16/a-shattered-life-the-last-days-with-creutzfeldt-jakob-disease. 

Ives, Reviewed by James. “Scientists Create ‘Mini-Brain’ for Studying Sporadic Creutzfeldt-Jakob Disease.” News, 16 June 2019, www.news-medical.net/news/20190616/Scientists-create-mini-brain-for-studying-sporadic-Creut zfeldt-Jakob-disease.aspx.

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