Creutzfeldt-Jakob Disease: "Mad Cow" Disease just for Humans

Updated: Dec 5, 2018


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Creutzfeldt-Jakob Disease (CJD) is another degenerative form of dementia. Far more rare than the previously discussed forms of dementia in this series, it affects about 1:1,000,000 people each year. With the news, lately, you may have heard of squirrels, deer, and cows

suffering from what has become known as Mad Cow Disease. Well, think of CJD as the usually fatal Mad Cow Disease that affects humans. Once again, abnormal proteins that are the causative factor in this disease. These improperly folded proteins are called prions and multiply exponentially to block neuron functions and cause cell death.


How is CJD Different?

One big difference between other dementia and Creutzfeldt-Jakob Disease--CJD is

transmissible to other people, either by heredity, sporadic means, or acquired by exposure

to infected brain or nervous system tissue (usually through medical procedures, except in cases like the man who contracted it from eating an infected squirrel's brain). CJD is not contracted through casual contact between people, and there are safeguards in place globally to screen blood donations for the disease. Aside from contact or consuming the brain or nervous system cells of an infected animal, a variant of CJD may be acquired through eating meat from an infected animal. This is the mad cow disease that the news media reports and that cattle producers dread--a variant called bovine spongiform encephalopathy, or BSE.


Signs & Symptoms

Like some other dementia, Creutzfeldt-Jakob Disease (CJD) manifests in both psychological and physical behaviors. In both cases, CJD is a rapidly degenerative dementia. Psychological manifestations include:

  • Personality changes

  • Memory Loss

  • Hallucinations

  • Depression

  • Paranoia

  • OCD

Physical manifestations include:

  • Balance and coordination problems

  • Muscle rigidity

  • Speech impairment

  • Seizures

Treatment & Prognosis

There is no cure for CJD--treatment regimens focus solely on available ways to relieve the patient's symptoms and provide comfort, especially during the final stages of the disease. About that...the "final stage" comes quite rapidly after onset and diagnosis. The infected individual has an average life expectancy of around 6 months, although there have been cases where a patient suffering from CJD has lived 2-4 years--although the quality of life is not reflected in the "long-tern" numbers. Much like AIDS, patients usually die because of infections or other vulnerabilities caused by Creutzfeldt-Jakob Disease.


Lists in this article adapted from Earlstein, F. (2016). Dementia Facts & Information. NRB Publishing: Nevada.


For more resources and references on CJD, see the following:

Creutzfeldt-Jakob Disease News & Research: https://www.drcarlforkner.com/dementia-memory-news

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