Creutzfeldt-Jakob Disease is a degenerative neurological disease that occurs in various forms. These forms are familial, sporadic, and acquired. This disease has to do with the PRNP gene, which controls the formation of the prion protein.
The first type of this disease is familial. Familial CJD occurs through a mutation in the PRNP gene, which encodes prion proteins, whose exact purpose is still unknown. -- There are over 50 different mutations known for the PRNP gene, but not all cause CJD -- It occurs in 5-15% of cases in the world --Libyan Jews are known to be predisposed to this type
The next type of CJD is sporadic. Sporadic CJD occurs through a spontaneous change in the protein folding of the prion protein. The cause of this is still uncertain. --85% of cases around the world --The most common type of CJD
Last Type of CJD is Acquired. -- To get this you MUST be infected with a prion protein (through consumption or surgery) --This form is rare (1% of cases worldwide)
What is a Prion? --A small infectious disease-causing agent (protein) that is believed to be the smallest infectious particle. A prion is neither bacterial nor fungal nor viral and contains no genetic material --Scientists don't know the real purpose for prions
CJD Tendencies: -- Affects older people (40-70 years old) -- Men and women are affected equally
Symptoms of CJD --Personality Changes --Anxiety --Depression --Memory Loss --Impaired Thinking --Blurred Vision --Insomnia --Difficulty Speaking --Difficulty Swallowing --Sudden, jerky movements --Death
Statistics: -- One in every million people are infected with CJD wroldwide -- About 300 cases in the U.S. every year
Brain turns into a “sponge-like” form, otherwise known as neuronal degeneration -- Prions replicate by converting normal protein forms to abnormal, they accumulate in the nerve cell (and as they replicate, more and more “form”) and cause the cell to degenerate --This protein clustering causes the cell to degenerate <br />
What Happens Next: -- Nerve cells begin to die creating visible holes in the brain. --These holes in the brain are what cause the &quot;symptoms&apos; those areas of the brain are dying --Once the brain areas degenerate, the patient dies
Diagnosis: -- Scientists are reluctant to perform the only true determinant of CJD - a brain biopsy -- So before they reach extremes, they do things like Lumbar Punctures (Spinal Tap), EEG, and MRI to get an idea -- If these come back looking positive for CJD, a brain biopsy is done
Treatment: -- There is no way to stop or even slow the progression of CJD; it is fatal 100% of the time -- 90% of those infected die within the first year -- Even if it was fixable, the neural degeneration that takes place before symptom onset would still be fairly devastating and irreversible so even if a treatment was created, it still would not solve all of the problems posed by this disease
Works Cited: 1) &quot;Creutzfeldt-Jakob Disease.&quot; Alzheimer&apos;s Disease and Dementia. N.p., n.d. Web. 10 Dec. 2014. 2) &quot;Creutzfeldt-Jakob Disease (CJD) (pathology).&quot; Encyclopedia Britannica Online. Encyclopedia Britannica, n.d. Web. 10 Dec. 2014. 3) &quot;Creutzfeldt-Jakob Disease.&quot; Definition. N.p., n.d. Web. 10 Dec. 2014. 4)&quot;Creutzfeldt-Jakob Disease: MedlinePlus.&quot; U.S National Library of Medicine. U.S. National Library of Medicine, n.d. Web. 10 Dec. 2014. 5) &quot;Home.&quot; Creutzfeldt-Jakob Disease Foundation. N.p., n.d. Web. 10 Dec. 2014.
Creutzfeldt-Jakob disease&apos;s effect on cellular communication is debated among scientists. However, it is agreed that neural degeneration causes a disruption in cell signaling, cell protection, and the formation of synapses of neurons. --Synapses are the spaces between neurons in which cell-to-cell communication occurs --Disruption in neuron communication leads to a disruption of communication between the Central Nervous System and the body