Creutzfeldt–Jakob disease (CJD) is a form of brain disorder which is both fatal and deteroirative. It's symptoms include behavioral changes, poor coordination, memory problems, and visual disturbances, dementia, involuntary movements, blindness, weakness, and coma occur. Statistics show that it claims the life of 90% of diagnosed patients within a year.
Causes
This is caused by an abnormal three-dimensional restructuring of the prion protein in the body, triggering the same folding pattern of the dominantly alpha helical regions into beta pleated sheets in the brain, which in turn destroys brain cells as a large quantity of insoluble protein are deposited in affected cells while the restructuring occurs, leaving the individual with a spongiform appearance in the affected brain. This damage leads to a sudden and rapid decline in the ability to think as well as involuntary muscle movements, confusion, difficulty with movement and sudden mood changes.
From records, up to 7.5% of diagonised cases are autosomal dominant inheritances from parent to offspring with a 50 % chance of passing the disease from parent to offspring, while others come as a resut of exposion to infected brain and spinal tissue.
An EU study determined that "87% of cases were sporadic, 8% genetic, 5% iatrogenic and less than 1% variant."
Source
Types
| Type | Age Range | Cause | Percentage of Cases |
|---|---|---|---|
| Sporadic CJD | 60 and 65 | Spontaneous: Implies that the change is sudden and occurs on it's own, possibly due to old age. | 85% |
| Familial CJD | 20 and 40 | Inheritance: Here parents pass the condition to their offspring. | 10 to 15% |
| Acquired CJD | 20 and 40 | Aquired CJD could come from any of the following: Infected instruments from Neurosugery, Tissue transplant and Human sourced growth hormones. Bovine spongiform encephalopathy ("mad cow disease") in consumed infected cattle products. | 1% |
Diagnosis
Due to the fact that the disease is rare, diagnosing might not produce accurate results as there's no completely reliable tests for it. This is because the symptoms are also related to other nervous disorders, meanwhile diagnosis could come in form of a Blood test, Brain CT, MRI or EEG Scan and Spinal fluid test.
Prevention
The risk of contracting CJD is still very low so there is really no cause for alarm for now, and this is due to the fact that only a very little amount of cases have been reported world wide. Sounds quite relieving right? Anyways, I'll still advice to be very careful with or possibly stay away from sick cattle.
Meanwhile, if you have a family history associated with this, you're adviced to see a genetics counselor, this will help you know your chances.
Hospitals and other medical bodies are adviced to use synthetic human growth hormones, destroy surgical instruments used for nervous tissue or brain related surgeries and also use single use tools for lumbr punctures.
Treatment
CJD is currently without a cure, all treatments administered to patient would be just to relieve the infected of symptoms and also to make him/her a little comfortable for the time left.
It is advised to administer anti depressants and pain killers to ease the pain. Some symtoms which may be treated include, ataxia (loss of physical co-ordination), urinary incontinence (loss of bladder control), bowel incontinence (loss of bowel control), dysphagia (difficulties with swallowing), dystonia ( spasms and stiffness in muscle), also help and support for blindness.
Reference
http://www.prionalliance.org/2013/12/02/what-are-human-prion-diseases/
https://www.nhs.uk/conditions/creutzfeldt-jakob-disease-cjd/
https://www.mayoclinic.org/diseases-conditions/creutzfeldt-jakob-disease/symptoms-causes/syc-20371226
https://medlineplus.gov/creutzfeldtjakobdisease.html
https://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_disease
https://www.alz.org/alzheimers-dementia/what-is-dementia/types-of-dementia/creutzfeldt-jakob-disease
https://www.aan.com/Guidelines/Home/GetGuidelineContent/567
https://www.nhs.uk/conditions/creutzfeldt-jakob-disease-cjd/treatment/
https://www.mayomedicallaboratories.com/test-catalog/Clinical+and+Interpretive/82528
https://www.ncbi.nlm.nih.gov/pubmed/9450766