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Creutzfeldt-Jakob Disease

brain agent patient cjd

Creutzfeldt-Jakob disease is a rare encephalopathy, or brain disease, that causes a swift, progressive dementia and neuromuscular changes. It was first described by German psychiatrist Alfons Maria Jakob (1884–1931) in 1921. He gave credit to Hans Gerhard Creutzfeldt (1885–1964), also a German psychiatrist, for describing the syndrome first without realizing he had stumbled onto a repeating set of symptoms that constitute a syndrome. Although it is now known that what Creutzfeldt described was not the same syndrome that Jakob had discovered, the disease retains its compound name.

Creutzfeldt-Jakob disease is the more common of two known human spongiform encephalopathies, the other being kuru. Both encephalopathies are thought to be caused by prions, infectious agents made up of genelacking proteins.

The early stages of Creutzfeldt-Jakob disease include symptoms similar to those of Alzheimer disease. Disturbances in vision and other senses, confusion, and inappropriate behavior patterns are the usual early signs. During the following months the patient will invariably progress first to dementia and then into a coma. Jerking movements of the arms and legs are common, and convulsions are less common. Muscle spasms and rigidity occur in the late stages of the disease. Usually, the patient will deteriorate and die in less than a year, although some will live for as long as two years.

Creutzfeldt-Jakob disease occurs throughout the world, usually equally among men and women at about age 60. Rarely do young people get the disease. Three forms of the disease have been identified, classified by virtue of transmission. About 10% of Creutzfeldt-Jakob patients in the United States have a positive family history of Creutzfeldt-Jakob disease (hereditary form). About 85% have no identifiable risk factors for the disease, and yet have developed Creutzfeldt-Jakob disease (sporadic form). Only 1% are thought to have contracted the disease through exposure to infective materials (acquired form).

There are no laboratory tests to help diagnose Creutzfeldt-Jakob disease. Many tests which are performed when a patient is suspected of having Creutzfeldt-Jakob disease are done to make sure that the patient does not have some other, potentially treatable disease. A brain scan using magnetic resonance imaging (MRI) will usually show the degeneration of the cerebrum and enlargement of the brain ventricles (fluid-filled openings in the center of the brain) which characterize encephalopathy. A definitive diagnosis can be made only when a specimen (biopsy) of the brain tissue is stained and studied under a microscope. Biopsying the brain, however, is a highly invasive procedure and is rarely done to diagnose Creutzfeldt-Jakob disease, as information gained from the biopsy does not lead to any change in treatment. Other neurological measurements are less directly correlated to diagnosing the disease. The electroencephalogram (which monitors the pattern of electric brain waves) may show certain repeated signs, but not always. The cerebrospinal fluid (which fills spaces in the brain and surrounds the spinal cord) may have an elevated level of protein when tested; this measurement is currently being investigated to determine whether the presence or elevation of specific proteins is diagnostic of this disease. The changes seen in brain tissue are not found in any organ outside the central nervous system. The etiologic agent that causes the disease can be found in other organs, but evidently it has no effect on their structure or function.

The etiologic agent of Creutzfeldt-Jakob disease has unique characteristics. The agent can withstand heat, ionizing radiation, and ultraviolet light—immunities that documented viruses do not possess. Most scientists consider the agent to be a unique, nonviral pathogen that does not contain any DNA or RNA, the nucleic acids containing the reproductive code for any organism. Thus, a new theory emerged and gained widespread acceptance and the infectious agent is now though to be an unconventional proteinaceous particle called a "prion", ("proteinaceous infectious agent). Prion proteins are believed to be proteins that are able to exist in two forms: a normal form, performing some unknown but presumably necessary physiological function in the host, and an abnormal, infectious form, which can cause the disease. When a substantial amount of the normal host protein is converted to the abnormal form (by processes which are not understood fully to date) prion disease can ensue.

The means of transmitting this disease is unknown. A case has been documented in which a patient who received a transplanted cornea from the eye of a person with Creutzfeldt-Jakob disease also developed the disease. Another person contracted the disease when electrodes previously used on an infected patient were used in an electroencephalogram. Even the spread of the disease within families is the result of a mysterious mechanism. It is not always seen and may be the result of more than one family member having a genetic predisposition to the disease.

There is no cure for Creutzfeldt-Jakob disease. Symptoms are treated to make the patients more comfortable, but nothing has been found that will interfere with the progress of the disease or kill the causal agent. In fact, very special precautions are required around these patients because the means of spreading the disease is not known. Only three means of sterilization of instruments or other objects that have touched the patient are known—lye, bleach, and intense heat. It is recommended that clothing and bed linens of the patient be burned, as washing will not kill the etiologic agent.

Researchers are very interested in more clearly identifying the infective agent, and defining its characteristics and the methods of transmission. Concern has been raised regarding the chance that the disease could potentially be passed through contaminated blood transfusions. No such case has been identified, but the issue has been raised. Contaminated beef has been held resonsible for a related disease called bovine spongiform encephalopathy, leading researchers to question whether Creutzfeldt-Jakob disease could also be spread through contaminated food products.

Recently in the United Kingdom, there have been several unusual cases of CJD in younger people. These cases surfaced in 1994 and 1995, with a disease that differed significantly from classical sporadic CJD and has been termed variant CJD (vCJD). The patients were all under the age of 42, with an average age of 28, as opposed to the typical age of 63 for classical CJD. The average course of the duration of vCJD was 13 months, in contrast to the average four to six month duration for classical CJD. The electroencephalographic (EEG) electrical activity in the brain for vCJD was different from classical CJD. While the brain pathology of the vCJD cases was identifiable as CJD, the pattern differed because of the presence of large aggregates of prion protein plaques. Scientists ascertained a likely connection between this new form of CJD and bovine spongiform encephalopathy (BSE), a prion disease in cattle, which reached epidemic proportions in the 1980s in the United Kingdom. There have now been over 100 cases of vCJD, most of which have occurred in the United Kingdom, and the numbers are still rising. A few cases of vCJD have been found in France, Ireland, Italy, and Hong Kong. Because the incubation period between exposure to the agent and the onset of symptoms may be as long as 40 years, it is uncertain whether these vCJD cases may signal the beginning of an epidemic or whether the incidence of vCJD will remain low.

Creutzfeldt-Jakob disease remains a medical mystery, since scientists are not yet certain about its means of transmission, its cure, or its prevention. Much work is being done to collect brain, tissue, and body fluid samples from all sufferers, in order to advance research into the disease.

See also Neuroscience.


Resources

Books

Margulies, Phillip. Creutzfeldt-Jacob Disease (Epidemics). New York: Rosen Publishers, 2003.

Prusiner, Stanley B. Prion Biology and Diseases. Cold Spring Harbor, NY: Cold Spring Harbor Laboratory Press, 1999.

Ratzan, Scott C., ed. Mad Cow Crisis: Health and the Public Good New York: New York University Press, 1998.


Organizations

Creutzfeldt-Jakob Disease Foundation, Inc. P.O. Box 5312 Akron, Ohio 44334. <http://www.cjdfoundation.org>.


Larry Blaser

KEY TERMS

. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

Cerebrum

—The upper, main part of the human brain, it is made up of the two hemispheres, and is the most recent brain structure to have evolved. It is involved in higher cognitive functions such as reasoning, language, and planning.

Encephalopathy

—Any abnormality in the structure or function of the brain.

Etiologic agent

—The cause of a disease. An etiologic agent could be a bacterium, a virus, a parasite, or some other factor.

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28 days ago

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29 days ago

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about 1 month ago

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about 1 month ago

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4 months ago


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5 months ago



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6 months ago

Hi,
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6 months ago

Hi,
My name is Sarah Anik, some months ago I had s*x with the third guy in my life whom i was about to marry and now I have herpes. I couldn’t believe it because he wasn’t a random guy, he’s the closet guy I’ve had as a boyfriend, but it has been a never ending nightmare since. I’ve always believed in GOD and have asked him for forgiveness which is ironic.
I felt like killing myself with the taught of having a husband and family will be quite difficult and i felt like I’m not really in the room when people are talking. I didn’t know what to do and pretty much cry and pray every day to God for help and looking at my family with so much love and feeling that i have disappointed them even though they don’t know. Until God lead me to DR. SOLUTION online who promised to help me cure my disease through herbals. At first i didn't believe him, but to my greatest surprise i was cured completely by herbals, which medical doctor also verified that i was cured. Special thanks to DR. SOLUTION.
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Vote down Vote up

6 months ago

Hi,
My name is Sarah Anik, some months ago I had s*x with the third guy in my life whom i was about to marry and now I have herpes. I couldn’t believe it because he wasn’t a random guy, he’s the closet guy I’ve had as a boyfriend, but it has been a never ending nightmare since. I’ve always believed in GOD and have asked him for forgiveness which is ironic.
I felt like killing myself with the taught of having a husband and family will be quite difficult and i felt like I’m not really in the room when people are talking. I didn’t know what to do and pretty much cry and pray every day to God for help and looking at my family with so much love and feeling that i have disappointed them even though they don’t know. Until God lead me to DR. SOLUTION online who promised to help me cure my disease through herbals. At first i didn't believe him, but to my greatest surprise i was cured completely by herbals, which medical doctor also verified that i was cured. Special thanks to DR. SOLUTION.
Don't be discourage because he that did it for me will also do it for you and you can contact him through his email address solutionhealinghome@gmail.com or text him on what'sapp, phone number +2348077683109 or visit his website www.healinghome.webs.com for more information.

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7 months ago

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8 months ago


ZIKA DISEASE

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9 months ago

Am Susan from Sweden I want to share a testimony on how this great man Dr Ekpiku help me to heal my herpes virus is the solution to herpes cure,Since last 4 months I have being a Herpes patient.it was confirm I have be in several hospital but no one can cure it one day I was browsing through the Internet I saw a testimony on how Dr Ekpiku from Africa healer and i saw so many testimony on blogger how is cure a lady called Grace and i asked her if she was cure by Dr Ekpiku,what she said was yes that is heal and be free for herpes,she told me that there is a great spell caster that can cure my herpes Virus,I asked her if she had his email, she gave me his email, so I contact him and i told him I have Herpes virus,and I asked for solutions, he started the herbal remedy for my health, he sent me the medicine,and i start taking the medicine as prescribed by him and 2 week later i was cured from HERPES, Dr. Ekpiku truly you are great healer, do you need his help also? Why don't you contact him through ekpikuspellhomeofgrace@gmail.com or his cell number +2348073673757,I’m the happiest girl in the world thank you Dr Ekpiku he cure any type of disease HIV/AIDS,CANCER,diabetes,ALS, Hepatitis B, HERPES, PVC/ through his via email;ekpikuspellhomeofgrace@hotmail.com.

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10 months ago

Hello everyone in this room, my name is millat mellisa I am from Georgia, December last year I was very ill , my daughter took me to the hospital and the doctor said I had cancer that I have few months to live. I as a woman with big dreams, am not ready to die, I search through the internet for a possible cure and I saw testimonies about Dr Ogala , I contacted him and he sent me some herbs that I used , few months later I went for a test at the hospital and cancer disappear , thanks to you Dr Ogala , if you have any one with this sickness , contact Dr Ogala he has the cure email: holyspellcast@yahoo.com

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11 months ago

Please there is a cure for dementia and no one knows. This is my husband’s story... My husband was a retired force man, he had partial stroke 5 years ago, and then i was still working. His condition got worse and he was hospitalized. After some time of treatment he began to behave abnormal, like someone who has lost memory of event and occurrence. i shared tears the very day i walked to him in the hospital and he could not even recognize me. This is am man that is very playful and highly romantic. Each day i hold him to myself and share tears the more. He was not able to do anything just look at me like a bull dog and do so many abnormal things. Later on doctor brought a recent report and he was diagnosed of dementia this doctor says have no cure that he would die a brainless man. Oh my God i fainted when i heard this. I raised my voice and cry to God to help me from this. So i began to hope and trust God to do miracle. My pastor was a great pillar of encouragement to me. i did all i can to get a cure for him, but none worked out, i spent thousands of USD trying to get a cure for him. But behold a friends doctor came visiting and said to me let’s look if herbal remedy can help him get any better so he gave me your website which is www. Drisimhenmhenherbalhome.com there i saw how people testified of the good works of herbs in their life, so i decided to contact the doctor because his contact details was on his website.. The first time i emailed him, i got no reply from him, so i decided to call him on the phone. I called him, and told him what he could do was to laugh and said to me that anything the enemy have planned against my husband must fail, he said to me that he have cured Creutzfeldt-Jakob Disease and this is exactly what my husband is suffering from. After all, he prepared herbs and sent to me, i have to force my husband to take it because before then he was not taking anything from me to eat anymore. but behold sir it was just like a magic gradually my husband begin to get better, better and today sir i am proud to say my husband is totally cured, He you cured my husband of this deadly dementia disease and now i keep my promise i made to him before the cure that i will let the entire world know about his herbs if my husband is cured. Sir, now i am overwhelmed. God bless you sir. My husband has the plans to travel to see the doctor facially and say a physical thanks to him in his country, and i will accompany him down to his home in his country to also show my gratitude him. God bless him in abundant. If you have same problem or have other health issues and want to contact Doctor Isimhenmhen Anthony, here is his website (http://drisimhenmhenherbalhome.com)

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12 months ago

I am a happy man today. Please I want all in this page to thank God and Doctor Uwadia Amenifo for me. I have a cousin who has for long suffered from Creutzfeldt - Jacob disease he was almost giving up the ghost, we tried many means to get him treated, but none work out. Even his doctor said to him that he cannot be cured that he will die in the pains and dementia. So we became very worried. So on a faithful day I search on the internet I don’t know how it happened I saw so many persons testifying of a man who cured them of various ailment so i got in contact with the herbal Doctor Uwadia Amenifo after I explained to him what my cousin living with me is passing through, he assured me that my cousin will be well and be cured of CJD if he can get his herbal mixture. So he gave us counseling and told us that there are some herbs he will prepare and send to my cousin, so after all necessary arrangement he was sending herbs and roots to my cousin. After some time my cousin begin to improve, and to God be the glory today he is healthy. So please I want you all to help me say a big thanks to Doctor Uwadia Amenifo for his help and treatment. My beloved, today my cousin is free from this Creutzfeldt - Jacob disease. So please if there is any one here who is suffering from same Creutzfeldt - Jacob disease I want to say a cure has come I strongly recommend you to Doctor Uwadia Amenifo. You can reach Doctor Uwadia Amenifo in his email doctoruwadiaamenifo@gmail.com, or you can also call him on +2349052015874. Please contact him now and get healed from those diseases. Thanks to you all

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over 1 year ago

I am very happy today to testify the goodness of God in my life. I want you all here to be grateful to Doctor Uwadia Amenifo for me; he is indeed a wonderful man. Just last month i read some testimonies from the internet by a man named "James Watt" he testified of how his cousin was cured of Creutzfeldt - Jakob disease by a herbal doctor. At first i doubted because i have been suffering from the said disease for over five years, and my doctor said there is no cure for it. But i was under medication as my life was dwindling by the day. But after i read his testimony, he included the contact details of the herbal doctor so i emailed the doctor and explained to him how i have suffered from Creutzfeldt - Jakob disease for over 5 years. But he assured me that he can cure me with his herbs and told me how he have cured so many of the said disease, well i accepted what he said to me, and followed all the procedures he gave me. after all, he prepared the herbs and sent it to me here in Australia i took it as prescribed just after three weeks, there was tremendous improvement in my state of health, and my doctor begin to wonder how greatly i am re covering. And just in the fourth week of taking the herbal mixture, i became totally healthy, and today i am perfectly OK, and free from Creutzfeldt - Jakob disease. So i contacted Doctor uwadia Amenifo and told him that I am ok now and he congratulated me, and asked me to help him tell the world about his herbs. Please i implore you all that have same or similar disease to have hope that a remedy have come. Please if you wish to contact Doctor Uwadia Amenifo, here is his contact details email (doctoruwadiaamenifo@gmail.com) or you can also reach him on his cell phone number (+2349052015874). So I want you all to please be happy for me, and also try to contact him if you have any health issues.

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over 1 year ago

I am a happy man today. Please I want all in this page to thank God and Doctor Uwadia Amenifo for me. I have a cousin who has for long suffered from Creutzfeldt - Jakob disease he was almost giving up the ghost, but I don’t know how it happened we got in contact with Doctor Uwadia Amenifo and he assured us that my cousin will be well. So he gave us counseling and told us that there are some herbs he will prepare and send to my cousin, so after all necessary arrangement he was sending herbs and roots to my cousin. After some time my cousin begin to improve, and to God be the glory today he is healthy. so please I want you all to help me say a big thank you to Doctor Uwadia Amenifo for his help and treatment. My beloved , today my cousin is free from this Creutzfeldt - Jakob disease. So please if there is any one here who is suffering from same Creutzfeldt - Jakob disease i want to say a cure has come I strongly recommend you to Doctor Uwadia Amenifo. You can reach Doctor Uwadia Amenifo in his email doctoruwadiaamenifo@gmail.com, or you can also call him on +2349052015874. Please contact him now and get healed from those diseases. Thanks to you all