BACK TOTOP Browse A-ZSearchBrowse A-ZABCDEFGHIJKLMNOPQRSTUVWXYZ0-9 E-mail FormEmail ResultsName:Email address:Recipients Name:Recipients address:Message: Print-FriendlyBookmarksbookmarks-menuCreutzfeldt-Jakob diseaseTransmissible spongiform encephalopathy; vCJD; CJD; Jacob-Creutzfeldt diseaseCreutzfeldt-Jakob disease (CJD) is a form of brain damage that leads to a rapid decrease in movement and loss of mental function. Causes CJD is caused by a protein called a prion. A prion causes normal proteins to fold abnormally. This affects other proteins' ability to function.ProteinProteins are the building blocks of life. Every cell in the human body contains protein. The basic structure of protein is a chain of amino acids. ...ImageRead Article Now Book Mark Article CJD is very rare. There are several types. The classic types of CJD are:Sporadic CJD makes up most cases. It occurs for no known reason. It starts on average at age 65 years.Familial CJD occurs when a person inherits the abnormal prion from a parent (this form of CJD is rare).Acquired CJD includes variant CJD (vCJD), the form related to mad cow disease.Variant CJD is caused by eating infected meat. The infection that causes the disease in cows is thought to be the same one that causes vCJD in humans. Variant CJD causes less than 1% of all CJD cases. It tends to affect younger people. Fewer than 200 people worldwide have had this disease. Almost all cases occurred in England and France. Iatrogenic CJD is also an acquired form of the disease. Iatrogenic CJD is sometimes passed through a blood product transfusion, transplant, or contaminated surgical instruments.CJD may be related to several other diseases caused by prions, including:Chronic wasting disease (found in deer) Kuru (affected mostly women in New Guinea who ate the brains of dead relatives as part of a funeral ritual) KuruKuru is a disease of the nervous system.ImageRead Article Now Book Mark Article Scrapie (found in sheep) Other very rare inherited human diseases, such as Gerstmann-Straussler-Scheinker disease and fatal familial insomnia Symptoms CJD symptoms may include any of the following:Dementia that gets worse quickly over a few weeks or months DementiaDementia is a loss of brain function that occurs with certain diseases. It affects one or more brain functions such as memory, thinking, language, j...ImageRead Article Now Book Mark Article Blurred vision (sometimes) Changes in gait (walking) Changes in gaitWalking abnormalities can be caused by many different types of problems. Problems with the joints, (such as arthritis), bones (such as deformities),...ImageRead Article Now Book Mark Article Confusion, disorientation ConfusionConfusion is the inability to think as clearly or quickly as you normally do. You may feel disoriented and have difficulty paying attention, remembe...ImageRead Article Now Book Mark Article Hallucinations (seeing or hearing things that aren't there) Lack of coordination (for example, stumbling and falling) Muscle stiffness, twitching Feeling nervous, jumpy Personality changes Sleepiness Sudden jerky movements or seizures Trouble speaking Exams and Tests Early in the disease, a nervous system and mental exam will show memory and thinking problems. Later in the disease, a motor system exam (to test muscle reflexes, strength, coordination, and other physical functions) may show:Abnormal reflexes or increased normal reflex responses Increase in muscle tone Muscle twitching and spasms Strong startle response Weakness and loss of muscle tissue (muscle wasting)Muscle wastingMuscle atrophy is the wasting (thinning) or loss of muscle tissue.ImageRead Article Now Book Mark Article Loss of coordination occurs due to changes in the cerebellum. This is the area of the brain that controls coordination.An eye exam shows areas of blindness that the person may not notice.Tests used to diagnose this condition may include:Blood tests to rule out other forms of dementia and to look for markers that may occur with the disease CT scan of the brain CT scan of the brainA head computed tomography (CT) scan uses many x-rays to create pictures of the head, including the skull, brain, eye sockets, and sinuses.ImageRead Article Now Book Mark Article Electroencephalogram (EEG) ElectroencephalogramAn electroencephalogram (EEG) is a test to measure the electrical activity of the brain.ImageRead Article Now Book Mark Article MRI of the brain MRI of the brainA head MRI (magnetic resonance imaging) is an imaging test that uses powerful magnets and radio waves to create pictures of the brain and surrounding...ImageRead Article Now Book Mark Article Spinal tap to test for the abnormal prion protein or for a protein called 14-3-3 in the spinal fluidSpinal tapCerebrospinal fluid (CSF) collection is a test to look at the fluid that surrounds the brain and spinal cord. CSF acts as a cushion, protecting the b...ImageRead Article Now Book Mark Article The disease can only be confirmed with a brain biopsy or autopsy. Today, it is very rare for a brain biopsy to be done to look for this disease. Treatment There is no cure for this condition. Different medicines have been tried to slow the disease, but none works well.The goals of treatment include:Provide a safe environment Control aggressive or agitated behavior Make the person comfortable Meet the person's needsThis may require monitoring and assistance in the home or in a care facility. Family counseling may help the family cope with the changes needed for home care.People with this condition may need help controlling unacceptable or dangerous behaviors. This involves rewarding positive behaviors and ignoring negative behaviors (when it is safe). They may also need help getting oriented to their surroundings. Sometimes, medicines are needed to help control aggression.It is helpful for people with CJD and their family to seek legal advice early on. Advance directive, power of attorney, and other legal actions can make it easier to make decisions about the care of the person with CJD.Advance directiveWhen you are very ill or injured, you may not be able to make health care choices for yourself. If you are unable to speak for yourself, your health...ImageRead Article Now Book Mark Article Outlook (Prognosis) The outcome of CJD is very poor. People with sporadic CJD are unable to care for themselves within 6 months or less after symptoms begin.The disorder is fatal in a short time, usually within 8 months. People who have variant CJD get worse more slowly, but the condition is still fatal. A few people survive for as long as 1 or 2 years. The cause of death is usually infection, heart failure, or respiratory failure.The course of CJD is:Infection with the disease Severe malnutrition Dementia in some cases Loss of ability to interact with others Loss of ability to function or care for oneself Death When to Contact a Medical Professional CJD is not a medical emergency. However, early diagnosis and treatment may make the symptoms easier to control, give patients time to make advance directives and prepare for the end of life, and give families extra time to come to terms with the condition. Prevention Medical equipment that may be contaminated should be removed from service and disposed of. People known to have CJD should not donate a cornea or other body tissue.Most countries now have strict guidelines for managing infected cows to avoid passing CJD to humans.Open ReferencesReferencesBosque PJ, Tyler KL. Prions and prion disease of the central nervous system (transmissible neurodegenerative diseases). In: Bennett JE, Dolin R, Blaser MJ, eds. Mandell, Douglas, and Bennett's Principles and Practice of Infectious Diseases. 9th ed. Philadelphia, PA: Elsevier; 2020:chap 179.Tee BL, Geschwind MD. Prion diseases. In: Jankovic J, Mazziotta JC, Pomeroy SL, Newman NJ, eds. Bradley and Daroff's Neurology in Clinical Practice. 8th ed. Philadelphia, PA: Elsevier; 2022:chap 94.AllVideoImagesTogCreutzfeldt-Jakob disease - illustration Creutzfeldt-Jakob disease is an organic brain syndrome caused by a protein-like particle called a prion. Loss of brain function resembles Alzheimer's disease, but is very rapid in progression. Complete dementia usually occurs by the sixth month, death follows quickly. There is no known cure. Creutzfeldt-Jakob diseaseillustrationCentral nervous system and peripheral nervous system - illustration The central nervous system comprises the brain and spinal cord. The peripheral nervous system includes nerves outside the brain and spinal cord.Central nervous system and peripheral nervous systemillustrationCreutzfeldt-Jakob disease - illustration Creutzfeldt-Jakob disease is an organic brain syndrome caused by a protein-like particle called a prion. Loss of brain function resembles Alzheimer's disease, but is very rapid in progression. Complete dementia usually occurs by the sixth month, death follows quickly. There is no known cure. Creutzfeldt-Jakob diseaseillustrationCentral nervous system and peripheral nervous system - illustration The central nervous system comprises the brain and spinal cord. The peripheral nervous system includes nerves outside the brain and spinal cord.Central nervous system and peripheral nervous systemillustrationRelated Information Protein in diet(Nutrition)Growth hormone test(Medical Test)Kuru(Condition)Encephalitis(Condition)Viral encephalitis - InDepth(In-Depth) Review Date: 8/28/2023 Reviewed By: Joseph V. Campellone, MD, Department of Neurology, Cooper Medical School at Rowan University, Camden, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. No warranty of any kind, either expressed or implied, is made as to the accuracy, reliability, timeliness, or correctness of any translations made by a third-party service of the information provided herein into any other language. © 1997- A.D.A.M., a business unit of Ebix, Inc. 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Creutzfeldt-Jakob diseaseTransmissible spongiform encephalopathy; vCJD; CJD; Jacob-Creutzfeldt diseaseCreutzfeldt-Jakob disease (CJD) is a form of brain damage that leads to a rapid decrease in movement and loss of mental function. Causes CJD is caused by a protein called a prion. A prion causes normal proteins to fold abnormally. This affects other proteins' ability to function.ProteinProteins are the building blocks of life. Every cell in the human body contains protein. The basic structure of protein is a chain of amino acids. ...ImageRead Article Now Book Mark Article CJD is very rare. There are several types. The classic types of CJD are:Sporadic CJD makes up most cases. It occurs for no known reason. It starts on average at age 65 years.Familial CJD occurs when a person inherits the abnormal prion from a parent (this form of CJD is rare).Acquired CJD includes variant CJD (vCJD), the form related to mad cow disease.Variant CJD is caused by eating infected meat. The infection that causes the disease in cows is thought to be the same one that causes vCJD in humans. Variant CJD causes less than 1% of all CJD cases. It tends to affect younger people. Fewer than 200 people worldwide have had this disease. Almost all cases occurred in England and France. Iatrogenic CJD is also an acquired form of the disease. Iatrogenic CJD is sometimes passed through a blood product transfusion, transplant, or contaminated surgical instruments.CJD may be related to several other diseases caused by prions, including:Chronic wasting disease (found in deer) Kuru (affected mostly women in New Guinea who ate the brains of dead relatives as part of a funeral ritual) KuruKuru is a disease of the nervous system.ImageRead Article Now Book Mark Article Scrapie (found in sheep) Other very rare inherited human diseases, such as Gerstmann-Straussler-Scheinker disease and fatal familial insomnia Symptoms CJD symptoms may include any of the following:Dementia that gets worse quickly over a few weeks or months DementiaDementia is a loss of brain function that occurs with certain diseases. It affects one or more brain functions such as memory, thinking, language, j...ImageRead Article Now Book Mark Article Blurred vision (sometimes) Changes in gait (walking) Changes in gaitWalking abnormalities can be caused by many different types of problems. Problems with the joints, (such as arthritis), bones (such as deformities),...ImageRead Article Now Book Mark Article Confusion, disorientation ConfusionConfusion is the inability to think as clearly or quickly as you normally do. You may feel disoriented and have difficulty paying attention, remembe...ImageRead Article Now Book Mark Article Hallucinations (seeing or hearing things that aren't there) Lack of coordination (for example, stumbling and falling) Muscle stiffness, twitching Feeling nervous, jumpy Personality changes Sleepiness Sudden jerky movements or seizures Trouble speaking Exams and Tests Early in the disease, a nervous system and mental exam will show memory and thinking problems. Later in the disease, a motor system exam (to test muscle reflexes, strength, coordination, and other physical functions) may show:Abnormal reflexes or increased normal reflex responses Increase in muscle tone Muscle twitching and spasms Strong startle response Weakness and loss of muscle tissue (muscle wasting)Muscle wastingMuscle atrophy is the wasting (thinning) or loss of muscle tissue.ImageRead Article Now Book Mark Article Loss of coordination occurs due to changes in the cerebellum. This is the area of the brain that controls coordination.An eye exam shows areas of blindness that the person may not notice.Tests used to diagnose this condition may include:Blood tests to rule out other forms of dementia and to look for markers that may occur with the disease CT scan of the brain CT scan of the brainA head computed tomography (CT) scan uses many x-rays to create pictures of the head, including the skull, brain, eye sockets, and sinuses.ImageRead Article Now Book Mark Article Electroencephalogram (EEG) ElectroencephalogramAn electroencephalogram (EEG) is a test to measure the electrical activity of the brain.ImageRead Article Now Book Mark Article MRI of the brain MRI of the brainA head MRI (magnetic resonance imaging) is an imaging test that uses powerful magnets and radio waves to create pictures of the brain and surrounding...ImageRead Article Now Book Mark Article Spinal tap to test for the abnormal prion protein or for a protein called 14-3-3 in the spinal fluidSpinal tapCerebrospinal fluid (CSF) collection is a test to look at the fluid that surrounds the brain and spinal cord. CSF acts as a cushion, protecting the b...ImageRead Article Now Book Mark Article The disease can only be confirmed with a brain biopsy or autopsy. Today, it is very rare for a brain biopsy to be done to look for this disease. Treatment There is no cure for this condition. Different medicines have been tried to slow the disease, but none works well.The goals of treatment include:Provide a safe environment Control aggressive or agitated behavior Make the person comfortable Meet the person's needsThis may require monitoring and assistance in the home or in a care facility. Family counseling may help the family cope with the changes needed for home care.People with this condition may need help controlling unacceptable or dangerous behaviors. This involves rewarding positive behaviors and ignoring negative behaviors (when it is safe). They may also need help getting oriented to their surroundings. Sometimes, medicines are needed to help control aggression.It is helpful for people with CJD and their family to seek legal advice early on. Advance directive, power of attorney, and other legal actions can make it easier to make decisions about the care of the person with CJD.Advance directiveWhen you are very ill or injured, you may not be able to make health care choices for yourself. If you are unable to speak for yourself, your health...ImageRead Article Now Book Mark Article Outlook (Prognosis) The outcome of CJD is very poor. People with sporadic CJD are unable to care for themselves within 6 months or less after symptoms begin.The disorder is fatal in a short time, usually within 8 months. People who have variant CJD get worse more slowly, but the condition is still fatal. A few people survive for as long as 1 or 2 years. The cause of death is usually infection, heart failure, or respiratory failure.The course of CJD is:Infection with the disease Severe malnutrition Dementia in some cases Loss of ability to interact with others Loss of ability to function or care for oneself Death When to Contact a Medical Professional CJD is not a medical emergency. However, early diagnosis and treatment may make the symptoms easier to control, give patients time to make advance directives and prepare for the end of life, and give families extra time to come to terms with the condition. Prevention Medical equipment that may be contaminated should be removed from service and disposed of. People known to have CJD should not donate a cornea or other body tissue.Most countries now have strict guidelines for managing infected cows to avoid passing CJD to humans.Open ReferencesReferencesBosque PJ, Tyler KL. Prions and prion disease of the central nervous system (transmissible neurodegenerative diseases). In: Bennett JE, Dolin R, Blaser MJ, eds. Mandell, Douglas, and Bennett's Principles and Practice of Infectious Diseases. 9th ed. Philadelphia, PA: Elsevier; 2020:chap 179.Tee BL, Geschwind MD. Prion diseases. In: Jankovic J, Mazziotta JC, Pomeroy SL, Newman NJ, eds. Bradley and Daroff's Neurology in Clinical Practice. 8th ed. Philadelphia, PA: Elsevier; 2022:chap 94.