Creutzfeldt-Jakob Disease Information Page

Creutzfeldt-Jakob Disease Information Page


What research is being done?

CJD is believed to be caused by an abnormal form of normal cellular proteins called prions. The normal prion protein is found throughout the body but is most abundant in the nervous system. The harmless and the infectious forms of the prion protein are nearly identical, but the infectious form takes a different folded shape than the normal protein. The abnormal proteins clump together and may lead to the nerve cell loss and other brain damage seen in CJD. Researchers are trying to discover factors that influence prion infectivity and how the disorder damages the brain. Other projects are examining how abnormal prions cross the protective blood-brain barrier and spread through the nervous system. Findings may identify new therapies to treat prion diseases.

 

Information from the National Library of Medicine’s MedlinePlus
Creutzfeldt-Jakob Disease

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What research is being done?

CJD is believed to be caused by an abnormal form of normal cellular proteins called prions. The normal prion protein is found throughout the body but is most abundant in the nervous system. The harmless and the infectious forms of the prion protein are nearly identical, but the infectious form takes a different folded shape than the normal protein. The abnormal proteins clump together and may lead to the nerve cell loss and other brain damage seen in CJD. Researchers are trying to discover factors that influence prion infectivity and how the disorder damages the brain. Other projects are examining how abnormal prions cross the protective blood-brain barrier and spread through the nervous system. Findings may identify new therapies to treat prion diseases.

 

Information from the National Library of Medicine’s MedlinePlus
Creutzfeldt-Jakob Disease

CJD is believed to be caused by an abnormal form of normal cellular proteins called prions. The normal prion protein is found throughout the body but is most abundant in the nervous system. The harmless and the infectious forms of the prion protein are nearly identical, but the infectious form takes a different folded shape than the normal protein. The abnormal proteins clump together and may lead to the nerve cell loss and other brain damage seen in CJD. Researchers are trying to discover factors that influence prion infectivity and how the disorder damages the brain. Other projects are examining how abnormal prions cross the protective blood-brain barrier and spread through the nervous system. Findings may identify new therapies to treat prion diseases.

 

Information from the National Library of Medicine’s MedlinePlus
Creutzfeldt-Jakob Disease


Definition
Definition
Treatment
Treatment
Prognosis
Prognosis
Clinical Trials
Clinical Trials
Organizations
Organizations
Publications
Publications
Definition
Definition

Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, fatal brain disorder. Onset of symptoms typically occurs at about age 60. There are three major categories of CJD:  sporadic (the most common form, in which people do not have any known risk factors for the disease); hereditary (in which the person has a family member with the disease and tests positive for a genetic mutation), and acquired (in which the disease is transmitted by exposure to brain and nervous system tissue, usually through certain medical procedures). A form called variant CJD can be acquired by eating meat from cattle affected by a disease similar to CJD, called bovine spongiform encephalopathy (commonly called “mad cow” disease). CJD cannot be transmitted through the air or through touching or most other forms of casual contact. Initial symptoms of CJD include problems with muscular coordination, personality changes including progressive and severe mental impairment, impaired vision that may lead to blindness, and involuntary muscle jerks called myoclonus. People eventually lose the ability to move and speak, and enter a coma. Tests that help in the diagnosis of CJD include electroencephalography (which records the brain's electrical pattern), detection of certain proteins in the fluid that surrounds the brain and spinal cord, and magnetic resonance imaging. The only way to confirm a diagnosis of CJD is by brain biopsy or autopsy. A brain biopsy is discouraged unless it is need to rule out a treatable disorder.

×
Definition

Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, fatal brain disorder. Onset of symptoms typically occurs at about age 60. There are three major categories of CJD:  sporadic (the most common form, in which people do not have any known risk factors for the disease); hereditary (in which the person has a family member with the disease and tests positive for a genetic mutation), and acquired (in which the disease is transmitted by exposure to brain and nervous system tissue, usually through certain medical procedures). A form called variant CJD can be acquired by eating meat from cattle affected by a disease similar to CJD, called bovine spongiform encephalopathy (commonly called “mad cow” disease). CJD cannot be transmitted through the air or through touching or most other forms of casual contact. Initial symptoms of CJD include problems with muscular coordination, personality changes including progressive and severe mental impairment, impaired vision that may lead to blindness, and involuntary muscle jerks called myoclonus. People eventually lose the ability to move and speak, and enter a coma. Tests that help in the diagnosis of CJD include electroencephalography (which records the brain's electrical pattern), detection of certain proteins in the fluid that surrounds the brain and spinal cord, and magnetic resonance imaging. The only way to confirm a diagnosis of CJD is by brain biopsy or autopsy. A brain biopsy is discouraged unless it is need to rule out a treatable disorder.

Treatment
Treatment

There is no treatment that can cure or control CJD, although studies of a variety of drugs are now in progress. Current treatment is aimed at alleviating symptoms and making the person as comfortable as possible. Opiate drugs can help relieve pain, and the drugs clonazepam and sodium valproate may help relieve involuntary muscle jerks. Intravenous fluids and artificial feeding may be needed in later stages of the disease.

×
Treatment

There is no treatment that can cure or control CJD, although studies of a variety of drugs are now in progress. Current treatment is aimed at alleviating symptoms and making the person as comfortable as possible. Opiate drugs can help relieve pain, and the drugs clonazepam and sodium valproate may help relieve involuntary muscle jerks. Intravenous fluids and artificial feeding may be needed in later stages of the disease.

Definition
Definition

Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, fatal brain disorder. Onset of symptoms typically occurs at about age 60. There are three major categories of CJD:  sporadic (the most common form, in which people do not have any known risk factors for the disease); hereditary (in which the person has a family member with the disease and tests positive for a genetic mutation), and acquired (in which the disease is transmitted by exposure to brain and nervous system tissue, usually through certain medical procedures). A form called variant CJD can be acquired by eating meat from cattle affected by a disease similar to CJD, called bovine spongiform encephalopathy (commonly called “mad cow” disease). CJD cannot be transmitted through the air or through touching or most other forms of casual contact. Initial symptoms of CJD include problems with muscular coordination, personality changes including progressive and severe mental impairment, impaired vision that may lead to blindness, and involuntary muscle jerks called myoclonus. People eventually lose the ability to move and speak, and enter a coma. Tests that help in the diagnosis of CJD include electroencephalography (which records the brain's electrical pattern), detection of certain proteins in the fluid that surrounds the brain and spinal cord, and magnetic resonance imaging. The only way to confirm a diagnosis of CJD is by brain biopsy or autopsy. A brain biopsy is discouraged unless it is need to rule out a treatable disorder.

Treatment
Treatment

There is no treatment that can cure or control CJD, although studies of a variety of drugs are now in progress. Current treatment is aimed at alleviating symptoms and making the person as comfortable as possible. Opiate drugs can help relieve pain, and the drugs clonazepam and sodium valproate may help relieve involuntary muscle jerks. Intravenous fluids and artificial feeding may be needed in later stages of the disease.

Prognosis
Prognosis

CJD is rapidly progressive. About 70 percent of individuals die within one year. In the early stages of disease, people may have failing memory, behavioral changes, lack of coordination and visual disturbances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities, and coma may occur.

x

CJD is rapidly progressive. About 70 percent of individuals die within one year. In the early stages of disease, people may have failing memory, behavioral changes, lack of coordination and visual disturbances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities, and coma may occur.

Prognosis
Prognosis

CJD is rapidly progressive. About 70 percent of individuals die within one year. In the early stages of disease, people may have failing memory, behavioral changes, lack of coordination and visual disturbances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities, and coma may occur.

Definition

Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, fatal brain disorder. Onset of symptoms typically occurs at about age 60. There are three major categories of CJD:  sporadic (the most common form, in which people do not have any known risk factors for the disease); hereditary (in which the person has a family member with the disease and tests positive for a genetic mutation), and acquired (in which the disease is transmitted by exposure to brain and nervous system tissue, usually through certain medical procedures). A form called variant CJD can be acquired by eating meat from cattle affected by a disease similar to CJD, called bovine spongiform encephalopathy (commonly called “mad cow” disease). CJD cannot be transmitted through the air or through touching or most other forms of casual contact. Initial symptoms of CJD include problems with muscular coordination, personality changes including progressive and severe mental impairment, impaired vision that may lead to blindness, and involuntary muscle jerks called myoclonus. People eventually lose the ability to move and speak, and enter a coma. Tests that help in the diagnosis of CJD include electroencephalography (which records the brain's electrical pattern), detection of certain proteins in the fluid that surrounds the brain and spinal cord, and magnetic resonance imaging. The only way to confirm a diagnosis of CJD is by brain biopsy or autopsy. A brain biopsy is discouraged unless it is need to rule out a treatable disorder.

Treatment

There is no treatment that can cure or control CJD, although studies of a variety of drugs are now in progress. Current treatment is aimed at alleviating symptoms and making the person as comfortable as possible. Opiate drugs can help relieve pain, and the drugs clonazepam and sodium valproate may help relieve involuntary muscle jerks. Intravenous fluids and artificial feeding may be needed in later stages of the disease.

Prognosis

CJD is rapidly progressive. About 70 percent of individuals die within one year. In the early stages of disease, people may have failing memory, behavioral changes, lack of coordination and visual disturbances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities, and coma may occur.

What research is being done?

CJD is believed to be caused by an abnormal form of normal cellular proteins called prions. The normal prion protein is found throughout the body but is most abundant in the nervous system. The harmless and the infectious forms of the prion protein are nearly identical, but the infectious form takes a different folded shape than the normal protein. The abnormal proteins clump together and may lead to the nerve cell loss and other brain damage seen in CJD. Researchers are trying to discover factors that influence prion infectivity and how the disorder damages the brain. Other projects are examining how abnormal prions cross the protective blood-brain barrier and spread through the nervous system. Findings may identify new therapies to treat prion diseases.

 

Information from the National Library of Medicine’s MedlinePlus
Creutzfeldt-Jakob Disease

Patient Organizations
Alzheimer's Association
225 North Michigan Avenue.
Floor 17
Chicago
IL
Chicago, IL 60601-7633
Tel: 312-335-8700; 800-272-3900 (24-Hour Helpline); 312-335-5886 (TDD)
Centers for Disease Control and Prevention (CDC)
U.S. Department of Health and Human Services
1600 Clifton Road
Atlanta
GA
Atlanta, GA 30333
Tel: 800-311-3435; 404-639-3311; 404-639-3543
CJD Aware!
2527 South Carrollton Ave.
New Orleans
LA
New Orleans, LA 70118-3013
Tel: 504-861-4627
Creutzfeldt-Jakob Disease (CJD) Foundation Inc.
3610 W. Market Street, Suite 110
Akron
OH
Akron, OH 44333
Tel: 800-659-1991
Department of Agriculture (USDA)
National Agricultural Library
10301 Baltimore Avenue
Beltsville
MD
Beltsville, MD 20705-2351
Tel: 301-504-5755; 301-504-6856 (TTY)
Family Caregiver Alliance/National Center on Caregiving
785 Market St.
Suite 750
San Francisco
CA
San Francisco, CA 94103
Tel: 415-434-3388; 800-445-8106
Food and Drug Administration (FDA)
U.S. Department of Health and Human Services
5600 Fishers Lane, CDER-HFD-240
Rockville
MD
Rockville, MD 20857
Tel: 301-827-4573; 888-INFO-FDA (463-6332)
National Organization for Rare Disorders (NORD)
55 Kenosia Avenue
Danbury
CT
Danbury, CT 06810
Tel: 203-744-0100; Voice Mail: 800-999-NORD (6673)
World Health Organization
Avenue Appia 20
1211 Geneva 27
Switzerland
Switzerland,
Tel: (+ 41 22) 791 21 11
Publications

Transmissible Spongiform Encephalopathies (TSEs) information sheet compiled by the National Institute of Neurological Disorders and Stroke (NINDS).

Information booklet about Alzheimer's disease, vascular dementia, and other types of dementia compiled by the National Institute of Neurological Disorders and Stroke (NINDS).

Creutzfeldt-Jakob disease information for healthcare workers and morticians, compiled by the National Institute of Neurological Disorders and Stroke (NINDS). 

Creutzfeldt-Jakob Disease (CJD) fact sheet compiled by the National Institute of Neurological Disorders and Stroke (NINDS).

Patient Organizations