Creutzfeldt-Jakob disease (CJD) is a rare and fatal neurodegenerative disease of unknown cause. Patients are usually aged between 50 and 75 and typical clinical features include a rapidly progressive dementia associated, myoclonus and a characteristic electroencephalographic pattern. Neuropathological examination reveals cortical spongiform change, hence the term `spongiform encephalopathy'.
Creutzfeldt-Jakob disease captured public attention in the 1990s when individuals in the United Kingdom developed a form of the disease ? variant CJD (vCJD) ? after eating meat from cattle suspected of having mad cow disease. However, "classic" Creutzfeldt-Jakob disease has not been linked to beef contaminated with mad cow disease.
The CJD Foundation is also concerned about the complexity of issues surrounding this brain disease. Our philosophy is to be proactive by establishing collaborations and linkages, communicating with family members, researchers, physicians and political representatives and informing the broader community about CJD.
CJD belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs). Spongiform refers to the characteristic appearance of infected brains, which become filled with holes until they resemble sponges under a microscope. CJD is the most common of the known human TSEs. Other human TSEs include kuru, fatal familial insomnia (FFI), and Gerstmann-Straussler-Scheinker disease (GSS).
? The first person to develop symptoms of what turned out to be vCJD became ill in January 1994. Most people who have developed vCJD have lived in the UK. Some of the patients had been long-standing residents in Wales, Scotland or Northern Ireland. As of early November 2002, the CJD surveillance unit for the UK reported 129 cases of vCJD, including 93 confirmed and 24 probable cases. In addition, there are 12 cases where vCJD is strongly suspected, but the diagnosis has not yet been definitively confirmed by post mortem analysis.
The incidence of Creutzfeldt-Jakob disease (CJD) is monitored in the UK by the National CJD Surveillance Unit (NCJDSU) based at the Western General Hospital in Edinburgh, Scotland. The Unit brings together a team of clinical neurologists, neuropathologists and scientists specialising in the investigation of this disease. This document is intended to summarise the research in progress at the NCJDSU and also provide some background information about CJD and other human spongiform encephalopathies. We have also provided some links to other resources and contrary points of view available on the Web.
About 90 percent of patients die within 1 year. In the early stages of disease, patients 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.
Creutzfeldt-Jakob disease (CJD) is a rare, degenerative brain disorder. Symptoms usually start around age 60. Memory problems, behavior changes, vision problems and poor muscle coordination progress quickly to dementia, coma and death. Most patients die within a year.
Creutzfeldt-Jakob disease (CJD) is a very rare and incurable degenerative neurological disorder (brain disease) that is ultimately fatal. Among the types of transmissible spongiform encephalopathy found in humans, it is the most common
Creutzfeldt-Jakob disease (CJD) is a rare. Actually it is a degenerative, invariably fatal brain disorder. Typically, its symptoms occurs at about age 60. There is currently no single diagnostic test for CJD. While CJD can be transmitted to other people, the risk of this happening is extremely small.
In contrast to the traditional forms of CJD, vCJD has affected younger patients (average age 29 years, as opposed to 65 years), has a relatively longer duration of illness (median of 14 months as opposed to 4.5 months) and is strongly linked to exposure, probably through food, to a TSE of cattle called Bovine Spongiform Encephalopathy (BSE). There are three major categories of CJD are :-
1) sporadic CJD 2) Hereditary CJD, and 3) Acquired CJD
Causes
There are several types of Creutzfeldt-Jakob disease (CJD). The disorder itself is rare, occurring in about 1 out of 1 million people. Prion diseases are a unique form of infectious diseases. The disease is not produced by a bacterial or viral infection; instead, the illness is related to progressive accumulation of prion proteins.
It is clear that Creutzfeldt-Jakob disease is caused by an infectious agent, but it is not yet clear what type of agent that is. Originally assumed to be a virus, evidence is accumulating that, instead, CJD is caused by a protein called a prion transmitted from victim to victim. The other spongiform encephalopathies also are hypothesized to be due to prion infection.
Both classic and variant CJD belong to a broad group of human and animal diseases known as transmissible spongiform encephalopathies (TSEs). . It usually first appears in midlife, beginning between ages 20 and 70, with average age at onset of symptoms in the late 50s.
Symptoms
The most characteristic symptom of CJD is rapidly progressing dementia, or loss of mental function. Dementia is marked by :-
memory losses impaired abstraction and planning language and comprehension disturbances poor judgment disorientation decreased attention and increased restlessness personality changes and psychosis hallucinations
Treatment
The only way to confirm a diagnosis of CJD is by brain biopsy or autopsy. In a brain biopsy, a neurosurgeon removes a small piece of tissue from the patient's brain so that it can be examined by a neurologist.
There is no known cure for Creutzfeldt-Jakob disease. Custodial care may be required early in the course of the disease. Medications may be needed to control aggressive behaviors. These include sedatives, antipsychotics, and others. Currently, clinical studies include tests of antiviral drugs, such as amantadine, a compound useful in parkinsonism.
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