Medical Dictionary: Jakob-Creutzfeldt disease


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Jakob-Creutzfeldt disease: Another name for Creutzfeldt-Jakob Disease.
Jakob-Creutzfeldt disease (condition): Very rare prion brain disease
Jakob-Creutzfeldt disease (condition): Creutzfeldt-Jakob Disease (CJD) is a rare 1-in-a-million disease that occurs spontaneously, with 10-15% inheritance, and unrelated to animal diseases. It usually leads to dementia.

CJD is a rare brain disease in humans caused by abnormal brain proteins (prions). It is similar to the various transmissible spongiform encephalopathies that afflict animals including "mad cow disease". Unfortunately, prognosis for CJD is poor as there are no effective treatments against prions. CJD is not usually contagious, except rarely by direct exposure to the brain (such as brain surgery).

Because CJD is rare it is often misdiagnosed as other brain-related diseases. Common misdiagnoses include Alzheimer's disease, motor neurone disease, ALS, Picks disease, Multi infarct Disease, and metabolic encephalopathies.

Jakob-Creutzfeldt disease: Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder. It affects about one person in every one million people per year worldwide; in the United States there are about 200 cases per year. CJD usually appears in later life and runs a rapid course. Typically, onset of symptoms occurs about age 60, and 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. 1

More information on medical condition: Creutzfeldt-Jakob Disease:



Footnotes:
1. excerpt from Creutzfeldt-Jakob Disease Fact Sheet: NINDS

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