brain, spinal cord, and nerve disorders
Creutzfeldt Jakob Disease (CJD)
Introduction
Creutzfeldt-Jakob disease is a prion disease characterized by progressive deterioration of mental function, leading to dementia, involuntary jerking of muscles (myoclonus), and staggering when walking. A variant form is acquired by eating contaminated beef. Creutzfeldt-Jakob disease usually occurs spontaneously but may result from eating contaminated beef or from inheriting an abnormal gene. At first, most people are confused and have memory problems, then muscles begin to jerk involuntarily and coordination is lost. Most people die within 4 months to 2 years. The diagnosis can usually be made by electrocephalography, analysis of cerebrospinal fluid, and magnetic resonance imaging. There is no cure, but medications can relieve some of the symptoms. (See also Overview of Prion Diseases .) Creutzfeldt-Jakob disease (CJD) is a prion disease, which develops when a normal protein called cellular prion protein (PrPC) changes shape (misfolds) and becomes disease-causing prion. Prions slowly accumulate in the brain and usually cause tiny bubbles to form in brain cells, which gradually die. When enough brain cells malfunction or die, symptoms develop, followed by the person's death. CJD may Occur spontaneously (called sporadic CJD) Occur in families (called familial CJD) Be acquired Sporadic CJD, the most common form, occurs at a rate of 1 or 2 new cases per million people each year throughout the world. It accounts for about 85% of cases. It usually affects people over 40 years old, usually around age 65. For this form, no cause is known. to change into disease-causing prion. It accounts for 5 to 15% of cases. Familial CJD is often inherited and usually starts at an earlier age and can last longer than sporadic CJD. Familial CJD is almost always inherited as an Non–X-Linked (Autosomal) Inheritance . That means that the mutation is not on the sex (X or Y) chromosomes and that only one mutated gene for the disease, one from either parent, is required for the disease to develop. Acquired CJDcan result from Eating contaminated beef (called variant CJD) Having had certain medical procedures when contaminated materials or instruments were unknowingly used (called iatrogenic CJD) CJD has never been reported to be transmitted through casual or even intimate contact with people who have the disease. Acquired CJD accounts for fewer than 1% of cases. 