Four Types of Creutzfeldt-Jakob Disease Type Sporadic or classical (sCJD) Inherited or familial Details The most common form

of Creutzfeldt-Jakob Disease, sporadic CJD occurs for an as-yet unknown reason, and accounts for about 85% of the cases. The illness is shorter than in other forms, and predominantly affects adults aged 50+. Caused by a genetic mutation, inherited CJD accounts for fewer than 15% of all CreutzfeldtJakob cases. The age of onset can be younger than for sporadic CJD, and the course of illness is generally longer. Iatrogenic means the disease is acquired through a medical procedure such as contamination from brain surgery, or a corneal transplant, or grafts of dura mater, the membrane that covers the brain and spinal cord. These account for a tiny percentage of cases. Acquired from eating beef tainted with bovine spongiform encephalopathy (BSE), variant Creutzfeldt-Jakob Disease is more commonly known as mad cow disease. So far, most cases have occurred in the UK. The age of onset is typically younger and the duration of the disease longer than in sporadic CJD.

Iatrogenic

Variant (vCJD)

What causes Creutzfeldt-Jacob Disease? For a long time, researchers believed a "slow virus" was responsible for causing transmissible spongiform encephalopathies (TSEs), of which Creutzfeldt-Jakob Disease is one type. In animals, a TSE known as scrapie has affected sheep and goats since the 1700s. In fact, many experts believe British cattle developed mad cow disease after being fed the remains of scrapie-infected sheep. But the cause of TSEs themselves remained a mystery until the 1980s, when a researcher at the University of California San Francisco suggested the cause was actually an infectious protein, which he named a prion. Prions (short for proteinaceous infectious particles) are chains of amino acids (the building blocks of proteins) that occur naturally in both human and animal brains, and are normally harmless. However, when the prion folds incorrectly into a shape that can't be used by the body, the accumulating protein deposit begins to cause serious problems. Instead of performing a useful function, the errant prions start to create a slow systemic decline. As diseased cells die, the released prions infect more cells, until the brain eventually resembles Swiss cheese.