The Discovery of Prions and Creutzfeldt-Jakob Disease

In 1920, at a psychiatric hospital in Hamburg, Germany, neuropathologist Alfons Maria Jakob began recording patients who fit no known illness. Over the next three years he documented six cases: rapid, relentless dementia, muscles stiffening into spasticity, limbs jerking without warning. Under the microscope their brain tissue looked sponge-like, riddled with tiny holes where healthy tissue should have been. A year after Jakob's first report, fellow German neuropathologist Hans Gerhard Creutzfeldt published a similar case of his own. Jakob, though his report came first, credited Creutzfeldt with priority anyway. In 1922 pathologist Walther Spielmeyer joined the two names together, calling it Creutzfeldt-Jakob disease. For decades afterward doctors assumed some kind of slow-acting virus was responsible, since nothing else explained a brain disintegrating from within with no sign of infection. No one yet had reason to suspect a single protein. That answer was still more than sixty years away.