Different prions affect different regions of the brain

Prions affect different regions of the brain. A sponge-like appearance results when nerve cells die. Symptoms depend on which region of the brain is affected.

Cerebral cortex When the cerebral cortex is affected, the symptoms include loss of memory and mental acuity, and sometimes also visual imparement (CJD).

Thalamus Damage to the thalamus may result in insomnia (FFI).

Cerebellum Damage to the cerebellum results in problems to coordinate body movements and difficulties to walk (kuru, GSS).

Brain stem In the mad cow disease (BSE), the brain stem is affected.

Mutations can result in different shapes of the prion protein that accumulates in different regions of the brain: In familial insomnia (FFI), mutated prions (violet squares) accumulate in the thalamus, with the result that the patients are unable to sleep. In Creutzfeldt-Jakob disease, the prion protein accumulates primarily in the cerebral cortex (red dots and area).

A precise diagnosis of a prion disease can only be made upon autopsy. The figures show thin sections of diseased brains. FFI, with typical proliferation of astrocytes, the support cells of the brain, is shown to the left (arrows). CJD, with the characteristic spongiform appearance with vacuoles (arrows) is shown to the right.

Different prions affect different regions of the brain

Nobel Prizes and laureates

Nobel Prizes 2023

Explore prizes and laureates