Prion disease

Prion disease

Definition

A transmissible disease that is caused by a protein that is able to induce abnormal folding of normal cellular proteins, leading to characteristic spongiform brain changes, which are associated with neuronal loss without an inflammatory response. Such disorders have typically long incubation periods, but are then generally rapidly progressive and are uniformly fatal.

Also known as spongiform encephalopathy — per MONDO

Also identified as

Affected Organs

Organ Involvement Source
Brain Disease Has Associated Anatomic Site NCIT · CC BY 4.0
Brain Disease Has Primary Anatomic Site NCIT · CC BY 4.0
Central nervous system Disease Has Associated Anatomic Site NCIT · CC BY 4.0
Central nervous system Disease Has Primary Anatomic Site NCIT · CC BY 4.0
Nervous system Disease Has Associated Anatomic Site NCIT · CC BY 4.0
Nervous system Disease Has Primary Anatomic Site NCIT · CC BY 4.0