Prion disease represents a group of conditions that affect the nervous system in humans and animals. In people, these conditions impair brain function, causing changes in memory, personality, and behavior; a decline in intellectual function (dementia); and abnormal movements, particularly difficulty with coordinating movements (ataxia). The signs and symptoms of prion disease typically begin in adulthood and worsen with time, leading to death within a few months to several years.
Inheritance Pattern
Autosomal dominant
Other Names
Inherited human transmissible spongiform encephalopathies; Prion protein diseases; Prion-associated disorders; Prion-induced disorders; Transmissible dementias; Transmissible spongiform encephalopathies; TSEs
Related Genetics Topics
- PRNP — MedlinePlus Genetics
Identifiers
- GTR: C0017495
- GTR: C0022336
- GTR: C0162534
- GTR: C0206042
- GTR: C1855588
- ICD-10-CM: A81.0
- ICD-10-CM: A81.00
- ICD-10-CM: A81.01
- ICD-10-CM: A81.09
- ICD-10-CM: A81.81
- ICD-10-CM: A81.82
- ICD-10-CM: A81.83
- MeSH: D017096
- OMIM: 123400
- OMIM: 137440
- OMIM: 245300
- OMIM: 600072
- SNOMED CT: 20484008
- SNOMED CT: 304603007
- SNOMED CT: 67155006
- SNOMED CT: 792004
- SNOMED CT: 840452004
Source: MedlinePlus Genetics, U.S. National Library of Medicine.
