Gerstmann-Straussler-Scheinker syndrome
ICD-10 Code
A81.82
Gerstmann-Straussler-Scheinker syndrome
Definition
A very rare and fatal disorder of spongiform encephalopathy usually caused by mutations of the prion protein (PRNP) gene. It is characterized by the accumulation of amyloid in the brain. Signs and symptoms include lack of motor coordination, unsteady gait, and difficulty walking. As the disease progresses, patients develop speech difficulties and dementia.
Also known as Gerstmann-Straussler-Scheinker disease, prion dementia, subacute spongiform encephalopathy, Gerstmann-Straussler type — per MONDO
Also identified as
- DOID 4249 per MONDO
- ICD10CM A81.82 per MONDO
- ICD9 046.71 per MONDO
- MESH C535800 per MONDO
- NCIT C84727 per MONDO
- OMIM 137440 per MONDO
- Orphanet 356 per MONDO
- SCTID 67155006 per MONDO
- UMLS C0017495 per MONDO
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 |