| Symbol
| GSSS
|
| Location
| 20p13
|
| Name
|
Gerstmann-Sträussler-Scheinker disease |
| Other name(s)
|
encephalopathy, subacute, spongiform, Gerstmann-Strausssler type
Gerstmann-Sträussler disease
cerebellar ataxia, progressive dementia, and amyloid deposits in CNS
prion dementia |
| Corresponding gene
|
PRNP
|
| Other symbol(s)
| GSD, GSSD
|
| Main clinical features
|
cerebellar ataxia, gait disturbance, progressive dementia, with numerous neurofibrillary tangles in cerebral cortex, thalamus, substantia nigra, periaqueductal gray matter, progressive supranuclear palsy-like syndrome |
| Genetic determination
| autosomal dominant |
| Related entries
| including a mild form with ataxia without cognitive impairment
|
| Function/system disorder
| neurology |
|
| psychiatric disorder |
| Type
| disease
|