Gerstmann–Sträussler–Scheinker syndrome

GPTKB entity

Predicate	Object
gptkbp:instanceOf	gptkb:neurodegenerative_disease gptkb:prion_disease
gptkbp:affects	central nervous system
gptkbp:alsoKnownAs	gptkb:GSS_syndrome
gptkbp:causedBy	mutation in PRNP gene
gptkbp:diagnosedBy	gptkb:neurological_examination genetic testing
gptkbp:firstDescribed	1936
gptkbp:frequency	very rare
gptkbp:ICD-10_code	A81.8
gptkbp:inheritance	autosomal dominant
gptkbp:MeSH_ID	D020868
gptkbp:mortalityRate	100%
gptkbp:namedAfter	gptkb:Ilya_Scheinker gptkb:Josef_Gerstmann gptkb:Ernst_Sträussler
gptkbp:OMIM	137440
gptkbp:onset	middle age
gptkbp:pathology	amyloid plaques spongiform changes
gptkbp:prognosis	fatal
gptkbp:progression	slowly progressive
gptkbp:relatedTo	gptkb:Creutzfeldt–Jakob_disease gptkb:fatal_familial_insomnia
gptkbp:symptom	gptkb:dementia spasticity ataxia parkinsonism dysarthria
gptkbp:treatment	supportive care
gptkbp:bfsParent	gptkb:Eugen_Sträussler gptkb:Creutzfeldt–Jakob_disease
gptkbp:bfsLayer	6
https://www.w3.org/2000/01/rdf-schema#label	Gerstmann–Sträussler–Scheinker syndrome