Huntington's disease-like 1 (DOID:0090103)
Alliance: disease page
Synonyms: autosomal dominant Huntington-like neurodegenerative disorder; early-onset prion disease with prominent psychiatric features; HDL1; HLN1; Huntington disease-like 1; Huntington-like neurodegenerative disorder 1
Alt IDs: OMIM:603218, ICD10CM:G10, ORDO:157941
Definition: A prion disease that is characterized by a phenocopy of Huntington disease (unwanted choreatic movements, behavioral and psychiatric disturbances and dementia) that has_material_basis_in autosomal dominant inheritance of 8 extra octapeptide repeats in the prion protein (PRNP) gene on chromosome 20p13.