Huntington's disease-like 1

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 prio

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.

Signs and symptoms

• Basal ganglia gliosis
• Incoordination
• Dysmetria
• Dysarthria
• Global brain atrophy
• Rigidity
• Chorea
• Mania
• Depression
• Dementia

Also known as: HDL1; HLN1; Huntington disease-like 1; Huntington-like neurodegenerative disorder 1; autosomal dominant Huntington-like neurodegenerative disorder