Comparison of the Huntington's Disease like 2 and Huntington's Disease Clinical Phenotypes.
David G AndersonAline Ferreira-CorreiaFilipe Brogueira RodriguesN Ahmad AzizJonathan CarrEdward J WildRussell L MargolisAmanda KrausePublished in: Movement disorders clinical practice (2019)
The HDL2 phenotype is similar to HD and is initially characterized by dementia, chorea, and oculomotor abnormalities, progressing to a rigid and bradykinetic state, suggesting the UHDRS is useful to monitor disease progression in HDL2. Although HDL2 patients scored higher on some UHDRS domains, this did not differentiate between the two diseases; it may however be emerging evidence of HDL2 having a more severe clinical phenotype.