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Supplemental: HD

Sensitivity to Change/ Ability to Detect Change (over time or in response to an intervention): These results are for the number of correct for negative emotions. In TRACK-HD, cross sectional differences from controls were found for both pre-manifest and early HD (Tabrizi et al, 2009); Longitudinal rate of change (annualized over 24 months) differed from change in controls for early HD but not pre-manifest HD (Tabrizi et al., 2011; Stout et al., in submission). PREDICT-HD detected both cross-sectional differences from controls and longitudinal changes over time (7 years) in pre-manifest HD. The TRACK-HD pre-manifest participants may be less likely to show cognitive effects than the PREDICT-HD pre-manifest participants because 1) they are further from estimated onset based on CAG repeat length and age (Langbehn et al., 2004) and

2) they are potentially less progressed in actuality because the TRACK-HD study excluded pre-manifest subjects based on UHDRS motor scores>= 5. Generally speaking, cognitive tests will be more effective metrics in studies of pre-manifest HD when the focus is on subjects that are close to onset.

 

Known Relationships to Other Variables: (e.g. gender, education, age, etc): Performance related to age but not to gender and education; change in performance not related to age, gender or education. Medication (i.e., neuroleptic) has shown to modulate emotion recognition abilities in early HD (Labuschagne et al., in submission).