Time will tell: Decision making in premanifest and manifest Huntington's disease

Objective: To investigate cognitive flexibility in premanifest and manifest Huntington's disease (HD).

Background: HD is an autosomal dominant neurodegenerative disease characterized by motor, cognitive, and behavioral abnormalities with typical motor symptoms. In this study, we wanted to assess decision making in premanifest (pre-HD) and manifest HD patients.

Methods: A total of 77 non-demented subjects including 29 pre-HD, 22 manifest HD patients, and 26 healthy controls (HC) were included. We stratified the pre-HD group based on their estimated years to disease onset into a far (FAR, n = 13) and a near (NEAR, n = 16) group. Furthermore, participants performed the Montreal cognitive assessment battery (MoCA), the trail making task part A and B (TMT A, TMT B), the Symbol digit modalities test (SDMT), and the beads task.

Results: In the beads task, HD patients gathered less information than all other groups (all p-values < .001). Furthermore, the NEAR group gathered less information than the FAR group (p < .001) and HC (p = .001). There was no difference between the HC and the FAR group (p = 1.0). In the TMT and the SDMT, HD patients were slower than all other groups (all p-values < .01) but there were no other significant differences.

Conclusions: Decision making with a higher degree of uncertainty may be an early neuropsychological sign to indicate the disease process prior to reaching criteria for motor diagnosis of HD.