Huntington’s disease (HD) patients tend to underestimate the severity of chorea.Aims
The study aimed at assessing the possible impact of manual dexterity task completion on the awareness of chorea in HD. It was hypothesized that the patients’ tendency to underestimate chorea will decrease after the confrontation with a demanding motor task so that post-task ratings of chorea will be higher and more consistent with the objective measures than the pre-test ratings.Methods/techniques
Ten symptomatic HD patients (aged 58±11 years with the age of motor onset 52±10 years, the median Unified Huntington’s Disease Rating Scale- UHDRS total motor score 39.50 and median Total Functional Capacity-TFC score 8) completed the Nine Hole Peg Test (the 9HPT), pegboard task, used to assess manual dexterity. The participants estimated the degree of chorea interference with task performance on a 10-point scale (from 0 – no interference to 10-major interference) both prior and post task performance with each hand.Results/outcome
The patients’ median ratings of chorea increased from 0.50 (pre) to 1.50 (post) in the dominant hand motor coordination and from 2.50 (pre) to 3.00 (post) in the non-dominant hand. The ratings of involuntary movements were significantly influenced by the motor task performance with the non-dominant hand (p=0.04), but not with the dominant hand (p=0.12). Also the patients’ post-task chorea ratings concerning the non-dominant hand were highly correlated with the objective 9HPT performance (rho=0.93 for involuntary movements and rho=0.90 for both coordination and slowing) and chorea in the upper extremity as assessed in UHDRS motor (rho=0.65).Conclusions
The preliminary results show that the awareness of motor impairment in a patient may vary and that the patients are less unlikely to underestimate motor symptoms after being confronted with a demanding motor tasks.