Deficits in temporal processing correlate with clinical progression in Huntington's disease

Acta Neurol Scand. 2017 Oct;136(4):322-329. doi: 10.1111/ane.12728. Epub 2017 Jan 3.


Objectives: Precise temporal performance is crucial for several complex tasks. Time estimation in the second-to-minutes range-known as interval timing-involves the interaction of the basal ganglia and the prefrontal cortex via dopaminergic-glutamatergic pathways. Patients with Huntington's disease (HD) present deficits in cognitive and motor functions that require fine control of temporal processing. The objective of the present work was to assess temporal cognition through a peak-interval time (PI) production task in patients with HD and its potential correlation with the Unified Huntington's Disease Rating Scale (UHDRS).

Materials and methods: Patients with molecular diagnosis of HD and controls matched by age, sex and educational level (n=18/group) were tested for interval timing in short- (3 seconds), medium- (6 seconds) and long (12 seconds)-duration stimuli.

Results: Significant differences were observed in the PI task, with worse performance in HD compared to controls. Patients underestimated real time (left-shifted Peak location) for 6- and 12-second intervals (P<.05) and presented decreased temporal precision for all the intervals evaluated (P<.01). Importantly, a significant correlation was found between time performance and the UHDRS (P<.01). Patients' responses also deviated from the scalar property.

Conclusions: Our results contribute to support that timing functions are impaired in HD in correlation with clinical deterioration. Recordings of cognitive performance related to timing could be a potential useful tool to measure the neurodegenerative progression of movement disorder-related pathologies.

Keywords: Huntington's disease; dopamine; medium spiny neurons; timing and time perception.

MeSH terms

  • Adult
  • Cognition / physiology*
  • Disease Progression
  • Female
  • Humans
  • Huntington Disease / diagnosis
  • Huntington Disease / physiopathology*
  • Male
  • Middle Aged
  • Time Perception / physiology*