Cognitive deficits in Huntington's disease are predicted by dopaminergic PET markers and brain volumes

Brain. 1997 Dec:120 ( Pt 12):2207-17. doi: 10.1093/brain/120.12.2207.


The main aim of this study was to investigate the relationship between dopaminergic markers and brain volumes for striatal and cortical structures, and cognitive performance in patients with Huntington's disease and control subjects. We used PET and MRI data as predictors of performance in tasks assessing executive function, visuospatial ability, episodic memory, verbal fluency, perceptual speed and reasoning. The dopamine neurotransmission parameters (D1 and D2 receptor density and dopamine transporter density) and the volumetric measurements for caudate and putamen accounted for substantial portions of the variance across the majority of cognitive tasks. In addition, frontal volume showed a strong relationship with all cognitive tasks. D1 binding and volume measurements for the temporal cortex and thalamic volume showed associations with a select number of cognitive tasks. The overall data pattern is consistent with the view that Huntington's disease may be characterized as a frontostriatal dementia, in which cognitive deficits may result from pathological changes at multiple sites in the frontostriatal circuitry.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Benzazepines / pharmacokinetics
  • Biomarkers
  • Brain / diagnostic imaging
  • Brain / metabolism*
  • Brain / pathology
  • Carbon Radioisotopes / pharmacokinetics
  • Carrier Proteins / metabolism*
  • Cocaine / analogs & derivatives
  • Cocaine / pharmacokinetics
  • Cognition Disorders / diagnostic imaging
  • Cognition Disorders / etiology*
  • Cognition Disorders / pathology
  • Dopamine / metabolism
  • Dopamine Plasma Membrane Transport Proteins
  • Female
  • Humans
  • Huntington Disease / diagnostic imaging
  • Huntington Disease / metabolism*
  • Huntington Disease / pathology
  • Huntington Disease / psychology*
  • Magnetic Resonance Imaging
  • Male
  • Membrane Glycoproteins*
  • Membrane Transport Proteins*
  • Memory
  • Middle Aged
  • Nerve Tissue Proteins*
  • Organ Specificity
  • Perception
  • Predictive Value of Tests
  • Raclopride
  • Radiography
  • Receptors, Dopamine D1 / metabolism*
  • Receptors, Dopamine D2 / metabolism*
  • Reference Values
  • Salicylamides / pharmacokinetics
  • Speech
  • Thinking
  • Tomography, Emission-Computed


  • Benzazepines
  • Biomarkers
  • Carbon Radioisotopes
  • Carrier Proteins
  • Dopamine Plasma Membrane Transport Proteins
  • Membrane Glycoproteins
  • Membrane Transport Proteins
  • Nerve Tissue Proteins
  • Receptors, Dopamine D1
  • Receptors, Dopamine D2
  • Salicylamides
  • Raclopride
  • 2beta-carbomethoxy-3beta-(4-iodophenyl)tropane
  • Cocaine
  • Dopamine