Regional brain and cerebrospinal fluid quinolinic acid concentrations in Huntington's disease.

Many of the characteristics neuroanatomical and neurochemical features of Huntington's disease (HD) are produced in experimental animals by an intrastriatal injection of the endogenous N-methyl-D-aspartate receptor agonist quinolinic acid (QUIN). Conceivably, a chronic over-production of QUIN in brain could be involved in the pathogenesis of HD. To investigate this hypothesis, concentrations of QUIN were measured both in cerebrospinal fluid (CSF) and postmortem tissue from patients with HD and neurologically normal age-matched controls. CSF QUIN concentrations were slightly lower in patients with HD, however the changes were not significant. Mean concentrations of QUIN tended to be lower in HD putamen, dentate nucleus and several cortical regions, although significant reductions were found only in Brodmann areas 17, 20 and 28. The mechanisms responsible for these small reductions in brain QUIN concentrations remain to be determined. These results do not support the hypothesis that a chronic increase of QUIN production is responsible for neurodengeneration in HD.