Change in PDE10 across early Huntington disease assessed by [18F]MNI-659 and PET imaging

Neurology. 2016 Feb 23;86(8):748-54. doi: 10.1212/WNL.0000000000002391. Epub 2016 Jan 22.

Abstract

Objective: To evaluate whether striatal [(18)F]MNI-659 PET imaging of phosphodiesterase 10A (PDE10) serves as a sensitive and reliable biomarker of striatal neurodegeneration in a longitudinal cohort of participants with early Huntington disease (HD).

Methods: A cohort of participants with HD, including both participants premanifest or manifest with motor signs, underwent clinical assessments, genetic determination, and 2 [(18)F]MNI-659 PET imaging sessions approximately 1 year apart. Eleven healthy control (HC) participants underwent clinical assessments and [(18)F]MNI-659 PET imaging once. Striatal binding potentials (BPnd) were estimated for brain regions of interest, specifically within the basal ganglia, and compared between baseline and follow-up imaging. Clinical measures of HD severity were assessed at each visit.

Results: Eight participants with HD (6 manifest; 2 premanifest) participated. Of those with manifest HD, all had relatively early stage disease (stage 1, n = 2; stage 2, n = 4) and a Unified Huntington's Disease Rating Scale total motor score <45. As expected, the HD cohort as a whole had a reduction in the basal ganglia BPnd to approximately 50% of that seen in HC. On follow-up scans, [(18)F]MNI-659 uptake declined in the putamen and caudate nucleus in all 8 participants. The mean annualized rates of decline in signal in the caudate, putamen, and globus pallidus and the putamen were 16.6%, 6.9%, and 5.8%, respectively. In HC, the annualized reduction in signal in striatal regions was less than 1%.

Conclusion: Longitudinal data in this small cohort of participants with early HD support [(18)F]MNI-659 PET imaging of PDE10 as a useful biomarker to track HD disease progression.

MeSH terms

  • Adult
  • Aged
  • Biomarkers / metabolism
  • Cohort Studies
  • Disease Progression
  • Female
  • Fluorine Radioisotopes / metabolism*
  • Humans
  • Huntington Disease / diagnostic imaging*
  • Huntington Disease / metabolism*
  • Longitudinal Studies
  • Male
  • Middle Aged
  • Phosphoric Diester Hydrolases / metabolism*
  • Phthalimides / metabolism*
  • Positron-Emission Tomography / trends*
  • Quinazolinones / metabolism*

Substances

  • 2-(2-(3-(4-(2-fluoroethoxy)phenyl)-7-methyl-4-oxo-3,4-dihydroquinazolin-2-yl)ethyl)-4-isopropoxyisoindoline-1,3-dione
  • Biomarkers
  • Fluorine Radioisotopes
  • Phthalimides
  • Quinazolinones
  • PDE10A protein, human
  • Phosphoric Diester Hydrolases