Abnormal response to cortical activation in early stages of Huntington disease

Fanny Mochel, Tra My N'Guyen, Dinesh Deelchand, Daisy Rinaldi, Romain Valabregue, Claire Wary, Pierre G. Carlier, Alexandra Durr, Pierre Gilles Henry

Research output: Contribution to journalArticlepeer-review

33 Scopus citations


Background:: We wished to identify noninvasive in vivo biomarkers of brain energy deficit in Huntington disease. Methods:: We studied 15 early affected patients (mean motor United Huntington Disease Rating Scale, 18 ± 9) and 15 age- and sex-matched controls. We coupled 31phosphorus nuclear magnetic resonance spectroscopy with activation of the occipital cortex in order to measure the relative concentrations of adenosine triphosphate, phosphocreatine, and inorganic phosphate before, during, and after visual stimulation. Results:: In controls, we observed an 11% increase in the inorganic phosphate/phosphocreatine ratio (P =024) and a 13% increase in the inorganic phosphate/adenosine triphosphate ratio (P =016) during brain activation, reflecting increased adenosine diphosphate concentrations. Subsequently, controls had a return to baseline levels during recovery (P =012 and 022, respectively). In contrast, both ratios were unchanged in patients during and after visual stimulation. Conclusions:: 31Phosphorus nuclear magnetic resonance spectroscopy could provide functional biomarkers of brain energy deficit to monitor therapeutic efficacy in Huntington disease.

Original languageEnglish (US)
Pages (from-to)907-910
Number of pages4
JournalMovement Disorders
Issue number7
StatePublished - Jun 2012


  • Energy metabolism
  • Huntington disease
  • NMR spectroscopy


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