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Abnormal response to cortical activation in early stages of Huntington disease
Author(s) -
Mochel Fanny,
N'Guyen TraMy,
Deelchand Dinesh,
Rinaldi Daisy,
Valabregue Romain,
Wary Claire,
Carlier Pierre G.,
Durr Alexandra,
Henry PierreGilles
Publication year - 2012
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.25009
Subject(s) - phosphocreatine , huntington's disease , stimulation , medicine , adenosine triphosphate , endocrinology , cortex (anatomy) , creatine , magnetic resonance imaging , chemistry , neuroscience , psychology , disease , energy metabolism , radiology
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 31 phosphorus 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: 31 Phosphorus nuclear magnetic resonance spectroscopy could provide functional biomarkers of brain energy deficit to monitor therapeutic efficacy in Huntington disease. © 2012 Movement Disorder Society