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Abstract #0493

Abnormal Response to Visual Cortex Activation in Early Stage Huntington Disease Patients Using 31P-NMR Spectroscopy

Fanny Mochel1, Tra-My N'Guyen1, Dinesh K. Deelchand2, Daisy Rinaldi1, Romain Valabregue3, Claire Wary4, Pierre G. Carlier4, Alexandra Durr1, Pierre-Gilles Henry2

1UMR S975, Institut du Cerveau et de la Moelle, Hopital La Salpetriere, Paris, France; 2Center for Magnetic Resonance Research, University of Minnesota, Minneapolis, MN, United States; 3CENIR, Institut du Cerveau et de la Moelle, Hopital La Salpetriere, Paris, France; 4NMR Laboratory AIM-CEA, Institute of Myology, Piti-Salptrire University Hospital, Paris, France

We measured dynamic 31P NMR spectra in human visual cortex before, during, and after visual activation in controls and in patients with Huntingtons Disease. In controls, we observed an 11% increase in Pi/PCr ratio (p=0.012) and a 13% increase in Pi/ATP ratio (p=0.008) during brain activation. In contrast, in HD patients, both Pi/PCr and Pi/ATP ratios were unchanged during and after visual stimulation, most likely reflecting reduced mitochondrial activity. This may provide a new functional biomarker of brain energy deficit to assess therapeutic efficacy in HD in clinical trials.

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Keywords

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