TY - JOUR
T1 - Riluzole protects Huntington disease patients from brain glucose hypometabolism and grey matter volume loss and increases production of neurotrophins
AU - Squitieri, Ferdinando
AU - Orobello, Sara
AU - Cannella, Milena
AU - Martino, Tiziana
AU - Romanelli, Pantaleo
AU - Giovacchini, Giampiero
AU - Frati, Luigi
AU - Mansi, Luigi
AU - Ciarmiello, Andrea
PY - 2009/7
Y1 - 2009/7
N2 - Purpose: Huntington disease (HD) mutation increases gain-of-toxic functions contributing to glutamate-mediated excitotoxicity. Riluzole interferes with glutamatergic neurotransmission, thereby reducing excitotoxicity, enhancing neurite formation in damaged motoneurons and increasing serum concentrations of BDNF, a brain cortex neurotrophin protecting striatal neurons from degeneration. Methods: We investigated metabolic and volumetric differences in distinct brain areas between 11 riluzole-treated and 12 placebo-treated patients by MRI and 18F-f uoro-2-deoxy-d-glucose (FDG) PET scanning, according to fully automated protocols. We also investigated the influence of riluzole on peripheral growth factor blood levels. Results: Placebo-treated patients showed significantly greater proportional volume loss of grey matter and decrease in metabolic FDG uptake than patients treated with riluzole in all cortical areas (p
AB - Purpose: Huntington disease (HD) mutation increases gain-of-toxic functions contributing to glutamate-mediated excitotoxicity. Riluzole interferes with glutamatergic neurotransmission, thereby reducing excitotoxicity, enhancing neurite formation in damaged motoneurons and increasing serum concentrations of BDNF, a brain cortex neurotrophin protecting striatal neurons from degeneration. Methods: We investigated metabolic and volumetric differences in distinct brain areas between 11 riluzole-treated and 12 placebo-treated patients by MRI and 18F-f uoro-2-deoxy-d-glucose (FDG) PET scanning, according to fully automated protocols. We also investigated the influence of riluzole on peripheral growth factor blood levels. Results: Placebo-treated patients showed significantly greater proportional volume loss of grey matter and decrease in metabolic FDG uptake than patients treated with riluzole in all cortical areas (p
KW - Brain-derived neurotrophic factor
KW - FDG-PET scan
KW - Neurotrophins
KW - Transforming growth factor
KW - Volumetric MRI
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U2 - 10.1007/s00259-009-1103-3
DO - 10.1007/s00259-009-1103-3
M3 - Article
C2 - 19280185
AN - SCOPUS:67349146480
VL - 36
SP - 1113
EP - 1120
JO - European Journal of Pediatrics
JF - European Journal of Pediatrics
SN - 0340-6199
IS - 7
ER -