DECREASED N-ACETYL-ASPARTATE CHOLINE RATIO AND INCREASED LACTATE IN THE FRONTAL-LOBE OF PATIENTS WITH HUNTINGTONS-DISEASE - A PROTON MAGNETIC-RESONANCE SPECTROSCOPY STUDY/
L. Harms et al., DECREASED N-ACETYL-ASPARTATE CHOLINE RATIO AND INCREASED LACTATE IN THE FRONTAL-LOBE OF PATIENTS WITH HUNTINGTONS-DISEASE - A PROTON MAGNETIC-RESONANCE SPECTROSCOPY STUDY/, Journal of Neurology, Neurosurgery and Psychiatry, 62(1), 1997, pp. 27-30
Background-Both the effect of the mutation and the pathogenesis of Hun
tington's disease are unknown and a lack of biological markers for the
natural history of the disease impedes the evaluation of novel therap
eutic approaches. Methods-Proton magnetic resonance spectroscopy was c
arried out on a frontal region of the cortex in 17 patients with clini
cally overt Huntington's disease and four asymptomatic gene carriers.
Results-Eight of 17 (47%) clinically affected patients with Huntington
's disease and each of the asymptomatic carriers had lactate peaks in
the frontal cortex which were not present in controls. The N-acetyl-as
partate/choline (NAA/Ch) ratio was significantly reduced in the sympto
matic patients indicating the presence of neuronal loss. The reduction
was related to the clinical severity of the disease and was absent in
the asymptomatic carriers. Conclusion-The finding of lactate peaks su
pports the hypothesis that disturbed cerebral energy metabolism contri
butes to the pathogenesis of Huntington's disease.