Ds. Sax et al., EVIDENCE OF CORTICAL METABOLIC DYSFUNCTION IN EARLY HUNTINGTONS-DISEASE BY SINGLE-PHOTON-EMISSION COMPUTED-TOMOGRAPHY, Movement disorders, 11(6), 1996, pp. 671-677
We compared perfusion of prefrontal, motor, and sensory cortices and b
asal ganglia in 29 Huntington's disease (HD) patients and nine control
s. We found a significant reduction in perfusion in patients with HD o
f short (<6 years, n = 10), medium (6-10 years, n = 8), and long durat
ion (>10 years, n = 11) compared with controls. Among short-duration p
atients, we observed decreases in cortical perfusion before evidence o
f atrophy on magnetic resonance imaging, suggesting that decreases in
neuronal activity, as reflected by perfusion levels, precede gross str
uctural changes. As expected, decreased perfusion was marked in basal
ganglia. The extent of cortical perfusion correlated with clinical ass
essments of functional capabilities as well as with the duration of di
sease. Prefrontal perfusion correlated with cognitive measures, and mo
tor cortical perfusion correlated with physical disability and activit
ies of daily living scores. We found no significant clinical correlati
ons with sensory cortical perfusion. Single-photon-emission computed t
omography may be a sensitive method for assessing disease progression
in clinical trials and pharmacologic intervention.