EVIDENCE OF CORTICAL METABOLIC DYSFUNCTION IN EARLY HUNTINGTONS-DISEASE BY SINGLE-PHOTON-EMISSION COMPUTED-TOMOGRAPHY

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.