Evidence of cortical metabolic dysfunction in early Huntington's disease by single-photon-emission computed tomography.
We compared perfusion of prefrontal, motor, and sensory cortices and basal ganglia in 29 Huntington's disease (HD) patients and nine controls. We found a significant reduction in perfusion in patients with HD of short (< 6 years, n = 10), medium (6-10 years, n = 8), and long duration (> 10 years, n = 11) compared with controls. Among short-duration patients, we observed decreases in cortical perfusion before evidence of atrophy on magnetic resonance imaging, suggesting that decreases in neuronal activity, as reflected by perfusion levels, precede gross structural changes. As expected, decreased perfusion was marked in basal ganglia. The extent of cortical perfusion correlated with clinical assessments of functional capabilities as well as with the duration of disease. Prefrontal perfusion correlated with cognitive measures, and motor cortical perfusion correlated with physical disability and activities of daily living scores. We found no significant clinical correlations with sensory cortical perfusion. Single-photon-emission computed tomography may be a sensitive method for assessing disease progression in clinical trials and pharmacologic intervention.
Department of Neurology, Boston University School of Medicine, Massachusetts 02118, USA.
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Powsner
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Kim
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Tilak
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Bhatia
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Myers
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001479:Basal Ganglia / Q000098:blood supply / Q000000981:diagnostic imaging; D001931:Brain Mapping; D002540:Cerebral Cortex / Q000098:blood supply / Q000000981:diagnostic imaging*; D004734:Energy Metabolism / Q000502:physiology*; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000000981:diagnostic imaging* / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009460:Neurologic Examination; D009483:Neuropsychological Tests; D012016:Reference Values; D012039:Regional Blood Flow / Q000502:physiology; D015899:Tomography, Emission-Computed, Single-Photon