Evidence of cortical metabolic dysfunction in early Huntington's disease by single‐photon‐emission computed tomography

Movement Disorders - Tập 11 Số 6 - Trang 671-677 - 1996
Daniel S. Sax1, Rachel A. Powsner2, Anthony Kim1, Shripad Tilak2, Rita Bhatia2, L. Adrienne Cupples3, Richard H. Myers1
1Department of Neurology, Boston University School of Medicine, Massachusetts, U.S.A.
2Department of Radiology, Boston University School of Medicine, Massachusetts, U.S.A.
3Boston University School of Public Health, Boston, Massachusetts, U.S.A.

Tóm tắt

Abstract

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. Perfrontal 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.

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