Sax D S, Powsner R, Kim A, Tilak S, Bhatia R, Cupples L A, Myers R H
Department of Neurology, Boston University School of Medicine, Massachusetts 02118, USA.
Mov Disord. 1996 Nov;11(6):671-7. doi: 10.1002/mds.870110612.
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.
我们比较了29例亨廷顿舞蹈症（HD）患者和9名对照者的前额叶、运动和感觉皮质以及基底神经节的灌注情况。我们发现，与对照者相比，病程短（<6年，n = 10）、病程中等（6 - 10年，n = 8）和病程长（>10年，n = 11）的HD患者灌注显著降低。在病程短的患者中，我们在磁共振成像显示萎缩迹象之前就观察到皮质灌注减少，这表明灌注水平所反映的神经元活动减少先于明显的结构变化。正如预期的那样，基底神经节的灌注减少明显。皮质灌注程度与功能能力临床评估以及病程相关。前额叶灌注与认知测量相关，运动皮质灌注与身体残疾及日常生活评分相关。我们未发现感觉皮质灌注与临床有显著相关性。单光子发射计算机断层扫描可能是临床试验和药物干预中评估疾病进展的一种敏感方法。
