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目的 探讨肝豆状核变性患者脑血流量的改变与神经系统症状的关系。方法 用非采血SPECT显像法测定 13例肝豆状核变性患者和 12例年龄相当的对照者全脑血流量 (CBF)和局部脑血流量 (rCBF) ;将患者主要临床症状分别按其严重程度评分 ,每一患者各项评分之和为其总评分(TCS)。分析TCS与CBF之间以及各临床症状与rCBF之间的相关性。结果 疾病组平均CBF为(40 94± 2 33)mL·10 0g- 1 ·min- 1 ,明显低于对照组平均CBF(43 82± 1 5 8)mL·10 0g- 1 ·min- 1 (P <0 0 1) ;rCBF降低以双侧基底节、额叶和丘脑最为明显 ,左侧颞叶rCBF也降低。疾病组TCS与CBF降低呈明显负相关 ,r=- 0 6 2 0 ,P <0 0 5。构音困难与双侧基底节rCBF降低呈显著负相关 (r值分别为 - 0 770和 - 0 5 76 ,P <0 0 1及P <0 0 5 ) ;运动徐缓或僵直与右侧顶叶及双侧丘脑rCBF降低呈负相关 (r值为 - 0 75 7,P <0 0 1和 - 0 6 35 ,P <0 0 5 )。结论 肝豆状核变性患者的神经系统症状可能与其大脑血流灌注下降有关。SPECT脑血流显像及定量测定对评价肝豆状核变性患者大脑功能具有一定的临床价值。
Objective To investigate the relationship between cerebral blood flow changes and neurological symptoms in patients with hepatolenticular degeneration. Methods Total cerebral blood flow (CBF) and regional cerebral blood flow (rCBF) were measured in 13 patients with hepatolenticular degeneration and 12 age-matched controls by non-spiked SPECT imaging. The main clinical symptoms of patients with severe hepatolenticular degeneration Degree score, the sum of each score of each patient for its total score (TCS). The correlation between TCS and CBF and between clinical symptoms and rCBF was analyzed. Results The average CBF of the disease group was (40 94 ± 2 33) mL · 10 0g -1 · min -1, which was significantly lower than that of the control group (CBF 4338 ± 15 8 mL · 10 0g -1 · min -1 P <0 01); rCBF reduced to the bilateral basal ganglia, frontal and thalamus is most obvious, left temporal lobe rCBF also decreased. There was a significant negative correlation between TCS and CBF in the disease group, r = - 0 6 2 0, P <0 05. There was a significant negative correlation between dysarthria and reduction of rCBF in bilateral basal ganglia (r values were -0.770 and -0.576 respectively, P <0.01 and P <0.05); bradykinesia or stiffness and right parietal lobe And bilateral hypothalamic rCBF decreased (r = -0.757, P <0.01 and -0.635, P <0.05). Conclusion The neurological symptoms in patients with hepatolenticular degeneration may be related to the decline of cerebral blood flow perfusion. SPECT cerebral blood flow imaging and quantitative determination of Wilson’s disease in patients with brain function evaluation of a certain clinical value.