肝豆状核变性脑血流量测定及与临床症状的相关性研究  

Decrease of CBF measured by SPECT correlates to neurological features in Wilson's disease

作　　者：李新辉[1] 潘爱艮[1] 金丽娟[2] 邓豪余[1] 

机构地区：[1]湖南医科大学附属湘雅医院核医学科,长沙410008 [2]湖南医科大学附属湘雅医院神经内科,长沙410008

出　　处：《中华核医学杂志》2000年第4期169-171,共3页Chinese Journal of Nuclear Medicine

摘　　要：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 changes of cerebral blood flow and its association with neurological features in the patients with Wilson's disease (WD) Methods In 13 WD patients and 12 age matched control subjects, we quantitatively determined CBF and rCBF with SPECT using the non blood withdraw method Five neurological signs including dysarthria, dystonia, bradykinesia/rigidity, tremor and gait ataxia were assessed semiquantitatively The severity of each sign was scored from 0 (absent) to 3 (severe) The individual scores collected from a subject were then summed up, resulting in a “total clinical score” (TCS) We studied the correlations between the CBF or rCBF and semiquantitative clinical scores Results CBF in case group was (40 94±2 33) mL·100 g -1 ·min -1 and was significantly lower than that in control group [(43 82±1 58) mL·100 g -1 ·min -1 ](P<0 01) Statistical comparison found more significant rCBF reduction in bilateral basal ganglia, thalamus, front lobe (P<0 01) and left temporal lobe (P<0 05) The reduction in CBF was correlated with the TCS (r=-0 620, P<0 05) The reduction in rCBF of bilateral basal ganglia correlated with the severity of dysarthria (r=-0 770, P<0 01 and -0 576, P<0 05) The reduction in rCBF of right parietal lobe and bilateral thalamus correlated with the severity of bradykinesia/rigidity (r=-0 757, P<0 01 and -0 635, P<0 05) Conclusions These results suggest Wilson's disease patients have CBF or rCBF reduction to different degrees, and lower rCBF was found in bilateral basal ganglia, thalamus, front lobe and left temporal lobe The neurological deficits may have relationship with reduction of cerebral blood flow in Wilson's disease SPECT is useful for evaluation of cerebral function in Wilson's disease patients

关 键 词：肝豆状核变性 SPECT 脑血流量 

分 类 号：R742.4[医药卫生—神经病学与精神病学] R816.1[医药卫生—临床医学]