肝豆状核变性^(99m)Tc-TRODAT-1的SPECT脑DAT显像研究

The study of ^(99m)Tc-TRODAT-1 SPECT in Wilson's disease patients.

目的研究肝豆状核变性(WD)患者99mTc-TRODAT-1 SPECT脑DAT显像特点及其与神经系统表现的关系。方法对18例有神经症状WD患者(脑型)、11例无神经症状WD患者(非脑型)和12例正常对照行99m Tc-TRODAT-1 SPECT脑DAT显像,利用计算机感兴趣技术(ROI)进行半定量分析(ST／CB比值作为半定量指标)。据WD神经症状改良评分表对18例脑型WD患者的神经症状进行评分。结果脑型WD患者ST／CB比值与正常对照组比较有显著性差异(P<0．01),非脑型WD患者ST／CB比值与正常对照组比较无显著性差异(P=0．13)。脑型WD患者神经症状改良评分与其ST／CB比值呈高度负相关,r=-0．78(P=0．001)。结论本研究结果提示WD患者DA能黑质纹状体通路突触前膜功能下降,其下降越明显,WD的神经症状就越严重。无神经症状的WD患者脑DAT功能无此改变。

Objective To study the traits of ^99mTc-TRODAT-1 SPECT in Wilson＇s disease （WD） patients and its relation with clinical manifestations. Methods There were 18 cases of brain group, 11 cases of non-brain group and 12 matched control subjects who underwent ^99mTc-TRODAT-1 SPECT. Specific striatal ^99mTc-TRODAT-1 binding ratios ST/CB （ with the cerebellum as the reference region） were calculated after the application of a region-of-interest （ROI） and assessed as semi-quantified parameters. Prominent neurological symptoms were registered and its severity was scored according to WD amending inquiry. Results The ST/CB of brain group were significantly decreased （ P 〈 0. 01 ） compared with control subjects. But the ST/CB of non-brain group was not different from those of control subjects. The ST/CB of brain group showed negatively correlated with the scores of WD amending inquiry. This correlation was proved to be linear and highly significant （ r = - 0. 78, P = 0. 001 ）. Conclusions This study suggests DAT was loss in presynaptic dopaminergic nerve terminals that were significantly correlated with the severity of the neurological symptoms in patients with neurological involvements. This maybe show that the more dominant the loss of DAT is, the more severe the neurological symptoms are. And there was no DAT change in the patients without neurological involvements.