CAJ | 학술논문

目的：观察肝豆状核变性（wilson disease, WD）患者脑组织氢质子磁共振波谱（1H-MRS）的改变及患者在驱铜治疗前后脑组织1H-MRS的变化情况,为WD的评价预后提供参考。方法选择39例WD患者，分为非脑型组18例；脑型疗前组及短期治疗组均为21例，其中长期治疗组20例。分别利用1.5T超导磁共振诊断仪对患者进行1H-MRS单体素信号采集，在驱铜治疗后再次检测，比较治疗前后各指标的变化。20例均无血缘关系的健康志愿者作为对照组。结果患者的年龄、性别与对照组有可比性。39例WD患者左侧壳核、尾状核头部NAA/Cr较丘脑部位明显降低（P<0.01）。非脑型组左侧壳核部位、丘脑部位NAA/Cr及Cho/Cr的均值较对照组明显降低（P<0.01）。脑型疗前组左侧壳核、尾状核头及丘脑部位NAA/Cr、Cho/Cr及NAA/Cho的均值较对照组明显降低（P<0.01或P<0.05）。短期治疗组左侧壳核、尾状核头及丘脑部位NAA/Cr、Cho/Cr及NAA/Cho的均值较脑型疗前组无统计学差异（P>0.05）；长期治疗组左侧壳核、丘脑部位NAA/Cr及NAA/Cho的均值较脑型疗前组明显升高（P<0.01或P<0.05），尾状核头Cho/Cr的均值与脑型疗前组比较明显升高（P<0.05）。患者左侧壳核、尾状核头及丘脑NAA/Cr的均值与病程呈负相关（P<0.05）。结论不同类型的WD患者之间的1H-MRS表现也存在着差异，短期驱铜治疗对WD患者1H-MRS的影响不明显，而长期、正规的综合驱铜治疗能使WD患者的脑代谢得到显著改善。NAA/Cr比值可能作为观察疗效的可逆性非侵袭性指标。
목적：관찰간두상핵변성（wilson disease, WD）환자뇌조직경질자자공진파보（1H-MRS）적개변급환자재구동치료전후뇌조직1H-MRS적변화정황,위WD적평개예후제공삼고。방법선택39례WD환자，분위비뇌형조18례；뇌형료전조급단기치료조균위21례，기중장기치료조20례。분별이용1.5T초도자공진진단의대환자진행1H-MRS단체소신호채집，재구동치료후재차검측，비교치료전후각지표적변화。20례균무혈연관계적건강지원자작위대조조。결과환자적년령、성별여대조조유가비성。39례WD환자좌측각핵、미상핵두부NAA/Cr교구뇌부위명현강저（P<0.01）。비뇌형조좌측각핵부위、구뇌부위NAA/Cr급Cho/Cr적균치교대조조명현강저（P<0.01）。뇌형료전조좌측각핵、미상핵두급구뇌부위NAA/Cr、Cho/Cr급NAA/Cho적균치교대조조명현강저（P<0.01혹P<0.05）。단기치료조좌측각핵、미상핵두급구뇌부위NAA/Cr、Cho/Cr급NAA/Cho적균치교뇌형료전조무통계학차이（P>0.05）；장기치료조좌측각핵、구뇌부위NAA/Cr급NAA/Cho적균치교뇌형료전조명현승고（P<0.01혹P<0.05），미상핵두Cho/Cr적균치여뇌형료전조비교명현승고（P<0.05）。환자좌측각핵、미상핵두급구뇌NAA/Cr적균치여병정정부상관（P<0.05）。결론불동류형적WD환자지간적1H-MRS표현야존재착차이，단기구동치료대WD환자1H-MRS적영향불명현，이장기、정규적종합구동치료능사WD환자적뇌대사득도현저개선。NAA/Cr비치가능작위관찰료효적가역성비침습성지표。
Objective To examine the brain metabolic changes in WD patients receiving copper chelation by us?ing 1H-MRS. Method Thirty-nine patients with WD was randomly divided into four groups: non-brain type group (18 cases), brain type prior-treatment group and short-term treatment group (21 cases), long-term treatment group (20 cases) from short-term treatment group, and 20 healthy volunteers served as a control group. 1H-MRS and MRI were performed on patients on 1.5/MR/MRS system to detect these above-mentioned items before and after treatment. Result The mean of NAA/Cr was significantly lower in the left putamen and head of the caudate nucleus than in the left basal ganglion in the 39 patients with WD. The mean of NAA/Cr and Cho/Cr in the left putamen and basal ganglion was significantly lower in non-brain type group than in control group(P<0.01). The mean of NAA/Cr Cho/Cr and NAA/Cho in the left putamen,head of the caudate nucleus and basal ganglion were significantly lower in brain type group than in control group(P<0.01 or P<0.05). The mean of NAA/Cr in the left putamen was much lower in brain type group than in non-brain type group (P<0.01). The mean of NAA/Cr, Cho/Cr and NAA/Cho of short-term treatment group in the left putamen, head of the caudate nucleus and basal ganglion was not significantly different between brain type group and short-term treatment group(P>0.05). The mean of NAA/Cr and NAA/Cho in the left putamen and basal ganglion was much higher in long-term treatment group than in brain type group(P<0.01 or P<0.05). The mean of Cho/Cr in the left head of caudate nucleus were much higher after treatment compared with prior-treatment group(P<0.05). The mean of NAA/Cr in the left putamen, head of the left caudate nucleus and basal ganglion in all groups was negatively correlated with course of the disease. Conclusion There are significant differences in brain metabolism among different type of WD. The long-term but not short-term copper chelation significantly improves brain metabolism. NAA/Cr may be used as a non-invasive indicator to examine the efficacy of treatment.