中华神经科杂志
中華神經科雜誌
중화신경과잡지
Chinese Journal of Neurology
2008年
10期
674-677
,共4页
周香雪%李洵桦%梁秀龄%蒲小勇%陈松林%刘冰%梁茵颖%李立%谢春格
週香雪%李洵樺%樑秀齡%蒲小勇%陳鬆林%劉冰%樑茵穎%李立%謝春格
주향설%리순화%량수령%포소용%진송림%류빙%량인영%리립%사춘격
肝豆状核变性%青霉胺%铜%磷酸丙酮酸水合酶
肝豆狀覈變性%青黴胺%銅%燐痠丙酮痠水閤酶
간두상핵변성%청매알%동%린산병동산수합매
Hepatolenticular%Penicillamine%Copper%Phosphopyruvate hydratase
目的 探讨肝豆状核变性(WD)患者在使用青霉胺治疗过程中出现神经症状加重的原因和机制.方法 选取初诊WD患者40例(其中脑型32例,肝型8例),健康对照20名.对WD患者进行神经症状评分,测定脑脊液铜、血清铜、24 h尿铜、脑脊液和血清神经元特异性烯醇化酶(NSE)、血脑屏障指数(AR值).使用青霉胺治疗3个月后,再次进行神经症状评分,用相同方法测定以上指标.所有数据进行统计学分析.结果 15例(46.9%)脑型WD患者使用青霉胺治疗后出现神经症状加重.症状加重的WI)患者治疗后脑脊液铜[(0.083±0.030)mg/L]、脑脊液及血清中NSE[(3.00±1.17)、(6.79±2.20)μg/L]高于治疗前[分别为(0.072±0.027)mg/L,(1.32±0.85)、(3.45±2.09)μg/L,t=3.12、2.53、2.71,P<0.05],血清铜低于治疗前[治疗前后分别为(0.37±0.09)和(0.25±0.08)mg/L,t=3.17,P<0.05].症状加重的患者尿铜排出量显著低于症状改善者.治疗前后AR值[分别为(9.53±3.18)和(12.24±3.17)]差异无统计学意义(t=1.45,P>0.05).结论 初诊WD患者使用青霉胺后是否出现症状加重与其神经系统病变程度和使用青霉胺治疗的剂量有关.青霉胺治疗早期神经症状加重的原因可能是脑铜的增加、神经元新近的损伤和坏死以及铜从中枢神经系统排出困难.
目的 探討肝豆狀覈變性(WD)患者在使用青黴胺治療過程中齣現神經癥狀加重的原因和機製.方法 選取初診WD患者40例(其中腦型32例,肝型8例),健康對照20名.對WD患者進行神經癥狀評分,測定腦脊液銅、血清銅、24 h尿銅、腦脊液和血清神經元特異性烯醇化酶(NSE)、血腦屏障指數(AR值).使用青黴胺治療3箇月後,再次進行神經癥狀評分,用相同方法測定以上指標.所有數據進行統計學分析.結果 15例(46.9%)腦型WD患者使用青黴胺治療後齣現神經癥狀加重.癥狀加重的WI)患者治療後腦脊液銅[(0.083±0.030)mg/L]、腦脊液及血清中NSE[(3.00±1.17)、(6.79±2.20)μg/L]高于治療前[分彆為(0.072±0.027)mg/L,(1.32±0.85)、(3.45±2.09)μg/L,t=3.12、2.53、2.71,P<0.05],血清銅低于治療前[治療前後分彆為(0.37±0.09)和(0.25±0.08)mg/L,t=3.17,P<0.05].癥狀加重的患者尿銅排齣量顯著低于癥狀改善者.治療前後AR值[分彆為(9.53±3.18)和(12.24±3.17)]差異無統計學意義(t=1.45,P>0.05).結論 初診WD患者使用青黴胺後是否齣現癥狀加重與其神經繫統病變程度和使用青黴胺治療的劑量有關.青黴胺治療早期神經癥狀加重的原因可能是腦銅的增加、神經元新近的損傷和壞死以及銅從中樞神經繫統排齣睏難.
목적 탐토간두상핵변성(WD)환자재사용청매알치료과정중출현신경증상가중적원인화궤제.방법 선취초진WD환자40례(기중뇌형32례,간형8례),건강대조20명.대WD환자진행신경증상평분,측정뇌척액동、혈청동、24 h뇨동、뇌척액화혈청신경원특이성희순화매(NSE)、혈뇌병장지수(AR치).사용청매알치료3개월후,재차진행신경증상평분,용상동방법측정이상지표.소유수거진행통계학분석.결과 15례(46.9%)뇌형WD환자사용청매알치료후출현신경증상가중.증상가중적WI)환자치료후뇌척액동[(0.083±0.030)mg/L]、뇌척액급혈청중NSE[(3.00±1.17)、(6.79±2.20)μg/L]고우치료전[분별위(0.072±0.027)mg/L,(1.32±0.85)、(3.45±2.09)μg/L,t=3.12、2.53、2.71,P<0.05],혈청동저우치료전[치료전후분별위(0.37±0.09)화(0.25±0.08)mg/L,t=3.17,P<0.05].증상가중적환자뇨동배출량현저저우증상개선자.치료전후AR치[분별위(9.53±3.18)화(12.24±3.17)]차이무통계학의의(t=1.45,P>0.05).결론 초진WD환자사용청매알후시부출현증상가중여기신경계통병변정도화사용청매알치료적제량유관.청매알치료조기신경증상가중적원인가능시뇌동적증가、신경원신근적손상화배사이급동종중추신경계통배출곤난.
Objective To explore the mechanism of the secondary deterioration of neurological symptoms in Wilson' s disease (WD) at early stage of treatment using D-penicillamine. Methods Forty non-treated WD patients, 32 of encephalic and 8 hepatic type respectively, were enrolled in the study. Their neural symptoms were scored using modified Young grade. Cerebrospinal fluid (CSF) copper, serum copper, urinary copper, neuron specific enolase (NSE) in CSF and the albumin ratio CSF/serum (AR) were measured at the same time. After 3 months of treatment with D-penicillamine, neural symptoms of patients were scored again. All dates were analyzed. Results After 3 months of treatment with D-penicillamine, 15 patients (46. 9%) developed a secondary deterioration in neurological symptoms. The concentration of copper and the NSE in CSF of patients whose neural symptom was increasingly deteriorated. The serum copper declined after treatment((0. 37± 0. 09) vs (0. 25 ± 0. 08) mg/L, t = 3. 17, P < 0. 05). The 24 hours urinary copper of patients whose symptoms had deteriorated was much lower than that of patients who had not. No significant change was found in AR ratio before and after the treatment (9. 53 ± 3.18vs12.24±3.17) in the worsened group (t=1.45, P>0. 05). Conclusions The degree of the injury in the neural system and the dose of penicillamine may affect the deterioration of the neural symptom.
