运动皮质的磁敏感加权成像定量评分有助于区分肌萎缩侧索硬化症的临床表型

正摘要目的分析磁敏感加权成像(SWI)上初级运动皮质的铁相关性低信号,区分肌萎缩侧索硬化症(ALS)及其亚型、类ALS以及健康对照者。方法纳入64例临床怀疑ALS且     

肌萎缩侧索硬化症磁共振扩散张量成像与临床相关性分析

目的:探讨肌萎缩侧索硬化症(ALS)磁共振扩散张量成像(DTI)结果与临床的相关性,从而为ALS的早期诊断提供可能的客观依据.方法:对16例ALS患者和15例健康志愿者行常规颅脑MRI扫描,均加扫轴面DTI序列.选取内囊后肢后3/4为兴趣区(ROI),测量各向异性分数(FA)和平均扩散系数(ADC),研究各临床指标(包括年龄、病程、ALS功能评分、病情进展速度)与FA值、ADC值的相关性.结果:在内囊后肢水平,ALS组FA值明显低于对照组 (t=3.452,P=0.002),ADC值明显高于对照组(t=2.670,P=0.012).内囊后肢FA值与ALS功能评分正相关(r=0.577,P=0.019),与年龄、病程、病情进展速度均无相关性(P＞0.05);内囊后肢ADC值和年龄、病程、ALS功能评分、病情进展速度均无相关性(P＞0.05).结论:ALS患者内囊后肢FA值和ADC值变化明显,前者更显著;FA值与ALSFRS评分呈正相关,提示FA值是评价上运动神经元损害更敏感指标,因此可为肌萎缩侧索硬化症诊断提供有价值信息,并在准确评估ALS患者治疗疗效、预后方面有着良好的应用前景.     

肌萎缩侧索硬化症的~1H-MR波谱研究

目的 探讨肌萎缩侧索硬化症(ALS)患者的1H-MRS表现及其与临床评分的关系.方法 对15例临床确诊及拟诊为ALS的患者(ALS组)和15名年龄相匹配的健康志愿者(对照组)进行1H-MRS扫描,采用单体素平均TE选择性单点分辨波谱序列(TE-Averaged PRESS).扫描图像经后处理,分别获得以中央前回为中心的运动皮层和内囊后肢处的以下成分波峰:N-乙酰天门冬氨酸(NAA)、谷氨酸(Glu)、谷氨酸复合物(Glx)以及肌酸(Cr),并测量NAA/Cr、Glu/Cr和Glx/Cr峰高相对值.采用t检验比较两组间各比值的差异,并分析上述各值与ALS患者临床评分的直线相关关系.结果 ALS组运动皮层和内囊后肢的NAA/Cr值为1.91±0.34、1.53±0.17,对照分别为2.23±0.33,1.66±0.07.两组间差异有统计学意义(t值分别为4.25、2.90,P值分别为0.00、0.01).AIS组运动皮层和内囊后肢Glu/Cr为0.34±0.05、0.29±0.06,Glx/Cr为0.40±0.04、0.33±0.06,均高于对照组(Glu/Cr分别为0.30±0.03、0.25±0.04,Glx/Cr分别为0.32±0.05,0.26±0.03),两组间差异有统计学意义(t值分别为2.56、2.40、7.34、5.30,P值分别为0.02,0.03、0.00、0.00).ALS患者Norris评分值为(57±8)分,ALS功能分级评分(ALSFRS)值为(29±4)分.直线相关分析发现ALS患者运动皮层Glx/Cr值与Norris评分呈负相关(r=-0.75,P=0.00),而与ALSFRS值无相关性.结论 ALS患者谷氨酸类代谢物含量升高.(1)H-MRS可反映ALS患者脑内代谢物的变化特征.     

ALS2调控初级纤毛发生的机制研究

目的 探究肌萎缩侧索硬化症蛋白2(ALS2)对初级纤毛发生的调控作用及其可能机制。方法 通过免疫荧光染色检测ALS2蛋白的亚细胞定位。采用siRNA干涉特异性敲低RPE-1细胞中ALS2 mRNA含量,检测ALS2蛋白含量减少对初级纤毛的影响。利用Western印迹检测初级纤毛组装与去组装过程中ALS2蛋白的动态变化。建立初级纤毛去组装实验体系,检测ALS2蛋白缺失对初级纤毛去组装过程的影响,Time-laps活细胞实时成像技术检测ALS2对初级纤毛去组装过程中纤毛末端剪切的影响。结果 免疫荧光显示,ALS2定位于初级纤毛发生的基体——中心体周围。在RPE-1细胞中敲低ALS2会显著提高初级纤毛异常发生的比例。蛋白水平动态检测结果显示,在初级纤毛去组装过程中ALS2蛋白表达水平逐渐增加;敲低ALS可显著抑制纤毛去组装过程。活细胞实时成像显示,ALS2蛋白缺失可抑制初级纤毛去组装过程的末端剪切。结论 ALS2作为中心体定位蛋白,通过促进初级纤毛末端剪切的正常进行,调控初级纤毛组装与去组装之间的动态平衡,防止纤毛异常生长引起的纤毛类疾病的发生。     

肌萎缩侧索硬化症患者大脑运动皮层的功能MRI研究

目的 研究肌萎缩侧索硬化症(ALS)患者顺次对指运动时脑部运动皮层的血氧水平依赖(BOLD)功能MRI(fMRI)变化.方法 对15例临床确诊及拟诊为ALS的患者(ALS组)和15名年龄、性别相匹配的健康志愿者(对照组)进行BOLD MR扫描.所有受试者均为右利手,无其他疾病,近期未服用过影响神经系统功能的药物,受试者进行频率为1～2次/s的顺次对指运动.使用3.0 T MR机获取梯度回波-回波平面成像(GRE-EPI)序列功能图像.所得数据采用统计参数图(SPM)2软件进行分析.激活脑区体积的比较采用t检验.结果 2组在完成相同运动任务时均有激活的脑区包括:双侧主感觉运动皮层(PSM)、双侧运动前区(PA)后部、双侧辅助运动区(SMA)、对侧外下运动前区(ILPA)、双侧顶叶区(PAR)和同侧小脑半球.ALS组激活体积较对照组激活体积明显增大的区域包括:双侧PSM及双侧PA后部[右手同侧激活:ALS组为(924.5±141.1)mm3,对照组为(829.9±98.4)mm3(P=0.05);右手对侧激活:ALS组为(9143.8±702.8)mm3,对照组为(8638.8±506.4)mm3(P＜0.05);左手同侧激活:ALS组为(1162.5±357.4)mm3,对照组为(902.5±184.2)mm3(P＜0.05);左手对侧激活:ALS组为(8255.2±870.2)mm3,对照组为(5934.6±616.4)mm3(P＜0.05)]、双侧SMA[右手双侧激活:ALS组为(6564.3±720.6)mm3,对照组为(4710.7±416.3)mm3(P＜0.05);左手双侧激活:ALS组为(6970.5±961.8)mm3,对照组为(3688.9±672.3)mm3(P＜0.05)]及同侧小脑半球[右手同侧激活:ALS组为(2720.0±1154.2)mm3,对照组为(254.3±84.4)mm3(P＜0.05);左手同侧激活:ALS组为(4794.4±1237.0)mm3,对照组为(1689.0±719.6)mm3(P＜0.05)].ALS组额外激活的区域包括:同侧ILPA、对侧小脑半球及双侧内囊后肢.结论 ALS组与对照组完成相同运动模式所激活的脑区相似,但ALS组激活的区域有所增加.ALS患者激活增大的脑区可能为功能重组,额外激活的脑区则可能为功能代偿.     

采用基于体素的形态测量学方法研究肌萎缩侧索硬化症患者脑灰质的改变

目的 利用基于体素的形态测量学方法(VBM)评价肌萎缩侧索硬化(ALS)患者的脑灰质体积变化及其与临床特征之间的相关性.方法 选取27例ALS患者和年龄、性别匹配的27名正常志愿者,采用VBM分析两组之间的全脑灰质体积和7个先验ROI:包括双侧中央前回,中央后回、额上、中、下回、额内侧回及岛叶灰质体积的改变;以P＜0.05[簇水平错误校正(FWE校正)]为差异有统计学意义.并使用偏相关分析,以年龄为控制因素,提取差异区域平均灰质密度与疾病严重程度评分、病程及疾病进展率进行相关性分析.结果 全脑灰质体积比较显示左侧中央前回、左侧中央后回、左侧额上回局部灰质体积减少,差异有统计学意义(统计体素数目分别为388、112、127,Z值分别为4.83、4.09、6.42,P值均＜0.05,FWE校正).ROI分析显示左侧中央前回、右侧中央前回、左侧中央后回、左侧额上回和左侧岛叶灰质体积减少,差异有统计学意义(统计体素数目分别为1104、34、114、91、107,Z值分别为5.87、3.71、4.26、6.29、3.51,P值均＜0.05,FWE校正).所有局部灰质体积减少程度与各临床指标间未发现有统计学差异的线性关系.结论 ALS可出现运动皮层及非运动皮层多个脑区灰质体积减少,进一步证实ALS系一种多系统变性病.相对于全脑比较,ROI分析能敏感地揭示更广泛的脑区改变.VBM所揭示的结构改变与临床指标之间缺乏相关性,考虑与病情本身的异质性和方法学的敏感性有关.     

肌萎缩侧索硬化症基于体素的形态测量扩散张量分析

目的 利用基于体素的形态测量学(VBM)及基于体素的扩散张量分析评价肌萎缩侧索硬化症(ALS)患者的脑灰质、白质体积及各向异性分数(FA)值的改变.方法 选取39例确诊或拟诊为ALS的患者(ALS组)及39名健康成年人(对照组)进行常规MR扫描及神经心理学评估,并采集3D快速扰相梯度回波(fast spoiled gradient echo,FSPGR)序列T_1WI和DTI数据.对3D T_1结构像进行配准、分割、平滑后,采用VBM分析,计算分割后的脑灰质、白质及脑脊液的体积.选取76名健康志愿者进行DTI,对原始图像进行后处理,制作FA模版,将ALS组和对照组受试者的FA图配准在所创建的FA模版上,测量FA值.统计方法采用协方差分析,因性别完全匹配,故对于VBM,年龄及全脑总体积作为协变量,而对于基于体素的扩散张量分析,仅年龄作为协变量,P＜0.01(未校正),相连像素＞20个的脑区为有差异的脑区.结果 全脑体积分析显示两组受试者的全脑灰质体积、全脑白质体积、全脑体积及脑灰质分数之间差异无统计学意义,但ALS患者的白质分数(0.29±0.02)小于健康对照组(0.30±0.02)(P=0.003).与健康对照组比较,ALS患者局部灰质体积减少脑区主要位于双侧额上回及中央前回,右侧额中回及颞中、下回,左侧枕上回、楔叶及左侧岛叶,ALS患者局部白质体积减少脑区主要位于胼胝体膝部,双侧额内侧回、旁中央小叶及岛叶,右侧额上回及额中回、左侧中央后回.ALS患者双侧扣带回及海马旁回FA值较对照组减低.结论 ALS并不是单纯的运动神经元病,是一种多系统受累的疾病,基于体素的扩散张量分析对于怀疑认知功能障碍的患者海马旁回及扣带回白质FA值变化的检出具有一定的潜在价值.     

MR液体饱和反转恢复序列在肌萎缩侧索硬化诊断及病情监测中的应用

目的:探讨液体饱和反转恢复序列(FLAIR)在肌萎缩侧索硬化(ALS)诊断及病情监测中的应用价值.材料和方法:对20例ALS患者行全脑的轴位FLAIR序列扫描,并对其中15例于2周及6个月后进行复查.由2名放射科医师分别对FLAIR图像进行评分,并与性别、年龄相当的正常志愿者进行比较.结果:ALS患者的皮层下白质及皮质脊髓束内可见不同亮度的高信号,而中央前回可见线状低信号.这两种征象的FLAIR评分均明显高于正常人;2周及6个月后的FLAIR复查评分之间无显著差别.ALS患者的FLAIR评分与ALS功能评分(ALSFRS)及病情进展速度无关,但与年龄及病程显著相关.结论:FLAIR序列可以为判断ALS患者上运动神经元是否受累提供客观依据,但病情监测作用有限.     

ALS患者运动皮层区的静息态功能磁共振成像分数低频振幅研究

目的 应用静息态功能磁共振成像(rs-fMRI)分数低频振幅(fALFF)方法对肌萎缩侧索硬化(ALS)患者运动皮层区基线脑活动变化进行研究. 方法 使用Siemens Trio Tim 3.0 T MRI对ALS组和正常对照组(各12例)分别进行静息态fMRI扫描.静息态fMRI数据处理助手(DPARSF)基于Matlab 2009a平台进行数据预处理并计算fALFF值,使用WFU PickAtlas软件提取运动皮层区[布鲁德曼(BA)4区和BA6区],应用静息态fMRI数据处理助手(REST)两样本t检验比较两组运动皮层区fALFF的改变.并分析运动皮质区存在显著差异脑区的fALFF值与临床参数如肌萎缩侧索硬化功能等级量表(ALSFRS-r)、病程、疾病预后比值的相关性.结果 相对正常对照组,ALS患者皮质运动区fALFF值降低区域主要位于双侧中央前回、双侧辅助运动区(P＜ 0.05,AlphaSim校正);相对于正常对照组,ALS患者皮质运动区fALFF值升高区域主要位于左侧中央前回、左侧辅助运动区、左侧旁中央小叶(P＜ 0.05,AlphaSim校正).其中左侧中央前回和患者的ALSFRS-r呈正相关(r=0.605,P=0.037);左侧旁中央小叶和患者的ALSFRS-r呈负相关(r=-0.633,P=0.027).差异脑区fALFF值和疾病病程、疾病预后比值之间并无显著的相关性(P值范围分别是0.154 ～0.968,0.303 ～0.995).结论 ALS患者存在运动皮质区神经元活动的异常,左侧中央前回fALFF值减低、左侧旁中央小叶fALFF值增高或可作为ALS患者疾病严重程度的潜在定量标志之一.表明fALFF是定量观测ALS运动皮质功能改变的一个有用指标.     

肌萎缩侧索硬化症的颈髓MR扩散张量成像研究

目的 了解常规MR检查阴性的肌萎缩侧索硬化症(ALS)患者颈髓DTI的各项参数值的变化情况.方法 选取临床诊断为ALS患者(ALS组)16例及16名年龄、性别匹配的健康志愿者(正常组)行颈髓常规MRI及横断面DTI.DTI图像经工作站(AW4.2)后处理,获得ADC图、部分各向异性(FA)图和相对各向异性(RA)图.在C3椎体水平层面分别在颈髓前索区、后索区及双侧皮质脊髓侧束区4个区域内选取面积约5 mm ×5 mm的ROI,测定其ADC值、FA值和RA值.使用独立样本t检验对两组间各项参数值进行比较,使用Pearson相关分析分别对ALS患者异常参数值与ALS患者病程、Norris评分及功能质量相关评分(ALSFRS)进行相关性分析.结果 ALS组左、右侧皮质脊髓侧束的FA、RA值较正常组减低.其中ALs组左侧皮质脊髓侧束的FA、RA值为0.762±0.089、0.762±0.107,正常组为0.863±0.098、0.890±0.105;ALS组右侧皮质脊髓侧束的FA、RA值为0.751±0.065、0.772±0.082,正常组为0.843±0.118、0.863±0.134,差异均具有统计学意义(t值分别为2.575、4.195、2.246、2.218,P值均<0.05).ALS组左、右侧皮质脊髓侧束的ADC值分别为(0.744±0.162)、(0.767±0.141)×10~(-3)mm~2/s,正常组为(0.640±0.149)、(0.643±0.168)×10~(-3)mm~2/s,两组间差异无统计学意义(t值分别为-1.319、-1.087,P值均>0.05).ALS组前索FA、RA及ADC值分别为0.637±0.113、0.622±0.138、(0.950±0.354)× 10~(-3)mm~2/s,正常组分别为0.670±0.117、0.656±0.136、(0.865±0.238)×10~(-3)mm~2/s,两组间差异无统计学意义(t值分别为0.854、-0.704、-1.155,P值均>0.05).ALS组后索FA、RA及ADC值分别为0.886±0.073、0.920±0.100、(0.613±0.137)×10~(-3)mm~2/s,正常组分别为0.906±0.078、0.914±0.135、(0.636±0.224)×10~(-3)mm~2/s,两组间差异也无统计学意义(t值分别为1.655、-0.148,-1.360,P值均>0.05).ALS患者左、右侧皮质脊髓侧束FA、RA值与ALS病程、临床Norris评分及ALSFRS之间均无相关性(P值均>0.05).结论 SE-EPI序列DTI用于ALS患者颈髓的检测,其扫描方法简单且比较敏感,可以反映常规MRI上未能显示的病理学改变.     

Value of axillary lymphoscintigraphy in patients with operated breast carcinoma

PURPOSE: To evaluate axillary dissection with axillary lymphoscintigraphy (ALS) in postoperative patients with breast carcinoma and its role in adjuvant radiotherapy (RT). Additionally, to define axillary dissection as complete and incomplete with ALS and to correlate it with the number of removed lymph nodes. MATERIAL AND METHODS: In the last two years, 121 women were studied four weeks after operation. Bilateral second interdigital subcutaneous injections were performed for ALS. Complete and incomplete axillary dissection were interpreted according to the number of surgically removed lymph nodes. ALS was interpreted as complete if no accumulation was shown. RESULTS: There was a good correlation between the number of surgically removed lymph nodes and complete and incomplete interpretation on ALS (p < 0.004). The number of removed lymph nodes was equal to or greater than 15 in 72% patients with complete dissection according to ALS. Of 48 patients with surgically incomplete axillary dissection, 18 (38%) showed no accumulation in the axillary region, while 25 of 68 (37%) patients with surgically complete dissection showed accumulation in the axillary region and were interpreted as incomplete according to ALS. Indication of RT was changed after ALS in patients with 1 to 3 involved lymph nodes. While RT was not considered in 12 of these patients before ALS, they were included in RT planning. On the other hand, 17 patients, considered for RT previously, were excluded from RT planning after ALS. CONCLUSION: Evaluation of axillary dissection with ALS especially in suspicious patients with 1 to 3 lymph node metastases might prevent unnecessary morbidity and can be useful in selecting patients who truly need axillary irradiation.     

Cortical T2 signal shortening in amyotrophic lateral sclerosis is not due to iron deposits

Signal shortening of the motor cortex in T2-weighted MR images is a frequent finding in patients with amyotrophic lateral sclerosis (ALS). The cause of signal shortening in ALS is unknown, although iron deposits have been suggested. To test this hypothesis, we acquired T2*-weighted gradient-echo (GRE) MR images in addition to T2-weighted turbo spin-echo in 69 patients with ALS. Signal shortening in T2-weighted images was found in 31 patients. In T2*-weighted GRE images, only three patients had signal shortening. One patient with additional bifrontal haemorrhage had frontal but no motor cortex signal shortening. Iron deposits do not cause cortical signal shortening in patients with ALS predominantly. Other factors are presumably more important in the generation of cortical T2 shortening in ALS.     

Detection of corticospinal tract compromise in amyotrophic lateral sclerosis with brain MR imaging: relevance of the T1-weighted spin-echo magnetization transfer contrast sequence

BACKGROUND AND PURPOSE: Hyperintensity in the posterior limb of the internal capsule at T2-weighted MR imaging, consistent with corticospinal tract (CST) degeneration, is described in amyotrophic lateral sclerosis (ALS). However, the lack of specific tests or biological markers hinders confirmation of the diagnosis, especially in the early stages. We investigated the CST in ALS with MR imaging. METHODS: We examined 25 patients (14 men, 11 women; mean age, 49.1 years; range, 29-68 years) and 21 age- and sex-matched control subjects without upper motor neuron signs. According to the revised El Escorial criteria, 22 patients had definite ALS; two, probable ALS; and one, suspected ALS. Fluid-attenuated inversion recovery (FLAIR; TR/TE/TI, 11,000/140/2600) and T1-weighted spin-echo (SE)/magnetization transfer contrast-enhanced (MTC; TR/TE, 510/12) imaging was performed at 1 T. Two experienced neuroradiologists blinded to the patients' history independently evaluated the CST. RESULTS: T1-weighted SE MTC imaging allowed visualization of the CST in both patients and control subjects. T1-weighted SE MTC images showed hypointensity along the CST and bilateral subcortical regions of the precentral gyri in all control subjects and hyperintensity in 80% of patients with ALS (P < .05). FLAIR images showed hyperintensity in these areas in both groups, with no significant difference. CONCLUSION: T1-weighted SE MTC imaging is sensitive and accurate in depicting CST lesions in ALS, whereas FLAIR imaging is not. T1-weighted SE MTC imaging is useful in diagnosing ALS by showing hyperintense areas along the CST, which separates patients from control subjects. This sequence should be included in the workup of patients with weakness and pyramidal signs.     

Cortical motor-sensory hypometabolism in amyotrophic lateral sclerosis: a PET study

We previously reported generalized cerebral glucose hypometabolism in amyotrophic lateral sclerosis (ALS) patients with upper motor neuron disease, using positron emission tomography (PET) with [18F]2-fluoro-2-deoxy-D-glucose. The present article presents a more detailed regional analysis of the hypometabolism, including measurements of the motor-sensory cortex at higher levels than used earlier. The analysis is based on 19 PET studies of 12 patients with ALS, four of whom had only lower motor neuron involvement, and 11 studies of age-matched control subjects. A brain size correction was included to eliminate differences in metabolism related to brain size but not to pathology. The eight ALS patients with both upper and lower motor neuron disease showed generalized hypometabolism, compared with the normal control subjects, that was greatest in the motor-sensory cortex and putamen. The motor-sensory deficit was strongly correlated with length of disease, and a marked sequential reduction was seen in repeat studies on four of the patients. There was also significant right-left asymmetry in these scans. No cerebral hypometabolism was seen in the four ALS patients without upper motor neuron involvement. Although the observed motor-sensory deficit in ALS is consistent with histopathological findings, the more generalized hypometabolism and the asymmetry suggest more widespread effects.     

A specific biomarker for amyotrophic lateral sclerosis: Quantitative susceptibility mapping

ObjectiveAccurate and timely diagnosis of amyotrophic lateral sclerosis (ALS) is a diagnostic challenge given the lack of specific diagnostic and imaging biomarkers as well as the significant clinic overlap with mimic syndromes. We hypothesize that MR quantitative susceptibility mapping (QSM) can help differentiate ALS from mimic diagnoses.MethodsIn a blinded retrospective study of MRIs with QSM from 2015 to 2018, we compared motor cortex susceptibility along the hand and face homunculi in ALS patients and patients with similar clinical presentations. Inclusion required a confirmed ALS or a mimic diagnosis. Comparative groups included age-matched patients with MRIs performed for non-motor neuron symptoms that were reported as normal or demonstrated leukoaraiosis. Quantitative susceptibility values were compared with ANOVA and Tukey-Kramer (post-hoc). ROC analysis and Youden's index were used to identify optimal cutoff values.ResultsFifty ALS, 35 mimic, and 70 non-motor neuron symptom patients (35 normal, 35 leukoaraiosis) were included. Hand and face homunculus mean susceptibility values were significantly higher in the ALS group compared to the mimic (p=0.001, p=0.004), leukoaraiosis (p<0.001, p=0.003), and normal (p<0.001, p<0.001) groups. ROC curve analysis comparing ALS to mimics resulted in an area under the curve of 0.71 and 0.67 for the hand and face homunculus measurements, respectively. In differentiating ALS from mimics, Youden's index showed 100% specificity and 36% sensitivity for hand homunculus measurements.ConclusionsQSM has diagnostic potential in the assessment of suspected ALS patients, demonstrating very high specificity in differentiating ALS from mimic diagnoses.     

Gabapentin therapy for amyotrophic lateral sclerosis: lack of improvement in neuronal integrity shown by MR spectroscopy

BACKGROUND AND PURPOSE: Proton MR spectroscopy has revealed impaired neuronal integrity in the motor cortex of patients with amyotrophic lateral sclerosis (ALS). We hypothesized that the N-acetylaspartate (NAA)-creatine (Cr) ratios in the motor cortex and adjacent brain could reflect the therapeutic effectiveness of gabapentin (GBP) treatment in ALS. METHODS: Eight patients with ALS underwent MR spectroscopy before and 26.5 days +/- 8.8 after starting GBP. In 10 patients with ALS who were not treated with GBP, paired spectra were obtained 21.4 days +/- 7.2 apart. Fourteen healthy subjects underwent a single MR spectroscopic examination. The NAA/Cr ratio was measured in the precentral gyrus, the postcentral gyrus, the superior parietal lobule, the supplementary motor area, and the premotor cortex. RESULTS: The NAA/Cr ratio was decreased in the precentral and postcentral gyri of patients with ALS compared with healthy controls. In those with ALS, the change in the NAA/Cr ratio was not different between treated patients and untreated patients in any of the regions studied. CONCLUSION: No improvement in neuronal integrity was detected in motor and nonmotor cerebral regions after GBP treatment. This result agrees with that of prior investigations showing the equivocal clinical effectiveness of GBP for ALS and supports the validity of the NAA/Cr ratio as a surrogate of therapeutic effectiveness.     

顺行性或逆行性改变:肌萎缩性侧索硬化的磁共振研究

目的 结合磁共振波谱成像(MRSI)、扩散张量成像技术(DTI)及MRI对肌萎缩性侧索硬化(ALS)进行对照研究,观察ALS皮质脊髓束(CST)走行区的代谢以及水分子运动的变化规律,探讨CST是否有顺、逆行性变化.资料与方法 采用MRI、MRSI及DTI技术对12例ALS患者和12名正常志愿者进行扫描,观察测量中央前回皮层下白质(SWM)、半卵圆中心(CS)、内囊后肢(PIC)、侧脑室体旁白质(PV)和大脑脚(CP)等5个CST不同解剖平面的氮-乙酰天门冬氨酸(NAA)与肌酸(Cr)比值,各向异性比(FA值)以及平均扩散率(MD值),分析比较ALS 患者和正常对照组以及不同侧别FA值、MD值以及NAA/Cr值的变化.结果 ALS的FA值较正常对照组明显降低(P＜0.001),在CST走行区的SWM、CS、PV 和 PIC平面,ALS组的FA值较正常组明显降低(P＜0.05或P＜0.01), MD值在ALS组有升高的趋势,但无统计学意义.在SWM和PV平面,ALS的NAA/Cr值较正常组降低明显(P＜0.05).FA值和MD值分别在PV和CP有左高右低的变化(P＜0.05).结论 MRSI与DTI相结合能够早期定量探测ALS患者CST的改变,左侧SWM可能为最先发生改变的区域,CST的改变可能为顺行性改变,在不同的平面改变不对称.     

Detection of pyramidal tract lesions in amyotrophic lateral sclerosis with magnetization-transfer measurements.

PURPOSETo determine the presence of small lesions in the pyramidal tract in patients with amyotrophic lateral sclerosis (ALS) by using magnetization-transfer (MT) measurements and MR imaging.METHODSMT ratios (MTRs) were measured in the posterior limb of the internal capsule in nine patients with ALS and in nine healthy volunteers.RESULTSThe mean value of MTRs (%) in patients with ALS was 15.76 +/- 1.48, while that of the control subjects was 19.83 +/- 1.54. The difference was statistically significant.CONCLUSIONSMT measurements are useful for detecting abnormalities associated with degeneration of the pyramidal tract in patients with ALS.