【作者】 邸平伟；

【导师】 罗蔚锋；

【作者基本信息】 苏州大学 ， 神经病学（专业学位）， 2013， 硕士

【摘要】 目的：探讨多系统萎缩（MSA）、进行性核上性麻痹（PSP）的临床表现特征。方法：对临床确诊的36例MSA、7例PSP，性别、年龄匹配的43例特发性PD作为对照组，进行详细的病史询问，体格检查。主要包括患者的发病年龄、首发症状、疾病进展过程出现的症状、病程、确定诊断时间，服用抗帕金森病药物的疗效，以及患者的文化程度、生活环境、家族史、脑血管病病史、特殊用药史、一氧化碳中毒史、毒物接触史等情况。结果：MSA-P组、MSA-C组、PSP组和PD组起病年龄分别为62.548.12岁、59.026.12岁、68.387.05岁，62.67.8岁；MSA-C发病年龄较PSP组显著低（P＜0.05），其余各组间发病年龄无显著差异。MSA-P组、MSA-C组和PSP组震颤发生率（分别为48.0%、36.4%、42.9%）均较PD组（88.4%）低（P＜0.05），MSA-C组强直症状发生率（90.1%）较PD组（86.0%）显著高（P＜0.05）。MSA-P组Romberg征阳性率（32.0%）较PD组（9.3%）显著高（P＜0.05），MSA-C组指鼻试验阳性率（63.6%）、Romberg征阳性率（63.6%）较PD组（23.3%，9.3%）显著高（P＜0.05）。MSA-C组体位性低血压发生率（36.4%）较PD组（4.7%）显著高（P＜0.05）。MSA-P痴呆、凝视麻痹、锥体束征/腱反射亢进发生率（分别为16.0%、12.0%、16.0%）较PD组（4.7%、0、0）显著高（P＜0.05），MSA-C组构音障碍、凝视麻痹、体位性低血压、锥体束征/腱反射亢进发生率（分别为63.6%、18.2%、36.4%、36.4%）较PD组（分别为23.3%、0、4.7%、0）显著高（P＜0.05）；PSP组跌倒、扫视/追踪减慢、痴呆、凝视麻痹、锥体束征/腱反射亢进发生率（分别为85.7%、57.1%、42.9%、71.4%、28.6%）较PD组（16.3%、11.6%、4.7%、0、0）发生率显著高（P＜0.05）。结论：MSA和PSP有各自独特的临床特征，相同症状、体征在各组发生率与PD组存在一定差异，有一定鉴别诊断价值。目的：探讨多系统萎缩（MSA）、进行性核上性麻痹（PSP）的MRI形态学上的特点，进行定量分析，为早期诊断和鉴别诊断提供客观依据。方法：经临床确诊的29例MSA、7例PSP为病例组，性别、年龄配对的36例帕金森病（PD）患者为病例对照组，性别、年龄配对的36例健康人为正常对照组，采用Philips Achieva1.5T超导型全身磁共振成像仪及标准Sense-head头线圈行常规MRI检查。主要观察指标：（1）T₂WI信号改变：①桥脑十字征（桥脑基底部可见十字样高信号）；②小脑中脚高信号；③壳核裂隙征（壳核外侧缘裂隙样高信号）。（2）T1WI图像上，①大脑萎缩；②小脑萎缩；③脑干（中脑、桥脑、延髓）萎缩。在图形工作站上应用软件View Forum4.1进行数据测量：在T1WI正中矢状位上测量桥脑面积、中脑面积并计算桥脑面积与中脑面积比值；在T1WI轴位上测量双侧小脑中脚宽度。结果：29例MSA中有5例（17.2%）出现十字征，4例（13.8%）出现裂隙征；其中MSA-P组小脑、延髓萎缩发生率（分别为33.3%、14.3%），MSA-C组的颞叶、小脑、中脑、桥脑、延髓萎缩发生率（分别为37.5%、62.5%、37.5%、37.5%、37.5%）较PD组（4.7%、7.0%、4.7%、4.7%、0）和对照组（4.7%、2.3%、0、0、0）显著高（P＜0.05），PSP组颞叶萎缩、中脑、桥脑萎缩发生率（分别为42.9%、85.7%、28.6%）较PD组（4.7%、4.7%、4.7%）和对照组（4.7%、0、0）显著高（P＜0.05）。MSA-C组的十字征、MCP高信号征发生率（50.0%、50.0%）较PD组（0、0）和对照组（0、0）显著高（P＜0.05）。7例PSP中有5例（71.4%）出现蜂鸟征，PSP组蜂鸟征发生率（71.4%）较PD组（0）和对照组（0）显著高（P＜0.05）。21例MSA-P的中脑、桥脑面积和小脑中脚宽度（130.951.9mm²、414.493.5mm²、12.02.5mm）和8例MSA-C的小脑中脚宽度（12.82.9mm）较PD组（149.325.3mm²、502.664.8mm²、15.81.4mm）和健康对照组（150.617.2mm²、503.844.9mm²、16.11.0mm）显著性减少（P＜0.05）。PSP组中脑、桥脑面积分别是97.434.2mm²、458.642.5mm²，其中，中脑面积与PD组和健康对照组显著性减小（P＜0.05）。结论：常规MRI形态学特点，定量分析有助于MSA、PSP及IPD的诊断及鉴别诊断，对MSA的分型具有一定的参考价值。目的：探讨多系统萎缩经颅超声（TCS）特点，为诊断及鉴别诊断提供客观依据。方法：经临床确诊的22例MSA作为病例组，性别、年龄配对的22例帕金森病（PD）患者为病例对照组。仪器采用GE vivid7，M3S探头，探头频率：2.0～2.5MHz，动态范围：45～55dB，深度：15～17mm。探头置于颞窗部位紧贴皮肤，平行于耳眶线（耳尖与眼角连线）。主要观察指标：（1）黑质回声强度进行半定量分级；（2）双侧黑质强同声总面积/中脑总面积比值。结果：22例行TCS检查MSA患者1例图像效果不佳（排除），剩余21例MSA患者中，7例（33.3％）表现黑质强回声，22例PD有2例TCS图像效果不佳（排除），剩余20例PD患者，13例（65.0％）表现为黑质强回声。MSA组黑质强回声发生率较PD组显著低（χ2=4.11，P=0.043）。MSA组7例黑质回声≥Ⅲ级，面积0.16～0.97cm²，PD组13例黑质回声≥Ⅲ级，面积为0.23～1.3cm²，对MSA组和PD组黑质强回声≥III级面积测量值和S/M比值进行比较，差异无统计学意义（P>0.05）。Wilcoxon检验表明，MSA组和PD组黑质强回声阳性的各分级分布有显著差异（Z=-3.12，P>0.05）。结论：MSA患者TCS黑质强回声发生率较PD组显著低，TCS检查有助于MSA和PD鉴别诊断。更多还原

【Abstract】 Objective: To explore the clinical features in multiple system atrophy and progressivesupranuclear palsy.Methods: We choose36patients with MSA,7patients with PSP diagnosed in thesecond affiliated hospital of Soochow University, Detailed history and physicalexamination of were collected, mainly includes the onset age, onset symptoms, symptomsduring progress of the disease, course of disease, diagnosis time, effect of anti-Parkinson’sdisease drug, and education, living environment, family history of cerebral vascular disease,special medication history, carbon monoxide poisoning history and poison contact history.Results: The onset age of MSA-P group, MSA-C group, PSP group and PD groupwere62.548.12years、59.026.12years、68.387.05years，62.67.8years respectively.The onset age of MSA-C group was significantly lower （P <0.05） compared with PDgroup. The incidence of tremor in MSA-P group, MSA-C group and PSP group （48.0%,36.4%,42.9%respectively） was significantly lower than that in PD group （88.4%）（P <0.05）, The incidence of rigidity in MSA-C group （90.1%） was higher than PD group（86.0%）（P<0.05）. The positive rate of Romberg sign in group MSA-P （32%） was higherthan PD group （9.3%）（P <0.05）.The positive rate of nasal test （63.6%） and Romberg sign（63.6%） in MSA-C group were higher than PD group （23.3%,9.3%respectively）（P <0.05）. The incidence of orthostatic hypotension （36.4%） in MSA-C group was higher thanPD group （4.7%）（P<0.05）. The incidence of cognitive impairment, palsy, pyramidal signs/tendon reflexes hyperfunction in MSA-P group （16%,12%,16%respectively） werehigher than group PD （4.7%,0,0respectively）（P<0.05）, The incidence of dysarthria, gazepalsy, orthostatic hypotension, extrapyramidal syndrome/tendon reflexes hyperfunction in MSA-C group （63.6%,18.2%,36.4%,36.4%respectively） were higher than PD group（23.3%,0,4.7%,0respectively）（P <0.05）; The incidence of falls, saccade/tracking down,cognitive impairment, gaze palsy, pyramidal signs/tendon reflexes hyperfunction in PSPgroup （85.7%,57.1%,42.9%,71.4%,28.6%respectively） were higher than PD group（16.3%,11.6%,4.7%,0,0respectively）（P <0.05）.Conclusion: MSA and PSP have their own unique clinical features, the incidence ofthe same symptoms/signs were different in each group, may be helpful in differentialdiagnosis with PD group Objective: To explore and quantitatively analyze morphology features in multiplesystem atrophy and progressive supranuclear palsy, provided objective evidence fordiagnosis and differential diagnosis of these diseases.Methods:29cases of MSA,7cases of PSP, gender, age matched36cases ofParkinson’s disease （PD） as case group, and gender, age matched36healthy objects ascontrols, using Philips Achieva1.5T superconducting whole-body magnetic resonanceimaging instrument and standard Sense-head head coil conventional MRI examination.Main measures:（1） T₂WI signal changes: the pontine cross sign （the basal portion of thepons visible cross high signal）;②middle cerebellar peduncle high signal;③putaminalslit syndrome （putamen outer margin fissured high signal）.（2） T1WI images, the brainatrophy;②cerebellar atrophy;③brainstem （medulla oblongata, midbrain, pons）atrophy. The datas were measured by of software View Forum4.1in the graphicworkstation: measuring area of pons and midbrain in the T1WI sagittal; measuring bilateralcerebellar peduncle width in the T1WI axis.Results:5cases（17.2%） of MSA show cross sign,4cases （13.8%） show slicksyndrome;The incidence of cerebellum atrophy, medulla atrophy in MSA-P group（33.3%,14.3%）, and temporal lobe atrophy, cerebellum atrophy, midbrain atrophy, pons atrophy,medulla atrophy in MSA-C group （37.5%,.62.5%,37.5%,37.5%,37.5%respectively） was significantly higher than PD group （4.7%,7%,4.7%,4.7%,0） and control group （4.7%,2.3%,0,0,0）（P <0.05）, The incidence of temporal lobe atrophy, midbrain atrophy,pontine atrophy in PSP group （42.9%,85.7%,28.6%, respectively） were significantlyhigher than PD group （4.7%,4.7%,4.7%） and control group （4.7%,0,0）（P <0.05）. Theincidence of crusade, high signal on MCP in MSA-C group （50%,50%） was significantlyhigher than PD group （0,0） and control group （0,0）（P <0.05）.5cases （71.4%） of PSPshow hummingbird sign, the incidence of hummingbird sign in PSP group（71.4%） wassignificantly higher than PD group （0） and control group （0）（P <0.05）. The midbrain area,pons area and middle cerebellar peduncle width in the21cases of MSA-P （130.9±51.9mm²,414.4±93.5mm²,12.0±2.5mm） and cerebellar peduncle width in MSA-C （12.8±2.9mm） decreased significantly than PD group （149.325.3mm²、502.664.8mm²、15.81.4mm） and control group （150.617.2mm²、503.844.9mm²、16.11.0mm）（P<0.05）. The midbrain area of PSP （97.434.2mm²） was significantly reduced than PDgroup and healthy control group （P <0.05）.Conclusion: Quantitative analysis of morphological features by conventional MRI,contributed to the diagnosis and differential diagnosis of MSA, PSP and IPD. and hadcertain value to the MSA subtype. Objective: To explore the TCS characteristics of multiple system atrophy, provideobjective evidence for diagnosis and differential diagnosis.Methods:21cases of MSA, gender, age-matched21cases of Parkinson’s disease （PD）patients as case group. The instrument adopts GE vivid7, M3S probe, probe frequency:2².5MHz, dynamic range:45⁵5dB,15¹7mm depth. The probe is arranged in thetemporal window close to the skin, the orbital line parallel to the ear （tip of ear and eyeline）. Main measures:（1） Semiquantitatively graded substantia nigra echogenicity;（2）Theratio: area of bilateral substantia nigra hypoechogenicity/total area of midbrain.Results: TCS was performed in22cases of MSA and22cases of PD,1patients withMSA and2patients with PD were excluded for the image ineffective,7cases （33.3%） of the remaining21MSA showed nigral hyperechogenicity,13cases （65%） of the remaining20PD cases showed the substantia nigra hyperechogenicity. Chi-square analysis showedthat the incidence of substantia nigra hyperechogenicity in MSA group was significantlyhigher than PD group （χ2=4.11, P=0.043）.7cases of MSA （33.3%） in substantia nigrahyperechogenicity≥grade Ⅲ, the area was0.16⁰.97cm².12cases （54.2%） of PD insubstantia nigra hyperechogenicity≥grade Ⅲ, area0.23¹.3cm², there were nosignificant difference of the area in substantia nigra hyperechogenicity≥Ⅲ and theratio of S/M between MSA and PD group （P<0.05）. The distribution of grade in positivesubstantia nigra echogenic was significant different between MSA and PD （Z=-3.12, P <0.05）.Conclusion: The incidence of substantia nigra hyperechogenicity in MSA wassignificantly lower than in PD.TCS was helpful for the differential diagnosis of MSA andPD.更多还原