多系统萎缩患者143例临床和影像学特征分析

目的 探讨多系统萎缩(MSA)不同亚型的临床和影像学特征及其相关性.方法 对143例符合1999年Gilman诊断标准的MSA患者进行临床分型和诊断分级,根据Horimoto分期对108例影像学出现异常的患者脑桥十字征和壳核裂隙征进行分析,并探讨不同临床亚型及病程与影像学异常的相关性.结果 143例MSA患者男女比例为1.3:1,其中MSA小脑萎缩型(MSA-C)93例,MSA帕金森型(MSA-P)39例,两者同时出现的即为MSA-P+C型11例;很可能的MSA 90例,可能的MSA 53例.108例MSA患者影像学出现异常,其中MSA-C型患者36例(36/76,47%)出现脑桥十字征,10例(10/76,13%)出现壳核裂隙征;MSA-P型患者6例(6/24,25%)出现脑桥十字征,6例(6/24,25%)出现壳核裂隙征.MSA-C型中病程较短的患者脑桥十字征分期较早.结论 本组病例中MSA-C型患者明显多于MSA-P型,可能与种族遗传背景有关.脑桥十字征和壳核裂隙征为MSA患者的显著影像学特征,MSA临床分型与影像学特征具有一定的相关性,其中脑桥十字征在MSA-C型较为显著,壳核裂隙征在MSA-P型较为显著.     

不同亚型多系统萎缩的影像学诊断

目的 探讨高分辨MR T1WI结构像和Flair图像对多系统萎缩(MSA)不同亚型[小脑共济失调亚型(MSA-C)和帕金森亚型(MSA-P)]的鉴别诊断价值。方法 回顾性分析经临床确诊的24例MSA-C(MSA-C组)患者和12例MSAP(MSA-P组)患者,另选择20例年龄、性别相匹配的健康对照者(对照组)的MRI检查结果。高分辨MR T1WI结构像用于测量3组的双侧小脑中脚宽度,Flair图像用于判读壳核后部是否存在萎缩以及壳核的线样铁沉积。结果 (1)对照组、MSA-C组与MSA-P组的双侧小脑中脚宽度平均值组间比较均P<0.05;MSA-C组双侧小脑中脚宽度平均值最小(P<0.05),但MSA-P组与对照组比较差异无显著性(P=0.08)。以双侧小脑中脚宽度平均值1.02 cm鉴别MSA-C和MSA-P的曲线下面积(AUC)为0.93,敏感度为100%,特异度为87.5%,准确率为88.9%。(2)壳核后部萎缩：对照组、MSA-C组和MSA-P组出现壳核后部萎缩的比例分别为2.50%、10.41%和45.83%,组间比较均P<0.05。(3)壳核线样铁沉积：对...     

多系统萎缩的临床特点分析

目的探讨多系统萎缩(MSA)的临床表现特点。方法回顾性分析18例多系统萎缩患者的一般资料、临床表现、重要辅助检查及治疗转归等。结果 18例患者中,最常见的症状是帕金森综合征和小脑性共济失调,最常见的首发症状为自主神经功能障碍。MSA好发于成年,以缓慢起病为主,逐渐进展;直立倾斜实验、肛门括约肌肌电图有助于发现亚临床病变;磁共振(MRI)检查阳性率高;临床定位以双侧小脑半球、延髓腹外侧面、脑桥小脑受累最常见。目前尚无特效治疗方法,只能对症治疗。结论根据临床特点,结合直立倾斜试验、肌电图、影像学等检查能大大提高MSA的临床诊断率。     

磁共振脑径线测量对多系统萎缩的诊断价值

目的 研究磁共振脑径线测量对于多系统萎缩(MSA)的诊断价值.方法 11例MSA患者,可能MSA 2例,拟诊MSA 9例.其中以帕金森综合征为主要表现(MSA-P)5例,以小脑性共济失调为主要表现(MSA-C)6例.健康对照组6名,病例对照组9例(帕金森病1例、其他类型的帕金森综合征8例).选取反映脑干、小脑和基底节形态学的径线进行测量,计算全脑三维体积,比较各项参数的组间差异.结果 MSA组的脑桥横径(mm,下同)明显短于健康对照组和病例对照组(27.6±2.0、30.5±0.6、29.9±1.1),MSA患者的四脑室前后径(11.9±2.8)明显长于健康对照(9.0±2.1).MSA-C组的脑桥横径明显短于健康对照组和病例对照组(27.2±2.1、30.5±0.6、29.9±1.1).MSA-C患者的四脑室前后径和横径(12.8±2.6和9.0±2.1)明显长于健康对照(17.3±2.1和13.8±1.7).MSA-P患者的脑桥横径较健康对照组短(28.2±1.8、30.5±0.6).MSA-P患者的苍白球最长径(23.7±5.0)和红核直径(6.6±0.8)明显较MSA-C患者(29.7±2.4和8.2±0.4)短.MSA-C患者的第四脑室横径较MSA-P患者宽(17.3±2.1、12.6±2.7),小脑中脚宽度较MSA-P患者缩短(13.3±1.9、15.8±1.2).结论 磁共振脑体积径线测量对于MSA患者脑组织局部萎缩的程度提供了量化的手段.脑桥的横径缩短可以客观地反映MSA患者脑桥的萎缩,但不能用于区分MSA-P和MSA-C.MSA-C患者更易出现第四脑室的扩大和MCP的萎缩,MSA-P患者更易出现红核萎缩.     

磁共振表现为脑桥十字征的多系统萎缩患者临床与影像学特点分析

目的 探讨磁共振表现为脑桥十字征的多系统萎缩患者临床与影像学特点,为认识多系统萎缩临床特征及为多系统萎缩的临床诊断提供依据.方法 总结3例经临床诊断的多系统萎缩患者,分析其临床特点及磁共振特征表现.结果 3例患者均以小脑性共济失调伴有自主神经功能障碍为主要表现,脑桥十字征是其主要的磁共振特征表现.结论 以小脑性共济失调...     

多系统萎缩的影像学诊断价值

多系统萎缩(MSA)是由Graham和Oppen-heimer于1969年首次命名的一组原因不明的散发性进行性神经系统变性疾病,病变主要累及黑质、纹状体、下橄榄核、脑桥、小脑及脊髓等部位。早期学者根据患者临床特点的不同将其分为3型:以帕金森综合征为主要表现的纹状体黑质变性     

"十字征"和多系统萎缩1例报告

“十字征”(crosssign)即MR的T2加权像上脑桥的十字形异常高信号影,由Savoiardo等[1]于1990年首次报道,见于橄榄体脑桥小脑萎缩(OPCA)的患者。我们继丁美萍等[2]再次报道1例多系统萎缩(MSA),MR上呈现典型的“十字征”表现,以进一步提高广大临床医生对此征的认识。1资料患者男性,58岁,2005年3月4日入院。入院前5年出现行走不稳,左右摇晃,言语含糊不清,双手持物不稳,精细动作不能伴言语不清,吟诗样语言,时有饮水呛咳。近2年性功能减退。上述症状缓慢进行性加重。近1年来出现由卧位坐起及站立时晕倒十余次而不能自行行走。既往体健,家族…     

多系统萎缩的临床与影像学分析

目的 探讨多系统萎缩(MSA)的临床特点、影像学特征与临床表现的相关性，为临床诊断提供依据。方法 按Gilman诊断标准，回顾性分析26例MSA病人临床资料、一般辅助检查结果及脑CT、MRI资料。结果 拟诊MSA2l例，可能MSA5例，其中橄榄桥脑小脑萎缩(OPCA)14例，Slay-Drager综合征(SDS)8例，纹状体黑质变性(SND）4例。MRI显示OPCA的主要病变部位在小脑、桥脑、延髓；SDS仅部分有小脑病变，大部分正常；SND主要病变在壳核。而小脑、桥脑、延髓病变不明显。脑CT改变均不明显。结论 临床表现与MRI结合可提高MSA中OPCA、SND的诊断率。在SDS病人MRI改变不明显。一般辅助检查、脑CT对MSA诊断意义不大。     

多系统萎缩5例临床分析

多系统萎缩（MSA）是一组原因不明的中枢神经多发性、系统性变性疾病。以往曾被称为橄榄桥小脑萎缩（OPCA）、原发性直立性低血压（IOH）、Shy—Drager综合征（SDS）、纹状体黑质变性（SND）等。其实是同一疾病不同部位神经元萎缩的先后次序与结合方式不同。本文观察多系统萎缩5例，报道如下。     

多系统萎缩临床分型和诊断标准探讨

本文对多系统萎缩(MSA)临床分型和诊断标准提出初步方案,并对我院1973～89年神经科住院病人进行回顾性研究。结果符合MSA诊断标准的共13例,其中SND型和IOH型各3例,OPCA型7例。最后就MSA临床诊断标准有关问题作了讨论。     

多系统萎缩患者的临床表现、MRI、肛门括约肌肌电图分析

目的分析多系统萎缩患者(MSA)临床表现、头部MRI、肛门括约肌肌电图改变特点,探讨它们在MSA诊断的价值。方法按Gilman诊断标准,回顾性分析46例MSA患者的临床资料、头部MRI及肛门括约肌肌电图检查结果。结果符合很可能MSA39例,可能MSA7例,其中MSA-A型24例,主要临床表现为自主神经功能障碍;MSA-C型16例,主要表现为小脑性共济失调;MSA-P型6例,主要表现为锥体外系症状。MRI显示部分MSA-A患者出现大脑皮质萎缩,小脑改变较轻;MSA-C型主要表现为延髓、脑桥、小脑萎缩;MSA-P主要病变在壳核和苍白球,而小脑、脑桥、延髓病变早期可以不明显。部分患者出现脑桥十字征和壳核裂隙征。36例患者做肛门括约肌肌电图检查,全部出现神经源性损害。结论 MSA早期诊断难度大,结合临床表现、头部MRI检查及肛门括约肌肌电图检查,可提高MSA的诊断准确率。     

多系统萎缩患者的临床、影像及电生理分析

目的 探讨多系统萎缩(multiple system atrophy,MSA)的临床、影像以及电生理改变特点,为临床诊断提供依据.方法 对62例MSA患者的临床资料、神经影像学以及肌电图检查结果进行回顾性分析.结果 我们共收集拟诊MSA患者62例,其中MSA-A型29例(46.8 % ),主要临床特点表现为直立性低血压为主的自主神经系统症状;MSA-C型24例(38.7 % ),主要表现为小脑性共济失调;MSA-P型9例(14.5 % ),则以锥体外系症状为主.头颅MRI显示MSA-A型患者部分出现小脑病灶;MSA-C型患者主要病变在小脑、脑桥和延髓;MSA-P型患者病变主要在壳核.51例患者行肛门括约肌肌电图(external anal sphincter electromyography,EAS-EMG)检查,其中46例示典型神经源性损害;19例(30.6 % )曾被误诊为其他疾病.结论 MSA早期易漏诊或误诊,结合临床表现、神经影像学以及EAS-EMG检查,可提高MSA的诊断率.     

Subcortical atrophy and perfusion patterns in Parkinson disease and multiple system atrophy

BackgroundThe clinical differentiation between Parkinson disease (PD) and multiple system atrophy (MSA) is difficult.ObjectivesArterial spin labeling (ASL) is an advanced MRI technique that obviates the use of an exogenous contrast agent for the estimation of cerebral perfusion. We explored the value of ASL in combination with structural MRI for the differentiation between PD and MSA.MethodsNinety-four subjects (30 PD, 30 MSA and 34 healthy controls) performed a morphometric and ASL-MRI to measure volume and perfusion values within basal ganglia and cerebellum. A region-of-interest analysis was performed to test for structural atrophy and regional blood flow differences between groups.ResultsMSA patients showed higher subcortical atrophy than both PD patients and HC, while no differences were observed between the latter. MSA and PD showed lower volume-corrected perfusion values than HC in several cerebellar areas (Crus I, Crus II, right VIIb, right VIIIa, right VIIIb), right caudate and both thalami. MSA and PD patients displayed similar perfusion values in all aforementioned areas, but the right cerebellar area VIIIb (lower in MSA) and right caudate and both thalami (lower in PD). Similar results were obtained when comparing PD and MSA patients with the parkinsonian variant.ConclusionsA perfusion reduction was equally observed in both MSA and PD patients in cerebellar areas that are putatively linked to cognitive (i.e., executive) rather than motor functions. The observed hypo-perfusion could not be explained by atrophy, suggesting the involvement of the cerebellum in the pathophysiology of both MSA and PD.     

Multiple system atrophy masking multiple sclerosis

Multiple system atrophy (MSA) and multiple sclerosis (MS) are progressive neurological disorders with overlapping clinical signs and symptoms. However, due to the course of the disease and the age of onset both disorders are rarely differential diagnosis for each other. We here report the remarkable association of the two diseases in one patient. As MSA dominated the clinical presentation, diagnosis and therapy of MS were delayed. We discuss the clinical symptoms in our patient and highlight the features that allow to differentiate both diseases.     

Comparison of cerebral atrophy evaluated by MRI in Parkinson's disease and multiple system atrophy

Cerebral atrophy was evaluated in 31 patients with Parkinson's disease (PD) and 24 patients with clinically probable multiple system atrophy (MSA). Measurements of ventricular and brainstem areas, obtained from axial and sagittal MRI sections, were performed using a computer digitalizing system. Cortical and cerebellar atrophies were subjectively assessed. All measures were scored by one observer who was blind to the diagnosis (BD). Age, sex, levodopa dosage, baseline motor Parkinsonian disability (i.e. without levodopa treatment) and neuropsychological impairment were not significantly different between the two groups of patients. The total brainstem area (p < 0.001), the mesencephalon (p < 0.05) and the pons (p = 0.05) areas were significantly smaller in MSA as were the lobar (p < 0.05) and the vermian (p < 0.01) parts of the cerebellum. In the MSA group, significant correlations were observed between the brainstem area and items from the motor part of the Unified Parkinson's Disease Rating Scale (dysarthria, posture, arising from a chair), and between the cerebellar atrophy and the gait disturbance. A progressive discriminant analysis using radiologic variables correctly classified 79% of patients in their own diagnosis group with a significant difference (p = 0.01). From these results, it appears that MRI could help to differentiate MSA from PD, especially when MRI detects a severe infratentorial atrophy suggestive of MSA. However, brainstem and cerebellum atrophies are not sufficient criteria to differentiate MSA from PD because they lack both specificity and sensitivity.     

多系统萎缩与认知功能障碍

随着多系统萎缩患者生存时间的延长,除自主神经功能障碍、锥体外系症状和小脑共济失调外,还可出现认知功能障碍表现,严重者甚至达痴呆诊断标准。本文就多系统萎缩认知功能障碍国内外研究进展进行简要综述。     

Multiple system atrophy. Clinical and MR observations on 42 cases

Probable or possible multiple system atrophy (MSA) was diagnosed on strict clinical criteria in 42 patients: 20 with combined parkinsonism and cerebellar ataxia, 9 with striatonigral degeneration (SND) and 13 with olivopontocerebellar atrophy (OPCA). All patients were then studied with 0.5 and/or 1.5 Tesla magnetic resonance (MR) units. MR imaged putaminal abnormalities in all 9 patients with SND and posterior fossa obnormalities consistent with OPCA in all 13 patients with this diagnosis. Of the 20 patients with parkinsonism and cerebellar involvement, classified as probable MSA, 7 presented putaminal abnormalities only, 3 abnormalities consistent with OPCA only and 10 a combination of both. These findings show strong MRI support for the clinical diagnosis of MSA.

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Sommario In base a precisi criteri clinici 42 pazienti furono riconosciuti affetti da atrofia multisistemica (MSA) probabile o possibile. Venti pazienti presentavano parkinsonismo e atassia cerebellare; in 9 fu fatta diagnosi di degenerazione strio-nigrica (SND) e in altri 13 di atrofia olivopontocerebellare (OPCA). Tutti i pazienti furono sottoposti a risonanza magnetica 0.5 e/o 1.5 T. La RM mostrava alterazioni nei putamen nei 9 pazienti con SND e alterazioni in fossa posteriore come attese nell'OPCA nei 13 casi diagnosticati affetti da OPCA. In 10 dei 20 pazienti con parkinsonismo e atassia cerebellare le alterazioni nei putamen e in fossa posteriore erano associate. I nostri dati confermano che la RM è un supporto diagnostico fondamentale nella diagnosi di MSA.

     

Eye of the tiger-like MRI in parkinsonian variant of multiple system atrophy

Parkinsonian variant of multiple system atrophy (MSA-P) clinically presents as autonomic dysfunction with parkinsonian features. Parkinsonian features include bradykinesia, rigidity, tremor, postural instability and poor levo-dopa response. Neuropathologically, MSA-P is characterized by selective neuronal loss and gliosis mainly affecting the putamen and caudate nucleus, substantia nigra, olivopontocerebellar pathway and intermediolateral cell column of the spinal cord. Therefore, the target of magnetic resonance imaging (MRI) is focused on signal changes or volume reduction on putamen, including putaminal slit, gliosis by diffusion studies and reduction of putaminal volume. There have been no reports describing clinical manifestations of MSA-P with imaging abnormalities over globus pallidus. Here, we describe three patients with typical presentations of MSA-P with autonomic dysfunction and disturbances of axial motor function with minimal appendicular symptoms, including postural instability and gait difficulties. MRI showed symmetrical hyperintensity over the center of globus pallidus surrounded by a mild low-signal rims at T2-weighted image that is similar to that of eye of the tiger sign except for the marked hypointense rims. Dopamine transporter scans showed symmetric reduction of uptake over bilateral basal ganglia. This is the first report concerning these unusual imaging findings in MSA-P patients and we believe there is a subgroup of MSA-P with clinical presentation of axial impairment and symmetrically abnormal signal changes of globus pallidus in MRI.