橄榄脑桥小脑萎缩与“十字征”

目的:探讨橄榄脑桥小脑萎缩(OPCA)的临床和影像学特点。方法:对1例橄榄脑桥小脑萎缩患者的临床资料进行回顾性分析并文献复习。结果:本病以小脑共济失调为首发表现,部分出现植物神经系统受损、锥体束或锥体外系症状。影像学除脑干变细、小脑体积变小外,可出现特征性的十字征表现。结论:因该病和某些小脑变性疾病表现相重叠,且无有意义的生化标记物,故其影像学十字征的发现对此病的诊断和鉴别诊断意义重大。     

多系统萎缩的临床分型和影像学改变特点分析

目的探讨多系统萎缩（multiple system atrophy，MSA）的临床表现类型与神经影像学改变新特征（脑桥“十字征”和“壳核裂隙征”）的关系，为临床尽早做出诊断提供依据。方法按照Gilman诊断标准回顾性分析11例MSA患者的临床表现、分型和头颅MRI资料。结果本组诊断为很可能MSA11例，其中橄榄体脑桥小脑萎缩（MSA-C型）8例。2例在发病后3年头颅MRI脑桥“十字征”达Ⅰ期；1例在病后2年达Ⅱ期；3例分别在病后1年、3年、5年达Ⅲ期；另外2例分别在病后2年和7年达Ⅳ期。8例“壳核裂隙征”均为0期。黑质纹状体变性（MSA-P型）2例：1例病后6年脑桥“十字征”0期，“壳核裂隙征”Ⅰ期，另1例发病后9年“壳核裂隙征”Ⅱ期，脑桥“十字征”Ⅳ期。Shy-Drager综合征（MSA-A型）1例：病程5年，MRI检查脑桥“十字征”和“壳核裂隙征”分期均为0期。结论临床表现与头颅MRI检查发现的脑桥“十字征”和“壳核裂隙征”可作为及早识别MSA-C型的神经影像学改变特征，“壳核裂隙征”可作为识别MSA-P型的神经影像学改变特征。     

多系统萎缩的临床与CT及磁共振分析

目的　评价临床表现与ＣＴ、磁共振（ＭＲＩ） 结合对多系统萎缩（ＭＳＡ） 的诊断价值。方法　选择５６ 例多系统萎缩病人,其中橄榄桥脑小脑萎缩（ＯＰＣＡ）３７ 例,Ｓｈｙ－ Ｄｒａｇｅｒ 综合征（ＳＤＳ）１３ 例,纹状体黑质变性（ＳＮＤ）６ 例。全部病人均行头颅ＣＴ、ＭＲＩ检查,并对其临床表现、分型、ＭＲＩ进行比较。结果　临床发现各型早期各有特点,且ＭＲＩ表现也各不相同。结论　临床表现与ＭＲＩ结合可提高ＭＳＡ 中ＯＰＣＡ、ＳＮＤ的诊断率,但在ＳＤＳ病人ＭＤＩ改变不明显。头颅ＣＴ对ＭＳＡ 诊断意义不大。     

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.     

多系统萎缩2例报告

目的探讨多系统萎缩(multiple system atrophy,MSA)的临床表现和影像学特点,避免误诊。方法报告2例MSA并进行分析。结果 1例62岁女性,病程4 a,表现为尿失禁、便秘、行走不稳,头颅MRI:脑桥中央竖线样信号增高并壳核外侧线样高信号。另1例53岁女性,病程3 a,表现为双下肢乏力、言语含糊、行走不稳,头颅MRI:脑桥见十字征。结论最初的症状中出现体位性低血压,并且MRI出现十字征和壳核边缘高信号征有助于MSA的准确诊断。     

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。     

3O例多系统萎缩的临床与影像学分析

多系统变性(MSD)是指任何原发性神经元变性疾病,包括亨亭顿舞蹈病、皮克病、Friedreich共济失调等疾病,Takei和Mirra把橄榄桥脑小脑萎缩(OP-CA)、黑质纹状体变性(SND)、夏伊—德雷格综合症(SDS)三者亦归类于多系统变性。但Graham和Op-penheimer提出不同观点:认为这三个独立疾病(OPCA、SND、SDS)是具异质性的同一种疾病,应命名为多系统萎缩(MSA),这个观点在相当长时期内并未引起人们的重视。我们参照Gilman的诊断标准,对我科1997年2月～2002年10月5年间收治的临床诊断为OPCA、SND、SDS和MSD共30例病人的临床资料和影像学结果作一分析,探讨Gilman有关MSA诊断标准的可行性。     

多系统萎缩的临床和CT、MRI特点

目的了解多系统萎缩(multiple system atrophy,MSA)患者的临床基本特征及CT和MRI的特点.方法回顾性地分析了1994年～2001年大连医科大学附属二院神经科40例MSA患者的临床资料、CT和MRI资料和随访结果.结果 MSA平均发病年龄为52.2±6.8岁,男性占70%,女性占30%;患者多为慢性发病,进行性加重,对美多巴治疗无明显效果.神经系统症状复杂,头颅CT和MRI可见脑干、小脑萎缩,环池及四脑室扩大.结论 MSA临床以自主神经症状、锥体外系、小脑性共济失调为主,CT和MRI主要表现脑干、小脑萎缩,环池及四脑室扩大,但MRI较CT敏感度更高.     

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...     

橄榄-脑桥-小脑萎缩与“十字征”八例

目的探讨MRI脑桥十字征的橄榄-脑桥-小脑萎缩(OPCA)患者临床与影像学特点。方法总结8例经临床诊断为OPCA,分析其临床特点及影像学特征表现。结果 8例患者的临床表现均以小脑性共济失调和脑干功能受损伴有自主神经功能障碍为主,MRI在脑桥轴位T2WI上均出现典型的"十字征"。结论以小脑性共济失调和脑干功能受损为主要表现的OPCA患者,多伴有自主神经功能障碍,脑桥十字征是MRI特征性表现之一,有助于该病的诊断。     

多系统萎缩的临床与MRI特征

目的探讨多系统萎缩(MSA)的临床与MRI特征及其对临床亚型诊断的意义。方法回顾性分析28例MSA患者的临床及MRI资料。结果橄榄脑桥小脑萎缩(OPCA)以小脑体征(75.0%)突出,MRI表现为脑桥萎缩(91.7%)、小脑蚓部萎缩(91.7%),第四脑室扩大(83.8%),T2WI出现脑桥、小脑对称性高信号(63.6%)及脑桥十字征;纹状体黑质变性(SND)以锥体外系症状(80.0%)明显,MRI改变多位于基底节核团,表现为壳核萎缩(60.0%),T2WI示壳核外侧缘缝隙样高信号(80.0%);ShyDrager综合征(SDS)以自主神经症状(81.8%)为主,出现早且重,MRI未见特异性变化。结论MRI有助于提高MSA及其亚型诊断。脑桥萎缩、T2WI高信号改变,尤其是脑桥十字征的出现有助于OPCA诊断;壳核萎缩与T2WI壳核外侧缘缝隙样高信号改变支持SND诊断。     

Hemi-parkinsonism in multiple system atrophy: a PET and MRI study

We selected 6 patients presenting with hemi-parkinsonism from a total of 20 patients with probable multiple system atrophy (MSA) and studied their nigrostriatal lesions using magnetic resonance (MR) imaging and positron emission tomography (PET) with ¹⁸F-labeled 2-deoxy-2-fluoro- -glucose (FDG). T₂ weighted MR images demonstrated a decreased signal intensity in the putamen of all patients. This decreased signal was more intense in the nucleus contralateral to the affected body side in 5 patients. A decreased signal in the substantia nigra was found, expanding more on the contralateral side in 3 patients. T₁-weighted images showed that the contralateral putamen was smaller in size than the ipsilateral. These findings indicated that the iron deposit and the neuronal cell loss in the degenerative process were more remarkable in the contralateral nuclei. FDG uptake in 5 patients had likewise declined more in the contralateral than in the ipsilateral putamen. The study shows that these patients have the nigrostriatal lesions as described in previous reports on MSA and that an asymmetric lesion relating to clinical signs is present in the nigrostriatal system. When a patient presents with hemi-parkinsonism alone, MR imaging and PET/FDG are useful for the clinical diagnosis of MSA.     

Progressive cerebral atrophy in multiple system atrophy

Nine patients with multiple system atrophy (MSA) were studied based on MRI findings of cerebral hemispheric involvement. The age at onset was 56.4+/-8.6 (mean+/-S.D.) years, duration of illness at the first MRI study 2.1+/-1.1 years, duration of illness at the last study 9.7+/-2.6 years, and the follow-up duration 7.6+/-2.3 years. Controls were 85 neurologically intact persons (60.2+/-11.1 years age). In the MRI study, measurements of the ratio of each area to the intracranial area were performed for the cerebral hemisphere, frontal, temporal and parietal-occipital lobes. A significant progression of atrophy to under the normal limit was observed in the cerebrum, frontal and temporal lobes. Besides the typical pathological lesions in MSA, five autopsied patients revealed frontal lobe atrophy with mild gliosis, mild demyelination and glial cytoplasmic inclusions (GCIs). One of these patients showed remarkable frontal lobe atrophy with degenerative changes in the cerebral cortex. We observed the involvement of the cerebral hemisphere, especially the frontal lobe.     

Longitudinal quantitative MRI in multiple system atrophy and progressive supranuclear palsy

ObjectiveMRI has been used in parkinsonism to assess atrophy, tissue water diffusivity, and mineral deposition but usually at a single time-point. However, multiple system atrophy (MSA) and progressive supranuclear palsy (PSP) are progressive diseases. This study assessed the value of longitudinal MRI in characterizing the time course of the degenerative process.MethodsTwo serial MRIs (mean 23 months apart) were retrospectively analyzed in 12 MSA, 6 PSP, and 18 age and sex matched controls. Assessment included selected cross-sectional areas, regional apparent diffusion coefficient (ADC) and gradient echo (GRE) intensity ratios of the lateral ventricles, caudate, putamen, middle cerebellar peduncle, pons and midbrain.ResultsOn follow-up imaging, there was a larger ADC increase in the putamen in PSP over time compared to controls (p = 0.02). In MSA there was greater volume loss in the pons over time compared to controls (p = 0.002). In MSA the changes in middle cerebellar peduncle ADC were correlated with motor symptom severity according to the Unified Parkinson's Disease Rating Scale Part III (p = 0.005).ConclusionsEvidence of progressive neurodegeneration can be observed on MRI in MSA and PSP within two years consisting of increasing putaminal ADC in PSP and pontine atrophy in MSA.     

Young‐onset multiple system atrophy: Clinical and pathological features

Background: The onset of multiple system atrophy (MSA) before age 40 years is referred to as “young‐onset MSA.” We identified clinical and pathological characteristics that might help with its early diagnosis and distinction from young‐onset Parkinson's disease and late‐onset MSA. Methods: We reviewed the available clinical and pathological features in cases that fulfilled consensus criteria for diagnosis of probable MSA or had autopsy confirmed MSA with an onset before age 40 years and compared the clinical features with 16 autopsy confirmed cases with young‐onset Parkinson's disease and a large published series of late‐onset MSA from the European MSA Study Group. Results: We identified 22 patients with young‐onset MSA, 8 of whom had available pathology. The mean age of onset was 36.7 years (standard deviation 2.3). Levodopa‐induced dyskinesia was more common, whereas myoclonus and pyramidal signs were less common in young‐onset Parkinson's disease when compared with young‐onset MSA. Dystonia, levodopa responsiveness, levodopa‐induced dyskinesia, and pyramidal signs were more common (P < .05) when compared with the data in late‐onset MSA. On postmortem analysis, the minimal‐change pathological variant was more common in young‐onset MSA (n = 2) than late‐onset MSA (P = .045), with a mean survival of 11.1 ± 3.2 years (range 5.5‐14.6) in pathologically confirmed cases of young‐onset MSA. Conclusion: This study has identified useful differences that may improve diagnostic accuracy, help us understand the pathological basis, and assist clinicians with the early diagnosis of young‐onset MSA. © 2018 International Parkinson and Movement Disorder Society     

多系统萎缩的临床与病理特点及诊断

目的 探讨多系统萎缩(multiple system atrophy,MSA)的临床和病理特点及诊断.方法 总结4例MSA患者资料,其中2例为病理资料,2例为临床资料.经解剖检验证实的MSA患者2例,通过苏术素-伊红(HE)、髓鞘染色(KB)及Gallyag-Braak(GB)银染色,并通过泛素、α-突触核蛋白等免疫组织化学染色进行观察.结果 4例患者均为男性,年龄分别为76、70、51及59岁.临床诊断:例1为帕金森综合征,例2为脊髓小脑共济失调,例3、例4均诊断为MSA.病理改变的大体所见:脑桥和小脑明显萎缩,壳核、苍白球、黑质也可见萎缩,脑室扩张.组织学所见:大脑皮质、黑质、纹状体、苍白球、脑桥核、下橄榄、无名质、迷走神绛背核、小脑、脊髓中间外侧角细胞及前角细胞等部位神经细胞脱失,胶质增生,上述部位自质可见广泛弥漫的少突胶质细胞胞质内缠结样包涵体.结论 临床上橄榄-脑桥-小脑萎缩常以脑桥、小脑及自主神经症状为主,Shy-Drager综合征以直立性低血压症状为主,纹状体黑质变性常以锥体外系症状为主.因上述3个疾病有共同的病理基础,故倾向于将其归为MSA的亚型.

Abstract：

Objective To study the clinical,pathological characteristics and diagnosis of multiple system atrophy(MSA).Methods Among 4 cases of MSA,2 of them were from clinical data,the other 2were frum pathological data.Two cases verified by autopsy were investigated using Hematoxylin-eosi,Kltiver.Barrera and Gallvas-Braak silver staining and confirmed by immunohistochemistry using ubiquitin,α-synuclein staining.Results All 4 patients were male.aged 51-76.Case No.1 wag diagnosed as Parkinson's svndrome.Case No.2 was diagnosed as spinal ataxia cerebella,and the other two eases were diagnosed as MSA.The following changes were found by pathological studies.Macroscopic atrophies were presented in pens and cerebella.as well as putamen,globus pallidus and substantia nigra.Cerebral ventricles were dilated.Neuronal lOSS and gliosis could be seen at cerebral cortex.nigrostriatal.globus pallidus,pontine nuclei,subslantia innominata,inferior olives,doral motor nucleus of vagus,cerebellum antl intermediolateral column of the spinal cord.In the white matter of these regions cytoplasmic inclusions bodies were extensively present in oligodendrocytes.Conclusions Olivopontocerebellar atrophy mainly shows the,clinical symptoms of pens,cerebellum,and autonomic nerves damage;Shy-Drager syndrome presents mainly with the erecting hypotenstion symptom,while striatonigral degeneration mainly involves extrapyramidal system.As these three diseases share the common basic pathological changes,they are preferred to be classified as the subtype of 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.