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。     

帕金森叠加综合征10例临床分析

目的 分析帕金森叠加综合征的临床特点、影像学特征,为临床诊断提供依据.方法 回顾性分析10例帕金森叠加综合征患者的临床及影像学资料.结果 诊断多系统萎缩(MSA)-C型5例,MSA-P型2例,进行性核上性麻痹(PSP)3例.MSA-C型以小脑症状突出,MSA-P型以锥体外系症状表现为主,PSP以姿势异常、躯干僵直、核上性眼球运动障碍为主要表现.头颅MRI显示MSA-C型的主要病变在脑干、小脑,MSA-P型病变在壳核,PSP的主要病变在中脑.结论 临床表现结合头颅MRI检查可提高帕金森叠加综合征的诊断率.脑干、小脑萎缩有助于MSA-C型诊断,壳核萎缩及壳核背外缘T2WI低信号支持MSA-P型诊断,中脑萎缩支持PSP诊断.     

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.     

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.     

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.     

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.     

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.     

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.     

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.     

Longitudinal MRI study of multiple system atrophy – when do the findings appear,and what is the course?

Several investigators have revealed features of multiple system atrophy (MSA) by magnetic resonance imaging (MRI). For use in clinical diagnosis, we determined the exact time when two main features of pontine and putaminal intensity changes appeared. Furthermore, in order to reveal the course from when the disorder first appeared and how it spread, we also investigated the course of MRI findings and differences between clinical subtypes. The cranial MRI of 42 patients with MSA were longitudinally studied including comments on the so called “cross sign” of pontine T2 high intensity, which was divided into 6 stages, and also on the linear T2 high intensity of the dorsolateral side of the putamen (“putaminal slit”) which was divided into 4 stages. Patients were classified as 16 MSA-C, 7 autonomic dominant type (MSA-A), and 19 MSA-P. The age at onset ranged from 41 to 74 years (mean, 55 ± 9). The duration of the disease in the MRI study ranged from 1 to 24 years. The pontine “cross sign” was completed (shows Cross, stage IV) earlier in MSA-C mainly before 5 years, later in MSA-P and even much later in MSA-A. Regarding the “putaminal slit”, MSA-P shows earlier bilateral changes (stage II), mostly before 3 years, compared with MSA-C, which requires 4 years to reveal even a unilateral change (stage I), or MSA-A which requires even more time. MRI findings showed a tendency to relate to clinical findings, since MSA-C exhibits “cross sign” completion earlier than bilateral “putaminal slit”; however, MSA-P shows bilateral “putaminal slit” earlier than “cross sign”, and MSA-A requires much more time to show both. Clinically, MSA-C, MSA-A, or MSA-P showed different MRI courses so that three subtypes could be defined also with MRI findings. Therefore these observations are useful not only for diagnosis of MSA itself, but also to distinguish clinical subtypes (MSA-C, MSA-A, or MSA-P) which have different rates of lesion progression. Received: 5 September 2001, Received in revised form: 10 December 2001, Accepted: 17 December 2001     

Multiple regional 1H-MR spectroscopy in multiple system atrophy: NAA/Cr reduction in pontine base as a valuable diagnostic marker

OBJECTIVE: We performed (1)H-MR spectroscopy ((1)H-MRS) on multiple brain regions to determine the metabolite pattern and diagnostic utility of (1)H-MRS in multiple system atrophy (MSA). METHODS: Examining single voxels at 3.0 T, we studied metabolic findings of the putamen, pontine base, and cerebral white matter in 24 MSA patients (predominant cerebellar ataxia (MSA-C), n = 13), parkinsonism (MSA-P), n = 11), in 11 age and duration matched Parkinson's disease patients (PD) and in 18 age matched control subjects. RESULTS: The N-acetylaspartate to creatine ratio (NAA/Cr) in MSA patients showed a significant reduction in the pontine base (p<0.0001) and putamen (p = 0.02) compared with controls. NAA/Cr in cerebral white matter also tended to decline in long standing cases. NAA/Cr reduction in the pontine base was prominent in both MSA-P (p<0.0001) and MSA-C (p<0.0001), and putaminal NAA/Cr reduction was significant in MSA-P (p = 0.009). It was also significant in patients who were in an early phase of their disease, and in those who showed no ataxic symptoms or parkinsonism, or did not show any MRI abnormality of the "hot cross bun" sign or hyperintense putaminal rims. NAA/Cr in MSA-P patients was significantly reduced in the pontine base (p = 0.001) and putamen (p = 0.002) compared with PD patients. The combined (1)H-MRS in the putamen and pontine base served to distinguish patients with MSA-P from PD more clearly. CONCLUSIONS:(1)H-MRS showed widespread neuronal and axonal involvement in MSA. The NAA/Cr reduction in the pontine base proved highly informative in the early diagnosis of MSA prior to MRI changes and even before any clinical manifestation of symptoms.     

Multiple system atrophy: natural history, MRI morphology, and dopamine receptor imaging with 123IBZM-SPECT.

Sixteen patients with a clinical diagnosis of probable multiple system atrophy (MSA) were examined clinically by MRI and by 123I-iodobenzamide single photon emission computed tomography (IBZM-SPECT). The clinical records of another 16 patients were also analysed retrospectively. On the basis of their clinical presentation, patients were subdivided into those with prominent parkinsonism (MSA-P, n = 11) and those with prominent cerebellar ataxia (MSA-C, n = 21). Autonomic symptoms were present in all patients and preceded the onset of motor symptoms in 63% of patients. Calculated median lifetime and the median time to become wheelchair bound after onset of disease were significantly shorter for MSA-P than for MSA-C (lifetime: 4.0 v 9.1 years; wheelchair: 3.1 vs 5.0 years) suggesting a better prognosis for cerebellar patients. A significant loss of striatal dopamine receptors (below 2 SD threshold) was detected by IBZM-SPECT in 63% of the patients (56% below 2.5 SD threshold). There was no difference between patients with MSA-C and those with MSA-P in the proportion with significant receptor loss and the extent of dopamine receptor loss. Planimetric MRI evaluation showed cerebellar and brainstem atrophy in both groups. Atrophy was more pronounced in patients with MSA-C than in those with MSA-P. Pontocerebellar hyperintensities and putaminal hypointensities on T2 weighted MRI were found in both groups. Pontocerebellar signal abnormalities were more pronounced in MSA-C than in MSA-P, whereas the rating scores for area but not for intensity of putaminal abnormalities were higher in MSA-P. MRI and IBZM-SPECT provide in vivo evidence for combined basal ganglia and pontocerebellar involvement in almost all patients in this series.     

The "Cross" Signs in Patients With Multiple System Atrophy: A Quantitative Study

Patients with multiple system atrophy (MSA) may show the "cross" sign in the pontine base that has been considered as an expression of the degeneration of pontine neurons and transverse pontocerebellar fibers. However, correlations between pontine base atrophy and existence of "cross" sign have not been fully investigated. The authors studied 68 patients with MSA (47 MSA-C [predominantly cerebellar ataxia], 21 MSA-P [predominantly parkinsonism], mean [+/-SD ] 58.7 +/- 10.9 years). T1-weighted (T1W) sagittal and axial images and T2-weighted (T2W) axial images were obtained for all patients and controls. To measure the areas of pontine basis and cerebellar vermis, the authors used midsagittal T1W images and analyzed a bit map transformed on a computer. They classified atrophy in the pontine base into 3 grades. There is significant correlation between atrophies of pontine base and existence of the cross sign. All patients with a smaller area of pontine base 2 standard deviations below those of normal controls had the cross sign. This supports that existence of the cross sign depends only on the extent of pontine base atrophies.     

Non-motor symptoms and the quality of life in multiple system atrophy with different subtypes

BackgroundThe differences in non-motor symptoms (NMS) and quality of life (QOL) between MSA patients with different subtypes remain unknown, so do the determinants of poor QOL in both subtypes.MethodsA total of 172 MSA patients were enrolled in the study. NMS of patients with MSA were assessed using the non-motor symptoms scale (NMSS) and Parkinson's Disease Questionnaire-39 item version (PDQ-39) was used to evaluate the QOL of patients with MSA.ResultsThe most prevalent NMS domain was urinary (91.3%) in both subtypes. The mood/apathy domain was more severe in MSA-P than MSA-C patients (P < 0.05). Drooling, constipation, and pain symptoms were more prevalent and severe in the MSA-P patients compared to the MSA-C patients (P < 0.05). We found that the MSA-C patients had a higher score of mobility than the MSA-P patients (P = 0.002); However, the MSA-P patients had a higher score of bodily discomfort than the MSA-C patients (P = 0.036). There were close correlations between NMS and PDQ-39 in both subtypes. Disease severity, cardiovascular symptoms, sleep/fatigue symptoms and gastrointestinal symptoms were determinants of poor QOL in MSA-P patients. While in MSA-C patients, longer disease duration, disease severity and mood/apathy symptoms were determinants of poor QOL.ConclusionNMS are more severe and prevalent in MSA-P patients, especially for mood/apathy and gastrointestinal symptoms. There is a close relationship between NMS and QOL in both MSA subtypes. Disease severity, longer disease duration and severe NMS are determinants of poor QOL in MSA.     

Visual event-related potential changes in two subtypes of multiple system atrophy,MSA-C and MSA-P

We investigated the visual event-related potentials (ERPs) in two subtypes of multisystem atrophy (MSA) in 15 MSA-C patients, 12 MSA-P patients, and 21 normal control (NC) subjects. We used a visual oddball task to elicit ERPs. No significant changes were seen in N1 or N2 latency, in either MSA-C or MSA-P, compared with the NC group. An early stage of visual information process related to N1 and a visual discrimination process related to N2 might be preserved in both MSA-C and MSA-P. The P3a peak was more frequently undetectable in MSA than in the NC group. Significant P3a amplitude reduction in both MSA-C and MSA-P suggests impairment of the automatic cognitive processing in both MSA-C and MSA-P. Significant difference was found in P3b latency and P3b amplitude only in MSA-C, compared with the NC group. The result suggests the impairment of the controlled cognitive processing after the visual discrimination process in the MSA-C group. We further investigated the correlation between visual ERP changes and magnetic resonance imaging (MRI) data. Quantitative MRI measurements showed reduced size of the pons, cerebellum, perisylvian cerebral area, and deep cerebral gray matter in both MSA-C and MSA-P, and of the corpus callosum only in MSA-P, as compared to NC group. In both MSA-C and MSA-P, P3b latency was significantly correlated with the size on MRI of the pons and the cerebellum. P3b latency in the whole MSA group was also significantly correlated with the size of the pons and the cerebellum. These results indicate that P3b latency changes in parallel with the volume of the pons and the cerebellum in both MSA-C and MSA-P. Received: 28 August 2001 Received in revised form: 22 January 2002 Accepted: 25 January 2002     

Magnetic resonance imaging distinguishes progressive supranuclear palsy from multiple system atrophy

Summary. To establish diagnostic magnetic resonance imaging (MRI) criteria for differentiating progressive supranuclear palsy (PSP) from multiple system atrophy (MSA), magnetic resonance images from eight patients with probable PSP, 30 with probable MSA {nine striatonigral degeneration (MSA-P) and 21 olivopontocerebellar atrophy (MSA-C)}, and ten age-matched controls were retrospectively studied. Anteroposterior diameters in the midline sagittal T1-weighted image of the rostral (RMT) and caudal midbrain tegmentum (CMT), caudal pons and medulla were measured. Divergence of the red nuclei (RN) in the axial T2-weighted image was judged. All PSP images had a smaller RMT diameter than the lower limit of the normal range, showed RN divergence, and had a pontine diameter within the normal range. All MSA images had a CMT diameter within the normal range; no MSA images showed divergence of RN. Forty-four percent (4/9) of MSA-P and 76% (16/21) of MSA-C images had a pontine diameter smaller than the lower limit of the normal range. On basis of the results, we propose MRI diagnostic criteria for differentiating PSP from MSA. Received March 23, 2000; accepted June 7, 2000     

Cerebellar and parkinsonian phenotypes in multiple system atrophy: similarities,differences and survival

Multiple system atrophy (MSA) is a neurodegenerative disease with two motor phenotypes: parkinsonian (MSA-P) and cerebellar (MSA-C). To elucidate whether in addition to the motor abnormalities there are other significant differences between these phenotypes, we performed a retrospective review of 100 patients (61 males, 39 females) with a diagnosis of possible (12 %), or probable (88 %) MSA. Four patients eventually had post-mortem confirmation (i.e., definite MSA). Sixty percent were classified as having MSA-P and 40 % as MSA-C. MSA-C and MSA-P patients had similar male prevalence (60 %), age of onset (56 ± 9 years), and frequency of OH (69 %). Brain MRI abnormalities were more frequent in MSA-C patients (p < 0.001). Mean survival was 8 ± 3 years for MSA-C and 9 ± 4 years for MSA-P patients (p = 0.22). Disease onset before 55 years predicted longer survival in both phenotypes. Initial autonomic involvement did not influence survival. We conclude that patients with both motor phenotypes have mostly similar survivals and demographic distributions. The differences here identified could help counseling of patients with MSA.     

Does the Type of Multisystem Atrophy,Parkinsonism, or Cerebellar Ataxia Impact on the Nature of Sleep Disorders?

Multiple system atrophy (MSA) is a neurodegenerative disease characterized by a combination of autonomic failure, parkinsonism, and/or cerebellar ataxia. The cause of MSA is unknown, but neuropathologically the disease is characterized by widespread α-synuclein-positive glial cytoplasmic inclusions and striatonigral and/or olivopontocerebellar neurodegeneration. Two motor phenotypes have been clinically identified: parkinsonian (MSA-P) and cerebellar (MSA-C). In order to elucidate if in addition to the motor abnormalities there are other significant differences between these two phenotypes, we performed a review of the studies on sleep disorders in the two MSA subtypes. Substantially, any significant difference in the sleep structure, as well as in the frequency and severity of the sleep disorders, has been found between MSA-P and MSA-C patients. Recent studies clearly showed similarities between the two MSA subtypes in terms of demographic distributions, natural history of the disease, and survivals. These findings suggest that although the dominant clinical presentations differ between MSA-C and MSA-P, a common pathophysiology may underlie both subtypes of MSA.