帕金森病/帕金森综合征~(18)F-FDG PET脑显像技术操作及报告解读要素

~(18)氟-脱氧葡萄糖正电子发射断层扫描(~(18)F-FDG PET)脑显像通过呈现脑部葡萄糖代谢的分布特征,可反映大脑局部的突触活性以及与疾病发生、进展特异相关的生理病理学改变,已被证实在帕金森病/帕金森综合征的鉴别诊断中具有较好的临床应用价值。文中在强调重视~(18)F-FDG PET脑显像技术操作规范的基础上,总结综述了原发性帕金森病、多系统萎缩、进行性核上性麻痹、路易体痴呆、皮质基底节变性等~(18)F-FDG PET脑代谢特征,并提出相关影像学数据分析软件的潜力和局限性,旨在对帕金森病/帕金森综合征患者~(18)F-FDG PET脑显像的报告解读及其在疾病鉴别诊断中的临床应用提出基本的指导性建议。     

不同类型痴呆脑代谢改变图型:~(18)F-FDG PET显像

目的探讨不同类型痴呆患者基于像素水平的脑代谢图型特点。方法对最终临床诊断为阿尔茨海默病(20例)、额颞叶痴呆(20例)、路易体痴呆(10例)、进行性核上性麻痹(7例)、原发性进行性失语(3例)、皮质基底节变性(1例)和多系统萎缩(1例)等认知功能障碍患者的18F-FDG PET显像资料进行回顾分析,描述各种神经变性疾病脑代谢降低区域和程度。结果 SPM分析表明,各种神经变性疾病引起的痴呆18F-FDG PET显像均表现为皮质代谢降低,但其代谢图型变化明显不同:阿尔茨海默病组以双侧颞顶叶和额叶皮质代谢降低为主,基本感觉运动皮质、枕叶、基底节和丘脑活性保留;额颞叶痴呆组额叶和颞叶皮质不对称性代谢降低,伴部分顶叶皮质和基底节、丘脑等皮质下核团不同程度代谢降低;路易体痴呆组枕叶、视皮质和双侧颞上回前部代谢降低;进行性核上性麻痹组双侧前额叶背外侧、颞叶前外侧、中脑和双侧尾状核代谢降低;原发性进行性失语组左侧额叶Broca区、左侧颞叶皮质(除左侧颞上回后部)和右侧颞叶内侧皮质代谢降低;皮质基底节变性组双侧中央沟周围额顶叶皮质(右侧显著)、右侧基底节代谢降低;多系统萎缩组双侧小脑背外侧皮质和左侧壳核代谢降低。结论神经变性疾病所致痴呆在18F-FDG PET显像中表现出各自特征性脑代谢降低图型,18F-FDG PET显像有可能成为痴呆鉴别诊断的一种辅助手段。     

帕金森叠加综合征神经精神症状及PET影像学特征

目的探讨帕金森叠加综合征患者的神经精神症状和~(18)F-脱氧葡萄糖(~(18)F-FDG)PET影像学特征。方法共8例很可能的帕金森叠加综合征患者,包括以小脑共济失调为主要表现的多系统萎缩(MSA-C型)1例、进行性核上性麻痹4例、皮质基底节变性1例、路易体痴呆2例,采用简易智能状态检查量表(MMSE)和蒙特利尔认知评价量表(MoCA)评价认知功能,神经精神科问卷评价神经精神行为,汉密尔顿抑郁量表21项评价情绪状态。结果 1例MSA-C型患者仅表现为焦虑。4例进行性核上性麻痹患者均存在不同程度认知功能障碍,其中3例还表现出明显抑郁、焦虑、易激惹和睡眠障碍。1例皮质基底节变性患者表现为执行功能和视空间能力、言语功能、注意力和定向力障碍,以及抑郁、焦虑、易激惹和睡眠障碍。2例路易体痴呆患者均不能复制MMSE量表之五边形,MoCA量表之画钟测验仅能勾画圆形轮廓,同时表现为幻觉、抑郁和淡漠。~(18)F-FDG PET显像,1例MSA-C型患者为小脑葡萄糖代谢降低;4例进行性核上性麻痹患者均为双侧对称性额叶、前扣带回、顶叶代谢降低,尤以丘脑、基底节区和脑干显著;1例皮质基底节变性患者为右侧额颞顶枕叶外侧、左侧顶叶外侧、双侧扣带回和楔前叶代谢降低;2例路易体痴呆患者均为双侧颞枕叶代谢降低。结论帕金森叠加综合征早期易误诊为心理疾病而延误治疗,还可因使用镇静催眠药而加重运动障碍和锥体外系症状。神经心理学改变与认知功能障碍有关。PET显像对疾病的早期诊断有重要临床价值。     

P型多系统萎缩与帕金森病的18F-脱氧葡萄糖脑代谢比较

目的 研究P型多系统萎缩(multiple system atrophy,MSA-P)与帕金森病(Parkinson's disease,PD)的脑部葡萄糖代谢差异.方法 MSA-P患者15例,PD患者32例,无神经系统疾病的健康对照8名,进行18F-脱氧葡萄糖(FDG)PET检查.分别取尾状核、豆状核、丘脑、小脑、双侧额叶、双侧顶叶、双侧颞叶和双侧枕叶为感兴趣区(ROI).应用PET专用软件计算各ROI的FDG放射性同位素值,以颅内各部位ROI的18F-FDG代谢比值为指标.结果 MSA-P的额叶、颞叶、顶叶、纹状体、丘脑的18F-FDG脑代谢与健康对照和帕金森病相比呈现对称性下降;丘脑18F-FDG代谢高于纹状体以及额、顶叶皮质;18F-FDG代谢比值同侧豆状核与丘脑为0.493±0.085,同侧尾状核头与丘脑为0.453±0.079.PD的额、顶、颞叶皮质18F-FDG代谢分别高于纹状体、丘脑;纹状体高于丘脑,首发症状肢体对侧豆状核与丘脑18F-FDG代谢比值为1.131±0.113;基底节代谢不对称.MSA-P的豆状核与丘脑18F-FDG代谢比值,同PD的豆状核与丘脑18F-FDG代谢比值相比差异有统计学意义(P＜0.01).结论 MSA-P与帕金森病的葡萄糖代谢差异显著,可以应用18F-FDG PET检查作为鉴别诊断的重要方法之一.     

阿尔茨海默病18F-FDG PET显像诊断的研究☆

目的探讨阿尔茨海默病(AD)脑葡萄糖代谢及其18F-脱氧葡萄糖正电子发射计算机断层扫描(18F-FDGPET)显像的影像学特征和PET诊断标准.方法静脉注射18F-FDG后行脑断层显像,检查13例AD、13例非AD痴呆及13例正常人.获得纹状体、丘脑、黑质、顶叶、颞叶、额叶、枕叶、海马单位面积放射性计数与小脑计数的比值(Rcl/cb),进行半定量分析,并与MR进行对照.结果AD患者PET异常率为100%,MR异常者占10/13.PET显像特征①对称性双侧颞顶叶及海马伴额叶或枕叶代谢减低占9例(9/13);②双侧颞叶对称性代谢减低伴海马或额叶代谢下降占3例(3/13);③双顶叶对称性代谢降低1例(1/13).12例(12/13)非AD痴呆表现为不对称、多发性代谢降低,降低区位于黑质、纹状体、丘脑及脑皮质区,MR异常率为11/13.结论在除外脑内结构特异性损害基础上,PET发现对称性双颞顶叶、海马或颞叶、顶叶,伴或不伴枕叶、额叶代谢下降,可诊断AD.PET对AD早期诊断及鉴别诊断具有临床意义.     

阿尔茨海默病~(18)F-FDG PET显像诊断的研究

目的 探讨阿尔茨海默病（AD）脑葡萄糖代谢及其18F-脱氧葡萄糖正电子发射计算机断层扫描（18F-FDG PET）显像的影像学特征和PET诊断标准。方法 静脉注射18F-FDG后行脑断层显像,检查13例 AD、13例非AD痴呆及13例正常人。获得纹状体、丘脑、黑质、顶叶、颞叶、额叶、枕叶、海马单位面积放射性计数与小脑计数的比值（Rcl/cb）,进行半定量分析,并与MR进行对照。结果AD患者PET异常率为100％,MR异常者占10/13。PET显像特征:①对称性双侧颞顶叶及海马伴额叶或枕叶代谢减低占9例（9/13）;②双侧颞叶对称性代谢减低伴海马或额叶代谢下降占3例（3/13）;③双顶叶对称性代谢降低1例（1/13）。12例（12/13）非AD痴呆表现为不对称、多发性代谢降低,降低区位于黑质、纹状体、丘脑及脑皮质区,MR异常率为11/13。结论 在除外脑内结构特异性损害基础上,PET发现对称性双颞顶叶、海马或颞叶、顶叶,伴或不伴枕叶、额叶代谢下降,可诊断AD。PET对AD早期诊断及鉴别诊断具有临床意义。     

抽动秽语综合征18F-FDG PET显像研究

目的分析比较正常人和抽动秽语综合征(TS)手术前后18F-FDG PET显像,探讨TS发病机制及手术治疗的可行性。方法经临床确认为TS的患者18例,行单侧苍白球切开术。术前1d和术后3月,在相同条件下分别行18F-FDG PET显像。目视法观察双侧运动区、基底节和小脑的18F-FDG分布。半定量分析法计算各观察部位与小脑18F-FDG摄取量的比值。采用全面评估抽动严重程度的YGTSS量表,对所有患者手术前后病情进行量化评估,以判断手术后的症状改善率,同时与20例正常人的脑显像比较。结果正常对照组,双侧感觉运动区皮质、双侧基底节、双侧小脑的18F-FDG呈对称分布。与正常对照比较,18例TS双侧感觉运动皮质18F-FDG摄取明显增高(P<0.01),双侧基底节18F-FDG摄取减低(P<0.05)。手术前后比较,术后手术侧感觉运动区皮质的18F-FDG分布减低(P<0.01),而基底节无明显变化(P>0.05)。18例患者术后症状改善率在63%~77%。结论双侧感觉运动区皮质18F-FDG摄取明显增高,双侧基底节18F-FDG摄取减低,为TS的18F-FDG PET显像特征。苍白球切开术明显地改善了对侧肢体抽动症状,使手术侧感觉运动区皮质18F-FDG摄取减低。     

帕金森病痴呆患者的神经心理测试与18氟-氟代脱氧葡萄糖正电子发射断层摄影术脑显像分析

目的 研究帕金森病痴呆(PDD)患者的神经心理学特点及18氟-氟代脱氧葡萄糖(18F-FDG)PET脑代谢显像的影像学特征.方法 (1)对12例PDND患者、12例PDD患者和12名健康对照者运用蒙特利尔认知评估量表(MoCA)进行检查.(2)静脉注射18F-FDG后行PET脑代谢显像,获得12例PDND患者和12例PDD患者的PET图像.结果 (1)PDD组的MoCA总成绩(分)低于PDND组,其中MoCA子项目中的视空间与执行功能(1.50±1.08与4.25 ±0.87,t=-1.891)、注意(3.67±1.37与6.00 ±0.00,t’=-2.199)、语言(1.83 ±0.94与2.67±0.49,t'=-2.745)、抽象(0.67 ±0.65与1.75±0.45,t=-4.732)、延迟回忆(0.25 ±0.45与3.00±1.28,t’=-7.021)和定向(4.25±1.71与6.00±0.00,t’=-3.545)成绩低于PDND组,差异均具有统计学意义(均P＜0.05).PDD组MoCA总成绩低于健康对照组,差异具有统计学意义(15.10 ±3.82与28.10±1.16,t'=-11.280,P＜0.05).(2)18 F-FDG PET脑代谢显像:PDND组的大脑半球脑代谢不对称性轻度减低,PDD组的大脑半球脑代谢不对称性重度减低.结论 PDD表现为多认知域受损,以视空间与执行功能、注意、语言、抽象、延迟回忆和定向障碍较为严重.18F-FDG PET脑代谢显像检测对PDD的诊断可能有一定的帮助.     

克-雅病~(18)F-FDG PET影像学特点分析

目的总结克-雅病患者~(18)F-脱氧葡萄糖(~(18)F-FDG)PET影像学特点。方法与结果共纳入2018年8月至2020年1月8例克-雅病患者(包括散发性克-雅病7例、遗传性克-雅病1例),临床主要表现为快速进展性痴呆。DWI均存在皮质和(或)基底节区高信号(8/8例);~(18)F-FDG PET均呈单侧或双侧额顶颞叶葡萄糖低代谢(8/8例),其次依次为枕叶(7/8例)、丘脑(5/8例)、基底节区(3/8例)、脑干(3/8例)和小脑(3/8例)低代谢。对比分析葡萄糖低代谢区域与相应DWI高信号区域,发现皮质50个葡萄糖低代谢区域中40个呈DWI高信号,尤以额叶的一致性较好;皮质下25个葡萄糖低代谢区域中仅9个呈DWI高信号,尤以基底节区的一致性较好;脑干和小脑仅呈现葡萄糖低代谢。结论克-雅病的诊断与鉴别诊断具有较高的敏感性,特别是病情进展迅速的患者,~(18)F-FDG PET低代谢多提示病变部位神经功能严重缺损。对于快速进展性痴呆且伴明显锥体外系症状或小脑症状的患者,~(18)F-FDG PET显示皮质低代谢而DWI未见明显高信号,应考虑克-雅病的诊断。     

阿尔茨海默病的18F-FDG PET脑显像特征及意义

目的探讨轻、中、重度阿尔茨海默病(AD)患者的脑~(18)F-FDG正电子发射断层显像(PET/CT)特点。方法对10例AD患者和10例健康对照的脑~(18)F标记的脱氧葡萄糖正电子发射断层显像(~(18)F-FDG PET)进行视觉分析,测量感兴趣区的FDG标准化摄取值(SUV),并通过计算获得其与同侧小脑FDG的SUV的比值,比较组间是否有显著性差异。结果轻度AD患者的FDG摄取代谢降低区为后扣带回及楔前叶,且呈双侧不对称性,中度AD患者的颞叶、顶叶FDG代谢降低双侧对称且降低明显,重度AD患者全脑皮质代谢明显降低。结论 AD患者的~(18)F-FDG PET脑显像特征反映认知功能的损害程度,是临床诊断和评估AD的有力工具。     

Comparison of cerebral glucose metabolism between multiple system atrophy Parkinsonian type and Parkinson's disease

OBJECTIVE: To investigate the difference in the regional cerebral glucose metabolism between multiple system atrophy Parkinsonian type (MSA-P) and Parkinson's disease (PD). MATERIAL AND METHODS: Fifteen patients with MSA-P, 32 patients with PD and eight cases of healthy control underwent positron emission tomography (PET) with (18)F-fluorodeoxyglucose ((18)F-FDG) showing glucose metabolism. Glucose metabolism ratios of various cerebral regions were compared as an indicator of regional cerebral glucose metabolic patterns. RESULTS: The metabolism ratios of frontal lobe/occipital lobe, parietal lobe/occipital lobe, temporal lobe/occipital lobe and corpus striatum/occipital lobe in patients with MSA-P were lower than those in patients with PD and control, respectively (p<0.01). For patients with MSAP, the metabolism ratio in thalamus was higher than those in lenticular nucleus and anterior cortical brain, respectively (p<0.01) and the changes of metabolism ratio in cortex, corpus striatum and thalamus were symmetric. For patients with PD, the metabolism ratio in corpus striatum was higher than that in thalamus and two side of the basal ganglia show asymmetric change of metabolism (p<0.01). CONCLUSION: This study suggests that significant differences exist in the patterns of regional cerebral glucose metabolism between MSA-P and PD. (18)F-FDG PET might be a useful adjunctive method for differential diagnosis between MSA-P and PD.     

P型和C型多系统萎缩的急性多巴反应性研究

目的研究P型和C型多系统萎缩（MSA）对左旋多巴的急性反应性。方法对P型MSA患者18例、C型MSA患者13例和帕金森病（PD）患者23例行急性阶梯式左旋多巴试验，药物剂量依次为左旋多巴／苄丝肼50mg／12．5mg、100mg／25mg、150mg／37．5mg、200mg／50mg和300mg／75mg。以UPDRS运动分量表作为评价标准，计算UPDRS运动评分平均最大改善率并比较各组患者的多巴反应性。结果左旋多巴／苄丝肼剂量为。100mg／25mg、150．mg／37．5mg、200mg／50mg和300mg／75mg时，MSA-P型组和MSA—C型组韵UPDRS运动评分平均最大改善率均显著低于PD组，MSA-P型组高于MSA-C型组。MSA-P型组患者随服用左旋多巴／苄丝肼剂量增加UPDRS评分平均最大改善率呈逐渐增高趋势，而MSA-C型不同剂量间UPDRS评分平均最大改善率差异无统计学意义。结论MSA-P型组具有剂量依赖的急性多巴反应性，而MSA-C型组基本无急性多巴反应性。     

Application of the International Cooperative Ataxia Scale rating in multiple system atrophy.

We assessed the International Cooperative Ataxia Scale (ICARS) as a means of extracting and rating cerebellar signs in multiple system atrophy (MSA). Cross-sectional analysis of internal consistency, factor structure, and correlation with parkinsonism severity (Unified Parkinson's Disease Rating Scale [UPDRS] III) of the ICARS, in 50 unselected MSA patients (mean age, 67.6 years; mean disease duration, 5.5 years), 50 age-matched and disease duration-matched Parkinson' disease (PD) patients, and 50 control subjects. Fifteen patients (30%) had MSA-C (cerebellar subtype) and 35 (70%) MSA-P (parkinsonism subtype), and 66% had at least one cerebellar sign. The total ICARS score was much higher (fivefold) in MSA compared to PD patients. The ICARS score was twofold higher in MSA-C than in MSA-P patients. MSA-C patients had a higher score than MSA-P mainly on posture and gait disturbances and kinetic functions subscores. All the ICARS items were significantly more severe in MSA than in PD patients, who in turn scored higher than the controls. In MSA, internal consistency was excellent (Cronbach = 0.93). Factor structure analysis revealed four clinically distinct subscores, in accordance with the scale structure, which accounted for 70% of the variance. The ICARS showed less consistency and accuracy in PD patients; however, the ICARS scores significantly correlated with the UPDRS-III scores in both MSA and PD patients. The ICARS appears a useful tool to extract and rate the severity of cerebellar signs in MSA; however, it is clearly contaminated by parkinsonian features.     

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     

Cognitive deficits in multiple system atrophy correlate with frontal atrophy and disease duration

Background and purpose:  Dementia remains an exclusion criterion in diagnosing multiple system atrophy (MSA). This study aimed to determine the cognitive changes and brain atrophy patterns in the Parkinsonian (MSA-P) and cerebellar (MSA-C) variants of MSA.
Methods:  Voxel-based morphometry (VBM) of magnetic resonance imaging (MRI) and neuro-psychological tests were applied to 10 MSA-C and 13 MSA-P patients, and compared to 37 age-matched controls. Correlation analyses were performed between cognitive test results and morphometric data extracted from the VBM data.
Results:  In neuro-psychological testing, the 23 MSA patients scored lower in the Stroop interference test and took longer in the trail-making test as compared with the controls, whereas MSA-C performed worse than MSA-P in the memory scores, Stroop test, and time to complete the trail-making test. MSA, as a group, showed atrophy in the cerebellum, insular cortex, fusiform gyrus, inferior orbito-frontal gyrus, superior temporal gyrus, and caudate nucleus. Memory scores correlated well with pre-frontal lobe atrophy but not in the insular area.
Conclusion:  In conclusion, although dementia is not a typical presenting feature of MSA and is regarded as a sub-cortical movement disorder, frontal atrophy, cognitive changes, and dementia are identifiable as MSA progresses.     

Cerebral metabolic changes in early multiple system atrophy: a PET study

Previous positron emission tomography (PET) studies have shown widespread hypometabolism in the brain of advanced MSA but the time course of these metabolic abnormalities is largely unknown. In order to clarify the principal disease processes in multiple system atrophy (MSA) in the early stage, we investigated regional cerebral glucose metabolism (rCMGglc) and nigral dopaminergic function in nine patients with early stage of MSA using [(18)F]fluorodeoxyglucose (FDG) and 6-L-[(18)F]fluorodopa ((18)F-Dopa) positron emission tomography (PET) (two men and seven women; age, 59.3+/-5.4 years; disease duration, 29.7+/-14.6 months). The rCMRglc in the early MSA patients significantly decreased in the cerebellum, brainstem, and striatum compared with that in nine normal subjects. A significant correlation was found between the severity of autonomic dysfunction and rCMRglc within the brainstem. The severity of extrapyramidal signs also correlated with the decline of F-Dopa uptake but not that of rCMRglc within the striatum. The degree of atrophy on MRI has correlated with neither the clinical symptoms nor rCMRglc at the cerebellum and the brainstem. Our PET studies demonstrated widespread metabolic abnormalities except for the cerebral cortex in the brain of MSA even in the early stage. The hypometabolism in the brainstem was tightly linked to the autonomic dysfunction. Not the striatal dysfunction but the nigral damage may be responsible for the extrapyramidal symptoms in early MSA.     

Nerve conduction studies in multiple system atrophy

To study the frequency and severity of peripheral neuropathy in multiple system atrophy (MSA), we performed nerve conduction studies in 42 MSA patients suffering from either cerebellar MSA (MSA-C) or parkinsonian MSA (MSA-P). Abnormal nerve conduction was present in 24% of the patients. Abnormalities were significantly more frequent in MSA-P (43%) compared to MSA-C (14%). Motor nerve conduction velocities were reduced in 4% of the MSA-C and in 7% of the MSA-P patients. Abnormal compound muscle action potentials were more frequent in MSA-P (29% versus 7% in MSA-C) pointing to a more pronounced loss of motor axons in this subgroup. Sensory nerve conduction velocities were abnormal in 4% of the MSA-C and 14% of the MSA-P patients, and mean sensory nerve action potentials were normal in all MSA-C and reduced in 7% of the MSA-P patients. The data provide evidence that the peripheral nervous system is differentially affected in MSA-C and MSA-P.     

Dystonia in multiple system atrophy

OBJECTIVE: To delineate the frequency and nature of dystonia in multiple system atrophy (MSA). METHODS: A cohort of 24 patients with clinically probable MSA over the past 10 years were prospectively followed up. Motor features were either dominated by parkinsonism (MSA-P subtype, n=18) or cerebellar ataxia (MSA-C, n=6). Classification of dystonic features and their changes with time was based on clinical observation during 6-12 monthly follow up visits. Parkinsonian features and complications of drug therapy were assessed. Most patients (22/24) died during the observation period. Neuropathological examination was confirmatory in all of the five necropsied patients. RESULTS: At first neurological visit dystonia was present in 11 (46%) patients all of whom had been levodopa naive at this time point. Six patients (25%) exhibited cervical dystonia (antecollis) (MSA-P n=4, MSA-C n=2), five patients (21%) showed unilateral limb dystonia (MSA-P n=4; MSA-C n=1). A definite initial response to levodopa treatment was seen in 15/18 patients with MSA-P, but in none of the six patients with MSA-C. A subgroup of 12 patients with MSA-P developed levodopa induced dyskinesias 2.3 years (range 0.5-4) after initiation of levodopa therapy. Most patients had peak dose craniocervical dystonia; however, some patients experienced limb or generalised dystonia. Isolated peak dose limb chorea occurred in only one patient. CONCLUSION: The prospective clinical study suggests that dystonia is common in untreated MSA-P. This finding may reflect younger age at disease onset and putaminal pathology in MSA-P. Levodopa induced dyskinesias were almost exclusively dystonic affecting predominantly craniocervical musculature. Future studies are required to elucidate the underlying pathophysiology of dystonia in MSA.     

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.     

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.