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Cross Sign (十字征)、Hummingbird Sign (蜂鸟征) （特别鸣谢丁香园liuy88战友提供） 以下两图分别有一个征像。请大家指出，并给出什么样的疾病可出现？ 图1
图2
liuy88 还是给点暗示吧：
leleworm 图1：Cross Sign(十字征)——MRI的T2加权像上脑桥的十字形异常高信号影。由Savoiardo于1990年首次报道，见于橄榄体脑桥小脑萎缩(OPCA)的患者。
liuy88 图1:hot cross bun sign（十字征）。 病理基础:脑桥核及其发出的通过小脑中脚到达小脑的纤维变性，而由齿状核发出构成小脑上脚的纤维和锥体束未受到损害。桥横纤维和小脑中脚的变性和神经胶质增生使其水量增加，形成MRI的T2加权像脑桥的十字形高信号。Pontine neurons and myelinated transverse pontocerebellar fibres were severely depleted, resulting in pallor and considerable atrophy of the basis pontis and middle cerebellar peduncles. The fibres of the corticospinal tract, which run craniocaudally in the dorsal pons, the pontine tegmentum, and the superior cerebellar peduncles ventrally, were all preserved, this differential involvement therefore being responsible for the cruciform appearance on MRI.（译：脑桥神经元和横桥纤维严重减少，造成脑桥基底部和小脑中脚的苍白和萎缩。上下走行于脑桥背侧的皮质脊髓束、脑桥背盖部、腹前侧的小脑上脚/结合臂均未受累。这种差异性受累是MRI上出现十字征的原因。） 见于：Multiple system atrophy。它分三个亚型：1.striatonigral degeneration；2.olivopontocerebellar atrophy；3.Shy-Drager syndrome。 Multiple system atrophy is a rare neurological disorder characterized by a combination of parkinsonism, cerebellar and pyramidal signs, and autonomic dysfunction. The term "Multiple System Atrophy" is synonymous with striatonigral degeneration (SND) when Parkinsonism predominates, olivopontocerebellar atrophy (OPCA) when cerebellar signs predominate, and Shy-Drager syndrome when autonomic failure is dominant. The incidence (new case per 100,000 person years) for ages 50 to 99 years is 3.0 (Bower et al, 1997), or about half as frequent as it's close relative, progressive supranuclear palsy (PSP). The mean age of onset is 54. （译：见于：多系统萎缩（MSA）。MSA分三个亚型：1.纹状体黑质变性（SND）；2.橄榄体脑桥小脑萎缩（OPCA）；3.Shy-Drager综合征。多系统萎缩是一种少见的神经系统疾病，以帕金森症状、小脑及锥体束征，以及自主神经功能紊乱为特点。当以帕金森综合征为主时，MSA即为纹状体黑质变性（SND）；当以小脑症状为主时，MSA即为OPCA；而以自主神经功能紊乱为主时，MSA即为Shy-Drager综合征。MSA在50到99岁间的发病率为每年约3/10万，大约是与其症状相似的进行性核上性麻痹（PSP）发病率的一半。平均发病年龄为54岁。） 图2：hummingbird sign（蜂鸟征）。 病理基础: atrophy of the midbrain tegmentum（中脑被盖部萎缩）。 见于：progressive supranuclear palsy (PSP)（进行性核上性麻痹）。 Progressive supranuclear palsy (PSP) is a degenerative neurological disorder of uncertain etiology characterized by gait ataxia, slowing or inability to generate voluntary saccadic eye movements, and axial rigidity. The most characteristic aspect of PSP is an inability to move the eyes, but the first symptom of PSP is usually unsteadiness and falling. （译：进行性核上性麻痹(PSP)是病因不明的神经系统退行性病变，临床表现为共济失调步态、自主眼球扫视运动无法完成或完成缓慢、躯干强直。最具特征性的表现为眼球运动障碍，但首发症状常为姿势不稳和跌倒。） Rostral midbrain atrophy in progressive supranuclear palsy (PSP) is detected by mid-sagittal plain magnetic resonance imaging (MRI). The shape of the atrophy looks like the bill of a hummingbird (hummingbird sign). （译：进行性核上性麻痹(PSP)MRI正中矢状位扫描可见中脑嘴萎缩，外形看起来似乎是蜂鸟的鸟嘴(hummingbird sign，蜂鸟征)。） Progressive supranuclear palsy (PSP) is a neurodegenerative disorder characterized by supranuclear ophthalmoplegia, which primarily affects vertical gaze. It is accompanied by the following symptoms: pseudobulbar palsy, dysarthria, dystonic rigidity of the neck and upper trunk, and dementia. On magnetic resonance imaging (MRI), PSP is characterized by atrophy of the midbrain tegmentum (MT). The atrophy of the rostral MT, when detected by mid-sagittal MRI, looks like the bill of hummingbird and is therefore referred to as the “hummingbird” sign. The distinct shape of the hummingbird sign on MRI is reminiscent of the hummingbird's long, thin, sharp beak. The mid-sagittal MRI in PSP patients includes the regions of the most rostral midbrain, the midbrain tegmentum, the pontine base, and the cerebellum, and these images on MR appear to correspond to the bill, crown, body, and wing, respectively, of a hummingbird. The location of this sign is relevant due to the fact that it correlates with the vertical gaze center. （译：进行性核上性麻痹(PSP)是以核上性眼肌麻痹尤其是垂直凝视麻痹为特征的神经变性疾病。常伴随以下症状：假性球麻痹、构音障碍、颈肌和上部躯干强直、痴呆。在MRI上PSP表现为中脑被盖部萎缩；在正中矢状位上，中脑被盖部嘴缘的萎缩看起来像是蜂鸟的鸟嘴，因此称为“蜂鸟征”(hummingbird sign)。在影像上表现为蜂鸟细长、尖锐的鸟嘴的特征性形态。PSP患者的中脑嘴、中脑被盖部、脑桥基底部、小脑再MRI正中矢状位上看起来分别与蜂鸟的鸟嘴、鸟颈、鸟身、鸟翼相似。以上症状的定位与垂直凝视中枢受累有关。） 鉴别诊断： MSA、PSP和Corticobasal Ganglionic Degeneration (CBD)（皮质基底节变性）是临床上根据体征症状难于鉴别的三种少见的疾病。它们的共同特点是有帕金森症状并包括其它的体征。 Corticobasal ganglionic degeneration (CBD) is a rare progressive neurological disorder characterized by a combination of Parkinsonism and cortical dysfunction. It is a rare sporadic progressive disorder first reported in 1968. CBD appears to be closely related to another, less rare, sporadic extrapyramidal degenerative disorder named Progresive Supranuclear Palsy (PSP). In CBD, cognitive symptoms dominate, while in PSP, eye movement symptoms dominate the picture. （译：皮质基底节变性（CBD）是一种罕见的散发性进行性神经系统疾病，以帕金森症状和皮质功能障碍为特征。于1968年首次报道。CBD的发生是与另一个稍多见的散发的锥体外系变性疾病——进行性核上性麻痹（PSP）密切相关联的。CBD以认知障碍为主，但PSP则以眼球运动障碍为主。） 萎缩部位鉴别要点见下列图表：
MSA
PSP
CBD
正常人
PSP
MSA
boffoshao 进行性核上性麻痹（Progressive supranuclear palsy, PSP）：属于帕金森叠加综合征。1964年由Steele等首先描述。病理以分布于脑干和基底节的大量神经纤维缠节和神经纤维网线（neuropil threads）为特征。55到70岁发病；常常以姿势平衡障碍和跌倒为首发症状，随后出现构音障碍和运动迟缓；往往是双侧同时发病；半数以上的病人很快出现认知障碍；可出现呆视、眼睑关闭迟缓和不眨眼以及特征性的核上性共视运动障碍，表现为眼球共同上视或下视麻痹，后者更具有诊断价值；肌强直以中轴躯干性肌强直为主，肢体的肌强直不如躯干明显；病人可有个性的改变，包括情感淡漠和抑郁；病人的面部表情呈现“惊恐面容”。病人症状对左旋多巴制剂治疗无效或疗效差，其平均病程为5年到7年。早期出现姿势平衡障碍、跌倒、垂直性核上性共视障碍有助与帕金森病鉴别。 MRI见到中脑、三脑室周围萎缩、四叠体变薄是PSP影像学特征。卢文甫报道2例认为中脑萎缩和脑干被盖、顶盖部T2加权像弥散性高信号是PSP的特点。PSP喙状萎缩的中脑在MRI平扫正中矢状位上形如蜂鸟嘴——蜂鸟征。 foxet 最近的一篇关于鉴别MSA和PD（Parkinson disease）的文献。我概括一下： 因为MSA跟PD在临床上有很相似的表现，但有效的鉴别方法并不多。左旋多巴的诊断性治疗有一定的诊断价值，PD往往对左旋多巴的反应比较好，而MSA疗效不显著；但是这并不绝对，有的MSA病人早期可以有很好的疗效，有的病人从发病到去世都没能确诊。 在影像上，MSA可以出现上述所讲的“十字征象等”，对鉴别PD与MSA有绝对的特异性；但没有十字征，不能绝对排除MSA。MSA和PD都可以累及黑质纹状体，所以他们的临床表现可以相似。但MSA更主要累及OPC（橄榄-桥脑－小脑）系统，所以MSA更常出现小脑共济失调等的表现，在影像上就会出现小脑中脚的萎缩。 这篇外文做了一定数量的研究，发现：用小脑中脚的径线去鉴别PD与MSA（8mm为cuffoff值）有100%的敏感性和特异性。 To prospectively assess if middle cerebellar peduncle (MCP) atrophy, evaluated at magnetic resonance (MR) imaging, can help differentiate multiple system atrophy (MSA) from Parkinson disease (PD). Materials and Methods: All participants provided informed consent for participation in the study, which was approved by the institutional review board. Sixteen consecutive patients with MSA, 26 consecutive patients with PD, and 14 healthy control subjects were examined with MR imaging. Images were interpreted independently by two experienced neuroradiologists blinded to clinical information, who visually inspected the images for the presence or absence of putaminal atrophy, putaminal hypointensity, slitlike hyperintensity in the posterolateral margin of the putamen, brainstem atrophy, hyperintensity of the MCP, and cruciform hyperintensity of the pons. Measurements of MCP width on T1-weighted volumetric spoiled gradient-echo images were performed in all subjects. Differences in MCP width among the groups were evaluated by using the Kruskall-Wallis test, followed by the Mann-Whitney U test for multiple comparisons and Bonferroni correction. All patients (mean age, 63.88 years；range, 55–72 years)with MSA had at least one of the features commonly observed in this disease on MR images, whereas control subjects (mean age, 66.93 years; range, 61–77 years) and all but one patient with PD (mean age, 65.31 years；range, 51–79 years) had normal MR images. The average MCP width was significantly smaller in patients with MSA (6.10 mm \u0001 1.18 [standard deviation]) than in those with PD (9.32mm \u0001 0.77, P \u0002 .001) or control subjects (9.80mm \u0001 0.66, P \u0002 .001). Conclusion: Measurement of MCP width on MR images may be useful for distinguishing patients with MSA from those with PD. 小脑中脚(MCP)测量跟其它征象敏感性和特异性的比较
MCP是在旁中央矢状位，测量小脑中脚的上缘到下缘的距离。
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（PS：此文已检索出，是2006年发表于《Radiology》上的一篇名为《MR Imaging of Middle Cerebellar Peduncle Width: Differentiation of Multiple System Atrophy from Parkinson Disease》的文献。原文网址为：http://radiology./cgi/content/full/239/3/825。为方便总结归纳，后续将单独将此文整理后发表于博客中。） liuy88 正研究的课题《mean FA map from 13 MCI（轻度认知损害） and 10 AD patients and 16 controls》图像，有助于理解十字征的形成机制，发送大家分享：
鳄鱼的眼泪 我也弄个OPCA但没有十字征。但第3幅图符合楼主总结的“瘦子”征。呵呵，自己胡乱叫的哈！
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