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Cross Sign (十字征)、Hummingbird Sign (蜂鳥征)

 lion2008 2013-04-19

Cross Sign (十字征)、Hummingbird Sign (蜂鳥征)

(特別鳴謝丁香園liuy88戰(zhàn)友提供)

以下兩圖分別有一個(gè)征像。請(qǐng)大家指出,并給出什么樣的疾病可出現(xiàn)?

圖1

圖2

 liuy88

還是給點(diǎn)暗示吧:

leleworm

圖1:Cross Sign(十字征)——MRI的T2加權(quán)像上腦橋的十字形異常高信號(hào)影。由Savoiardo于1990年首次報(bào)道,見于橄欖體腦橋小腦萎縮(OPCA)的患者。

liuy88

圖1:hot cross bun sign(十字征)。

病理基礎(chǔ):腦橋核及其發(fā)出的通過小腦中腳到達(dá)小腦的纖維變性,而由齒狀核發(fā)出構(gòu)成小腦上腳的纖維和錐體束未受到損害。橋橫纖維和小腦中腳的變性和神經(jīng)膠質(zhì)增生使其水量增加,形成MRI的T2加權(quán)像腦橋的十字形高信號(hào)。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.(譯:腦橋神經(jīng)元和橫橋纖維嚴(yán)重減少,造成腦橋基底部和小腦中腳的蒼白和萎縮。上下走行于腦橋背側(cè)的皮質(zhì)脊髓束、腦橋背蓋部、腹前側(cè)的小腦上腳/結(jié)合臂均未受累。這種差異性受累是MRI上出現(xiàn)十字征的原因。)

見于:Multiple system atrophy。它分三個(gè)亞型: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.

(譯:見于:多系統(tǒng)萎縮(MSA)。MSA分三個(gè)亞型:1.紋狀體黑質(zhì)變性(SND);2.橄欖體腦橋小腦萎縮(OPCA);3.Shy-Drager綜合征。多系統(tǒng)萎縮是一種少見的神經(jīng)系統(tǒng)疾病,以帕金森癥狀、小腦及錐體束征,以及自主神經(jīng)功能紊亂為特點(diǎn)。當(dāng)以帕金森綜合征為主時(shí),MSA即為紋狀體黑質(zhì)變性(SND);當(dāng)以小腦癥狀為主時(shí),MSA即為OPCA;而以自主神經(jīng)功能紊亂為主時(shí),MSA即為Shy-Drager綜合征。MSA在50到99歲間的發(fā)病率為每年約3/10萬,大約是與其癥狀相似的進(jìn)行性核上性麻痹(PSP)發(fā)病率的一半。平均發(fā)病年齡為54歲。)

圖2:hummingbird sign(蜂鳥征)。

病理基礎(chǔ): atrophy of the midbrain tegmentum(中腦被蓋部萎縮)。

見于:progressive supranuclear palsy (PSP)(進(jìn)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.

(譯:進(jìn)行性核上性麻痹(PSP)是病因不明的神經(jīng)系統(tǒng)退行性病變,臨床表現(xiàn)為共濟(jì)失調(diào)步態(tài)、自主眼球掃視運(yùn)動(dòng)無法完成或完成緩慢、軀干強(qiáng)直。最具特征性的表現(xiàn)為眼球運(yùn)動(dòng)障礙,但首發(fā)癥狀常為姿勢(shì)不穩(wěn)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).

(譯:進(jìn)行性核上性麻痹(PSP)MRI正中矢狀位掃描可見中腦嘴萎縮,外形看起來似乎是蜂鳥的鳥嘴(hummingbird sign,蜂鳥征)。)

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.

(譯:進(jìn)行性核上性麻痹(PSP)是以核上性眼肌麻痹尤其是垂直凝視麻痹為特征的神經(jīng)變性疾病。常伴隨以下癥狀:假性球麻痹、構(gòu)音障礙、頸肌和上部軀干強(qiáng)直、癡呆。在MRI上PSP表現(xiàn)為中腦被蓋部萎縮;在正中矢狀位上,中腦被蓋部嘴緣的萎縮看起來像是蜂鳥的鳥嘴,因此稱為“蜂鳥征”(hummingbird sign)。在影像上表現(xiàn)為蜂鳥細(xì)長、尖銳的鳥嘴的特征性形態(tài)。PSP患者的中腦嘴、中腦被蓋部、腦橋基底部、小腦再M(fèi)RI正中矢狀位上看起來分別與蜂鳥的鳥嘴、鳥頸、鳥身、鳥翼相似。以上癥狀的定位與垂直凝視中樞受累有關(guān)。)

鑒別診斷:

MSA、PSP和Corticobasal Ganglionic Degeneration (CBD)(皮質(zhì)基底節(jié)變性)是臨床上根據(jù)體征癥狀難于鑒別的三種少見的疾病。它們的共同特點(diǎn)是有帕金森癥狀并包括其它的體征。

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.

(譯:皮質(zhì)基底節(jié)變性(CBD)是一種罕見的散發(fā)性進(jìn)行性神經(jīng)系統(tǒng)疾病,以帕金森癥狀和皮質(zhì)功能障礙為特征。于1968年首次報(bào)道。CBD的發(fā)生是與另一個(gè)稍多見的散發(fā)的錐體外系變性疾病——進(jìn)行性核上性麻痹(PSP)密切相關(guān)聯(lián)的。CBD以認(rèn)知障礙為主,但PSP則以眼球運(yùn)動(dòng)障礙為主。)

萎縮部位鑒別要點(diǎn)見下列圖表:

MSA

PSP

CBD

正常人

PSP

MSA

 boffoshao

進(jìn)行性核上性麻痹(Progressive supranuclear palsy, PSP):屬于帕金森疊加綜合征。1964年由Steele等首先描述。病理以分布于腦干和基底節(jié)的大量神經(jīng)纖維纏節(jié)和神經(jīng)纖維網(wǎng)線(neuropil threads)為特征。55到70歲發(fā)?。怀3R宰藙?shì)平衡障礙和跌倒為首發(fā)癥狀,隨后出現(xiàn)構(gòu)音障礙和運(yùn)動(dòng)遲緩;往往是雙側(cè)同時(shí)發(fā)?。话霐?shù)以上的病人很快出現(xiàn)認(rèn)知障礙;可出現(xiàn)呆視、眼瞼關(guān)閉遲緩和不眨眼以及特征性的核上性共視運(yùn)動(dòng)障礙,表現(xiàn)為眼球共同上視或下視麻痹,后者更具有診斷價(jià)值;肌強(qiáng)直以中軸軀干性肌強(qiáng)直為主,肢體的肌強(qiáng)直不如軀干明顯;病人可有個(gè)性的改變,包括情感淡漠和抑郁;病人的面部表情呈現(xiàn)“驚恐面容”。病人癥狀對(duì)左旋多巴制劑治療無效或療效差,其平均病程為5年到7年。早期出現(xiàn)姿勢(shì)平衡障礙、跌倒、垂直性核上性共視障礙有助與帕金森病鑒別。

MRI見到中腦、三腦室周圍萎縮、四疊體變薄是PSP影像學(xué)特征。盧文甫報(bào)道2例認(rèn)為中腦萎縮和腦干被蓋、頂蓋部T2加權(quán)像彌散性高信號(hào)是PSP的特點(diǎn)。PSP喙?fàn)钗s的中腦在MRI平掃正中矢狀位上形如蜂鳥嘴——蜂鳥征。

foxet

最近的一篇關(guān)于鑒別MSA和PD(Parkinson disease)的文獻(xiàn)。我概括一下:

因?yàn)镸SA跟PD在臨床上有很相似的表現(xiàn),但有效的鑒別方法并不多。左旋多巴的診斷性治療有一定的診斷價(jià)值,PD往往對(duì)左旋多巴的反應(yīng)比較好,而MSA療效不顯著;但是這并不絕對(duì),有的MSA病人早期可以有很好的療效,有的病人從發(fā)病到去世都沒能確診。

在影像上,MSA可以出現(xiàn)上述所講的“十字征象等”,對(duì)鑒別PD與MSA有絕對(duì)的特異性;但沒有十字征,不能絕對(duì)排除MSA。MSA和PD都可以累及黑質(zhì)紋狀體,所以他們的臨床表現(xiàn)可以相似。但MSA更主要累及OPC(橄欖-橋腦-小腦)系統(tǒng),所以MSA更常出現(xiàn)小腦共濟(jì)失調(diào)等的表現(xiàn),在影像上就會(huì)出現(xiàn)小腦中腳的萎縮。

這篇外文做了一定數(shù)量的研究,發(fā)現(xiàn):用小腦中腳的徑線去鑒別PD與MSA(8mm為cuffoff值)有100%的敏感性和特異性。

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.

Results:

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.

小腦中腳(MCP)測(cè)量跟其它征象敏感性和特異性的比較

 MCP是在旁中央矢狀位,測(cè)量小腦中腳的上緣到下緣的距離。
MCP>8的是PD;MCP<8的是MSA;之間沒有重疊。
對(duì)照組MCP均比PD和MSA高。

A

B

C

D

(PS:此文已檢索出,是2006年發(fā)表于《Radiology》上的一篇名為《MR Imaging of Middle Cerebellar Peduncle Width: Differentiation of Multiple System Atrophy from Parkinson Disease》的文獻(xiàn)。原文網(wǎng)址為:http://radiology./cgi/content/full/239/3/825。為方便總結(jié)歸納,后續(xù)將單獨(dú)將此文整理后發(fā)表于博客中。)

liuy88

正研究的課題《mean FA map from 13 MCI(輕度認(rèn)知損害) and 10 AD patients and 16 controls》圖像,有助于理解十字征的形成機(jī)制,發(fā)送大家分享:

鱷魚的眼淚

我也弄個(gè)OPCA但沒有十字征。但第3幅圖符合樓主總結(jié)的“瘦子”征。呵呵,自己胡亂叫的哈!

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