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多发性硬化的常见MRI征象分析
冯逢

冯逢.多发性硬化的常见MRI征象分析.磁共振成像, 2011, 2(4): 309-313. DOI:10.3969/j.issn.1674-8034.2011.04.014.


[摘要] 本文介绍了多发性硬化的临床表现及其病理学改变,重点描述多发性硬化MRI表现的典型征象,包括急性期/亚急性期的"煎蛋征"、"开环征"、"弓形征"、"晕环征",胼胝体早期改变的"条纹征"、"点线征",沿小静脉走行的"Dawson手指征"以及脊髓受累的典型表现等,这些征象有助于多发性硬化的诊断、鉴别诊断以及疾病进展及治疗效果的评估。
[Abstract] This article introduced the clinical manifestations and the pathological changes in multiple sclerosis (MS). The typical MRI signs, including "fried egg sign", "open-ring sign", "arclike sign", and "halo sign" in acute/subacute stage, "subcallosal striation sign", "dot-dash sign" in early stage with callosal involvement, "Dawson's fingers" along the white matter venules, and typical findings in the spinal cord, were described and elucidated. These characteristic signs are important and helpful in the diagnosis and differential diagnosis of MS, and are valuable in the assessment of disease progression and evaluation of therapeutic effect.
[关键词] 多发性硬化;磁共振成像;影像学征象;诊断
[Keywords] Multiple sclerosis;Magnetic resonance imaging;Imaging signs;Diagnosis

冯逢* 中国医学科学院,北京协和医学院,北京协和医院放射科,北京 100730

通讯作者:冯逢,E-mail: cjr.fengfeng@vip.163.com


作者简介:
        冯逢(1966-),女,医学博士,主任医师,教授,博士生导师;研究方向:中枢神经系统影像诊断。

收稿日期:2011-06-04
接受日期:2011-07-04
中图分类号:R445.2; R744.51 
文献标识码:A
DOI: 10.3969/j.issn.1674-8034.2011.04.014
冯逢.多发性硬化的常见MRI征象分析.磁共振成像, 2011, 2(4): 309-313. DOI:10.3969/j.issn.1674-8034.2011.04.014.

       多发性硬化(multiple sclerosis, MS)是一种慢性、致残性、多灶性中枢神经系统炎性疾病,传统上被认为是白质的病变,近年来超高场磁共振成像(magnetic resonance imaging, MRI)清晰显示MS大脑灰质中也存在病灶[1]。MS主要的病理改变是血管周围炎症、髓鞘脱失、胶质增生以及疾病后期出现的轴索甚至神经细胞胞体的损伤。病理改变是影像学征象的基础,MRI,尤其是高场、超高场的磁共振成像设备以其优良的软组织分辨力为显示和分析MS病灶提供了有力的保障。MRI作为MS首选的影像学检查方法,在其诊断、鉴别诊断、疾病发展进程的评价,以及治疗效果的评价上均非常重要,成为临床MS诊治中必不可少的检查手段[2]。因此,了解和正确分析MS病灶的MRI征象及特点,是神经放射医师所必需掌握的。

1 临床表现

       MS的临床特征是病变在时间上和空间上的多发性,常表现为症状的发作与缓解交替出现的特点。其临床表现也多种多样,取决于病变侵犯中枢神经系统的部位。首发症状多为肢体无力、感觉障碍、视力障碍、走路不稳等,小脑、脊髓受累可以出现小脑和/或脊髓功能异常,包括小脑性共济失调、眼球运动障碍、构音障碍、截瘫、尿便功能障碍、括约肌功能障碍,还可能出现智能及精神的异常。

2 病理表现

       MS的病理学特征是在白质出现脱髓鞘斑块,斑块的形状、大小各异,从数毫米到数厘米不等。病理组织学上不同时期的MS斑块表现不同[3]:①急性期新生斑块:含大量吞噬类脂的巨噬细胞;病灶周围血浆蛋白外渗,出现水肿;无少突胶质细胞及星形细胞。②亚急性斑块:出现大量的增大的星形细胞;小血管周围出现淋巴细胞套;出现少突胶质细胞。③静止的慢性斑块:细胞较少,只有小型的星形细胞;轴索大致正常;小静脉增厚,血管周围间隙扩大;形成星形胶质纤维。④活动性慢性斑块:慢性斑块边缘出现新鲜破坏的髓鞘,可见吞噬类脂的巨噬细胞;斑块边缘出现少突胶质细胞。

3 MRI影像表现

       MS的病变斑块可以出现在中枢神经系统的任何部位,好发部位包括视神经、脑室周围、胼胝体、深部白质、中脑、小脑和脊髓[4]

       MRI上脑内各期病灶的影像表现也有所不同。①急性期及亚急性期的斑块多呈卵圆形或圆形,有膨胀感[5],呈长T1、长T2异常信号,信号多不均匀,斑块周边多可见等信号成分,周围可见因血浆蛋白渗出的水肿带,称为"煎蛋征"(fried egg sign) (图1)。MRI增强扫描是检测MS活动性病灶的敏感方法[6]。静脉注射含钆(Gadolinium, Gd)的对比剂后,病灶可呈结节状强化或环形强化,MS强化的斑块易见到不完全的环形强化,称为"开环征"(open-ring sign)(图2),或称为"弓形征"(arclike sign)[6]。近年来临床应用较普遍的扩散加权成像(diffusion weighted imaging, DWI)显示,急性期斑块的ADC值常升高,这主要是由于血管源性水肿所致;较少文献报道急性期时MS斑块的ADC值降低[7],急性期MS斑块周边在DWI上可出现环形高信号,其表观扩散系数(apparent diffusion coefficient, ADC)值相对减低,可称为"晕环征"(halo sign),考虑为急性期MS斑块病灶边缘聚集大量巨噬细胞,细胞外间隙缩小,水分子扩散受限(图3),但这种情况在MS的急性期持续的时间不长,会转变为ADC值升高的血管源性水肿,亚急性期的MS斑块ADC值较高。②慢性静止期的斑块病灶占位效应不明显,多为线条状,长轴多垂直于侧脑室,仍呈长T1、长T2异常信号,但DWI上无"晕环征",增强后斑块也无异常强化(图4)。③活动性慢性斑块,影像特点上是上述两种斑块的复杂组合。

       胼胝体是MS早期好发的部位之一,MS胼胝体改变的影像学表现也颇具特征。文献报道了在薄层(2 mm层厚)的矢状位T2 FLAIR序列上可以看到胼胝体下的"条纹征"(subcallosal striation sign)[8]和胼胝体下室管膜的"点线征"(dot-dash sign)[9],均可作为MS早期的征象。因此矢状位的T2 FLAIR成像也成为评价MS不可缺少的序列。"条纹征"是指胼胝体下出现垂直于室管膜的条纹状高信号(图5)。"点线征"的定义是胼胝体下的室管膜出现不规则的圆点与细线相连的高信号(图6)。这两个征象是MS较敏感的早期征象,可见于脑内未出现经典MS斑块的患者,因此要认识这些重要的征象。需要提出的是胼胝体下室管膜的类似改变还可见于其他的一些情况,如小血管缺血性病变在胼胝体上的表现,灰质桥等,需要结合患者年龄及病史,仔细观察加以鉴别。

       Dawson手指征(Dawson's fingers)是MS常见的影像学表现之一,是指MS斑块围绕脑室周围的髓静脉分布的特点,这与其病理上表现的围绕小静脉的炎细胞浸润吻合,呈垂直于侧脑室发散分布的表现,类似于手掌五指张开的表现(图7)。这种征象在矢状位、轴位、冠状位均可以观察到。

       MS的斑块还可以发生在脊髓,以颈段脊髓多见。约有31.1%的MS斑块单独发生在脊髓而不伴有脑内病灶[10]。脊髓内MS斑块的影像特点是:斑块可呈卵圆形、斑点状,或为纵向延伸的病灶,其长轴与脊髓长轴一致,长度多小于2个椎体节段,横轴位显示病灶多位于脊髓的周边,如脊髓的后索、侧索、软膜下区等处,斑块大小常小于脊髓横截面积的1/2(图8)。

图1  女,36岁,双下肢麻木7月,无力4月。1A:侧脑室体水平轴位FSE T2WI,侧脑室旁深部白质、左侧额叶皮层下可见多发类圆形长T2异常信号,其中左侧额叶皮层下可见卵圆形病灶,周围可见水肿征象(箭头),称为"煎蛋征";1B:同水平层面对比剂增强后的SE T1WI,可见多数病灶周边可见轻至中度的异常强化,呈环形(白箭头),或者开环样强化(长箭头),提示为急性或亚急性MS病灶;1C:同水平层面的DWI,显示急性期斑块周边的环形高信号(箭头);1D:同水平层面的ADC图,显示DWI上的高信号环,ADC略低,提示扩散受限,称为"晕环征"(箭头)
Fig 1  A 36-year-old female with numbness of lower extremities for 7 months and weakness for 4 months. Fig 1A: Axial FSE T2WI, multiple ovoid hyperintense lesions are showed at periventricular and left frontal white matter. The hyperintense lesion in left frontal lobe and the surrounding edema (arrow) compose the "Fried Egg" appearance. Fig 1B: Postcontrast SE T1WI of the same slice reveals mild to moderate ring-like (short arrow) or open ring-like (long arrow) enhancement of most lesions, indicating acute or subacute lesions. Fig 1C: DWI of the same slice shows the ring-like hyperintensity (arrow) surrounding the acute plaque. Fig 1D: ADC map of the same slice shows the "Halo sign" (arrow), i.e., low ADC value of the hyperintense ring on DWI, indicating restricted diffusion.
图2  男,36岁,左侧面部及肢体麻木7月余,加重4天。2A、2B:轴位不同层面上Gd对比剂增强后T1WI,显示MS斑块呈不完全的环形异常强化,称为"开环征"(箭头),或"弓形征"(长箭头),提示为急性或亚急性斑块。
图3  女,19岁,视力下降3个月,肢体力弱20天。3A:轴位半卵圆中心层面DWI,显示左侧半卵圆中心脱髓鞘病灶呈类圆形DWI高信号,周边信号更高,呈环形(箭头);3B:同层面的ADC图,显示DWI高信号环的ADC值降低,表现为"晕环征"(箭头)。
图4  女,19岁,双下肢无力5年,视物不清、二便不利1年余,4A:侧脑室体水平轴位FSE T2WI,显示典型的静止期病灶,长轴垂直于侧脑室(箭头),为横轴位所示的"Dawson's Fingers"征;4B:半卵圆中心水平轴位FSE T2WI,静止期病灶呈小条形(箭头);4C、4D:相同层面对比剂增强后的SE T1WI,显示上述斑块呈稍长T1信号,增强后未见明显异常强化(箭头)
Fig 2  A 36-year-old male with numbness on the left face and extremities for 7 months with worsening symptoms for 4 days. Fig 2A and 2B showing post Gadolinium-enhanced T1WI, the MS plaques exhibite the unclosed ring-like enhancement, which is also known as "open-ring" (short arrow) or "arclike" (long arrow) enhancement, indicating acute or subacute lesions.
Fig 3  A 19-year-old female with vision reduction for 3 months and weakness of extremities for 20 days. Fig 3A: DWI, an ovoid hyperintense lesion surrounded by a brighter ring (arrow) is found in the left centrum semiovale. Fig 3B: ADC map of the same section, the low ADC value is shown, corresponding to the brighter ring on DWI, and indicates the "halo sign" (arrow).
Fig 4  A 19-year-old female with weakness of lower extremities for 5 years, having blurred vision and difficulties in urination and defecation over 1 year. Fig 4A: Axial FSE T2WI shows a typical remitting lesion, whose long axis is perpendicular to right ventricle, suggesting the "Dawson's fingers" sign. Fig 4B: Axial FSE T2WI shows a bar-like remitting lesion (arrow) in left centrum semiovale. Fig 4C and 4D: Postcontrast SE T1WI of the same sections as 4A and 4B revealing hypointense plaques without marked enhancement (arrows).
图5  女,52岁,复发缓解型MS,病史3年,矢状位正中层面T2 FLAIR,胼胝体下缘可见多发走行大致垂直于室管膜的细线样高信号,符合"条纹征"的表现(长箭头)。
图6  女,26岁,双眼视力减退及双下肢麻木1年,矢状位正中层面T2 FLAIR,显示胼胝体下室管膜表面出现圆点呈串珠样排列,为"点线征"(箭头)
Fig 5  A 52-year-old female with relapsing-remitting multiple sclerosis for 3 years, whose mid-sagittal T2 FLAIR image indicates multiple hyperintense lines perpendicular to ependyma at the bottom of corpus callosum, suggesting the "subcallosal striation" sign (long arrows).
Fig 6  A 26-year-old female with reduced bilateral vision and numbness of the lower extremities for 1 year, whose mid-sagittal T2 FLAIR image demonstrates a string of beads on the surface of ependyma at the bottom of corpus callosum, suggesting the "dot-dash sign" (arrows).
图7  女性,38岁,反复发作的肢体麻木,无力,视力减退4年。7A:轴位侧脑室体部层面FSE T2WI,显示MS病灶沿着侧脑室旁小静脉分布,垂直于侧脑室;7B:轴位与图7A同层面T1 Flair像,MS病灶呈等至长T1信号;7C:矢状位旁正中层面T1WI,显示MS病灶垂直于侧脑室,呈"Dawson's手指征"(箭头)。
图8  女,27岁,头晕、走路不稳1年半。8A、8B:颈段脊髓矢状位FSE T2WI,显示C2~3、C4~5脊髓内多发卵圆形长T2异常信号影,长轴平行于脊髓,小于2个椎体节段(箭头);8C:轴位FSE T2WI,显示MS斑块位于脊髓的左侧白质分布的区域,病灶截面积小于1 /2脊髓横断面面积(箭头);8D:患者脑部侧脑室体水平轴位FSE T2WI,可见双侧侧脑室旁白质多发MS斑块影
Fig 7  A 38-year-old female with sensory deficit of lower extremities,weakness and vision reduction for 4 years. Fig 7A: Axial FSE T2WI shows MS lesions perpendicular to ventricles distributing along the periventricular venule. Fig 7B: T1 Flair of the same slice shows isointense or hypointense plaques. Fig 7C: T1WI of parasagittal plane shows MS lesions perpendicular to ventricles, demonstrating the "Dawson's Fingers" sign.
Fig 8  A 27-year-old female with dizziness and unstable walking for one and a half years. Fig 8A, 8B: Sagittal FSE T2WI of cervical spinal cord show multiple hyperintense ovoid lesions at the level of C2-3 and C4-5, whose long axises are parallel to spinal cord and shorter than 2 vertebras (arrows). Fig 8C: Axial FSE T2WI shows a MS plaque located in the left white matter eccentrically (arrow). Fig 8D: Axial FSE T2WI shows multiple periventricular MS plaques.

4 MRI新技术在MS中的应用及展望

       近年来,一些新的MRI成像技术也应用于MS的研究与临床诊治过程中。其中,以扩散张量成像(diffusion tensor imaging, DTI)、磁共振波谱(magnetic resonance spectroscopy, MRS)、磁敏感性加权成像(susceptibility weighted imaging, SWI),以及血氧水平依赖性脑功能成像(blood-oxygen-level dependent functional MRI, BOLD-fMRI)等研究居多。DTI可以定量评价白质束结构完整性破坏的程度,并可通过数据的后处理,显示白质束的形态、走行等情况,但是其技术以及数据后处理模型上还存在着需要解决的问题;MRS可以反映炎性脱髓鞘病变的生化改变,如急性脱髓鞘斑块可表现为N-乙酰天门冬氨酸(NAA)的下降,胆碱(choline, Cho)的升高,肌醇(myoinositol, mI)在急性期可降低,在慢性期可升高,此外,可出现乳酸峰(lactate, Lac)等代谢物的变化,能够更好地反映MS的病理生理改变,评价病变的进展情况,如出现Lac提示病灶存在无氧酵解,考虑为急性或亚急性的斑块,但是活体MRS技术受到信噪比的限制,对于较小的斑块则其敏感性下降;SWI可以更好地反映MS炎性斑块与小静脉的密切关系,但磁敏感性为场强依赖的特性,需要高场及超高场的MRI设备才能很好地实现这项技术,通常在3 T以上场强的MRI设备上这项技术有较好的应用;BOLD-fMRI是近年来研究的热点,对于MS的运动功能减低的中枢机制的评价,此外,超高场MRI成像系统(如7 T的人体MRI系统),能够提供更高的信噪比,可以清晰显示发生在皮层的MS病灶,扩展了我们对MS疾病过程的认识,有助于深入与MS相关的癫痫、认知功能障碍等方面的研究。

       总之,MRI在MS的诊断、疾病发展进程的了解和治疗效果的监测上均起着举足轻重的作用,应该很好地学习和掌握,不断提高。

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