局灶性脑皮层发育不良 MRI 表现与病理相关性分析

目的：研究局灶性脑皮层发育不良（FCD ）M RI表现与病理相关性。方法经手术病理证实FCD患者74例,根据 FCD的M RI表现,把FCD分为3种类型：放射带型、高信号型、轻微型。分析FCD的M RI分型与病理类型的相关性。结果74例患者中,放射带型12例,表现皮层和／或皮层下T2 WI／FLAIR上高信号,白质内三角形或带状异常信号从皮层向侧脑室方向延伸。高信号型32例,表现皮层和／或皮层下片状 T2 WI／FLAIR高信号,伴有皮层增厚,但是无放射带表现。轻微型30例,仅表现皮层T2 WI／FLAIR略高信号,伴有皮层增厚,但无皮层下高信号表现。放射带型FCD的病理类型多为ⅡB型,高信号型 FCD的病理多表现为ⅡA型及ⅡB型,轻微型FCD病理稍多见于ⅠA型和ⅠB型。结论了解FCD的M RI表现及分型有利于提高FCD的正确诊断,并有助于提示病理类型。     

Gelastic seizures in a child with focal cortical dysplasia of the cingulate gyrus

We present a case of gelastic seizures in a child with focal cortical dysplasia of the anterior cingulate gyrus. This is only the second published case of a confirmed lesion at this site presenting in such a way. The underlying neurological mechanism is described. Received: 23 October 1995 Accepted: 2 July 1996     

局灶性脑皮质发育不良病理分型的MRI特点

目的探讨不同病理分型的局灶性脑皮质发育不良（FCD）的MRI特点。方法回顾分析行癫痫手术治疗后病理符合FCD患者的临床资料，所有患者术前均行MR检查。根据Palmini的FCD分型标准，分型后分别总结患者的MRI特点。结果共28例患者符合标准，其中FCDⅠ型与Ⅱ型各14例。FCDⅠ型患者中，9例病灶位于颞叶，6例合并海马硬化。其中MRI表现异常者8例，表现为灰白质交界不清，局部皮层增厚、形态异常等特征；FCDⅡ型患者中，病灶位于额叶7例，2例合并海马硬化。其中MRI表现异常者11例，多表现为脑回形态异常，液体抑制反转恢复序列（FLAIR）、T2WI上白质信号增高，以及白质内条带状异常增高信号向脑室方向延伸等特点。结论不同病理分型的FCD在MRI上表现的特点有所不同，了解这些特点有助于提高癫痫外科术前评估的准确性以及对手术预后的判断。     

不同病理类型局灶性皮质发育不良的MRI特征

目的 总结局灶性皮质发育不良(FCD)的MRI特征性表现,探讨不同病理类型FCD各自的特异性MRI征象.方法 回顾性分析经病理证实的44例FCD患者的MRI表现.将44例患者根据病理检查结果分为FCD Ⅰ型和Ⅱ型,观察以下MRI征象在两种类型中的出现情况:(1)局灶性皮层增厚;(2)灰、白质分界不清;(3)液体衰减反转恢复(FLAIR)序列和(或)T2WI上白质内向脑室方向延伸的锥形异常增高信号;(4)脑叶发育不全;(5)FLAIR上灰质信号强度增高;(6)T2WI上灰质信号强度增高;(7)FLAIR上皮层下白质信号强度增高;(8)T2WI上皮层下白质信号强度增高;(9)T1WI上皮层下白质信号强度减低.2组间的比较采用χ2检验及校正χ2检验.结果 44例FCD患者中FCD Ⅰ型30例,FCDⅡ型14例.共32例患者MRI表现异常,灰、白质分界不清为最常见征象(23例).其中FCD Ⅰ型患者中MRI表现异常者21例,特征性表现为脑叶发育不全11例,FCDⅡ型脑叶发育不全0例,两种类型间差异有统计学意义(连续性校正χ2=5.0286,P=0.0249).FCDⅡ型患者中MRI表现异常者11例,特征性表现为局灶性皮层增厚10例,FCD Ⅰ型为11例(χ2=4.6234,P=0.0315);FLAIR上白质信号增高9例,FCD Ⅰ型为7例(χ2=6.9180,P=0.0085);白质内向脑室方向延伸的锥形异常增高信号4例,FCD Ⅰ型为0例(连续性校正χ2=6.2883,P=0.0122);差异均有统计学意义.其他征象两种类型间差异无统计学意义.结论 不同病理类型FCD的MRI表现各有特点,了解这些特点有助于不同病理类型FCD的早期诊断和术前评估.     

Rostral vermian cortical dysplasia: MRI

The MRI findings in rostral vermian dysplasia are described for the first time. Defective foliation and abnormal fissuration of the rostral vermis can clearly be depicted on coronal images. The abnormalities are limited to the anterior lobe of the vermis and its hemisphere extension. A hypothesis is put forward to explain the abnormalities. It is suggested that the vermian changes result from an intrauterine insult at the end of the first trimester. There appears to be a variable degree of expression and associated cerebellar and cerebral cortical abnormalities can be seen. The clinical significance of these findings remains incompletely understood but may be related to the severity of the abnormalities. It is also suggested that a mild degree of vermian rostral dysplasia may represent an incidental imaging finding. Received: 10 May 1998 Accepted: 23 June 1998     

MRI in craniofacial fibrous dysplasia

Summary Five patients with biopsy-proven craniofacial fibrous dysplasia underwent MRI with T1- and T2-weighted sequences and a gadolinium-enhanced T1-weighted spin-echo sequence. Low to intermediate signal intensity was usually seen in the largest part of the lesion on both spin-echo sequences, but smaller regions of hyperintensity on T1- and T2-weighted images and intermediate signal intensity throughout a lesion on T1-weighted images were also seen. All lesions enhanced but only two became iso- or hyperintense compared to fat. High clinical and pathological activity in three cases correlated with high signal intensity on both spin-echo sequences and with strong enhancement in two of the three. The presence of large veins or sinusoids on pathological examination did not correlate with the enhancement pattern.     

Reye's syndrome with cortical laminar necrosis: MRI

Serial MRI findings are described in two patients with Reye's syndrome, demonstrating diffuse cortical and white matter changes. In the acute stage, T2-weighted images showed subtle but definite laminar high signal and contrast-enhanced T1-weighted images laminar enhancement, along the entire cerebral cortexbilateraly. In the chronic stage, unenhanced T1-weighted images showed diffuse cortical laminar high signal. These characteristic MRI features seemed very similar to those of laminar cortical necrosis in hypoxic brain damage. MRI also displayed delayed white matter changes with cerebral atrophy.     

Imaging and radiological-pathological correlation in histologically proven cases of focal cortical dysplasia and other glial and neuronoglial malformative lesions in adults

Focal cortical dysplasia (FCD) is a pathological entity first described in 1971. Other more subtle cortical malformations found in patients with epilepsy include microdysgenesis (MD), and glioneuronal hamartias. Although these glial and neuronoglial malformations have distinct histological features, there is terminological confusion in the radiological literature. Few cases have been reported in adults with both imaging and histology. We address these issues, giving a radiological-pathological correlation of histologically proven cortical malformations in adults. We describe clinical, radiological and histological features of 12 cases (five FCD, five MD with glioneuronal hamartias, and two hamartomas), unassociated with other conditions, and discuss them in the light of the literature. FCD is usually seen on MRI as cortical thickening, with or without signal change, which may extend into the adjacent white matter. On histology, abnormal neurons and/or glial cells, blurring of the grey-white matter interface, myelin pallor, demyelination, and gliosis may be found. Glioneuronal hamartias and hamartomas usually appear as complex masses on MRI. FCD and hamartias may be associated, and a combination of imaging findings may be seen on MRI. Atrophy of the ipsilateral hippocampus may be present on MRI in patients with hamartias, and minor cell loss on histology, but not definitive hippocampal sclerosis. Although the imaging findings of cortical malformations are protean, some characteristic MRI features, with histological correlates, may be found. The relevance of most of these observations remains unclear. Received: 14 December 1998/Accepted: 2 July 1999     

Focal cortical dysplasia of the temporal lobe with late-onset partial epilepsy: serial quantitative MRI

We describe serial studies of focal cortical dysplasia causing temporal lobe seizures and progressive aphasia in a 54-year-old woman. Initially, MRI volumetry of the temporal lobes showed significant left cortical thickening corresponding to an elevated aminoacid uptake in the left temporoparietal and inferior frontal cortex on SPECT using 3-[¹²³I]iodo-α-methyl-l-tyrosine (IMT). After 1 year there was severe shrinkage of the left temporal lobe, possibly the result of recurrent complex partial seizures. Received: 16 July 1999/Accepted: 20 September 1999     

Malformations of cortical development: 3T magnetic resonance imaging features

Malformation of cortical development (MCD) is a term representing an inhomogeneous group of central nervous system abnormalities, referring particularly to embriyological aspect as a consequence of any of the three developmental stages, i.e., cell proliferation, cell migration and cortical organization. These include cotical dysgenesis, microcephaly, polymicrogyria, schizencephaly, lissencephaly, hemimegalencephaly, heterotopia and focal cortical dysplasia. Since magnetic resonance imaging is the modality of choice that best identifies the structural anomalies of the brain cortex, we aimed to provide a mini review of MCD by using 3T magnetic resonance scanner images.     

Proton magnetic resonance spectroscopy in disturbances of cortical development

Proton magnetic resonance spectroscopy(¹H-MRS) can be used for looking at cerebral metabolites in vivo. However, measurement of concentrations of cerebral metabolites in patients with disturbances of cerebral development have not been successful. Our purpose was to measure the concentrations of cerebral metabolites in such patients. We carried out quantitative ¹H-MRS in eight patients with cortical dysplasia, four with lissencephaly and three with heterotopic grey matter and six age-matched normal controls. Regions of interest for ¹H-MRS were set over the affected cortex in the patients and the occipital cortex in controls. The calculated concentration of N-acetylaspartate ([NAA]) was significantly lower in the affected cortex in patients with cortical dysplasia (P < 0.05), lissencephaly (P < 0.01), and heterotopia (P < 0.05) than in controls, idnicating a decreased number and/or immaturity or dysfunction of neurones in the affected cortex. The concentration of choline ([Cho]) was significantly lower in patients with lissencephaly (P < 0.01) than in controls, indicating glial proliferation and/or membrane abnormality. Received: 19 April 2000 Accepted: 30 May 2000     

致痫性局灶性脑皮质发育不良的影像特征及MR扫描方案优化

目的 通过致痫性局灶性脑皮质发育不良(FCD)的MR影像与病理对照研究,分析FCD的发病特点,探讨最佳MR扫描方案.方法 回顾性分析经手术病理证实的36例(40个病灶)FCD患者的MR表现及扫描方法.根据Palmini病理分型,将FCD的病理结果分为FCD Ⅰ型及FCDⅡ型.观察各型病灶在脑内分布及合并海马硬化的比例,采用Fisher精确概率法比较FCD各型在脑内分布的差异性.将轴面FSE T2WI、SE T1WI、液体衰减反转恢复(FLAIR)序列定义为常规扫描,在常规扫描的基础上增加斜冠状面FSE T2WI及FLAIR定义为优化扫描.患者均进行常规扫描和优化扫描,评价FCD主要征象在各扫描方位、序列的显示情况,应用McNemar检验比较2种扫描方法对不同部位FCD及海马硬化显示的差异性.结果 36例(40个病灶)FCD患者中,病变位于颞叶29个(72.5％),额叶9个(22.5％),顶叶2个(5.0％).FCD Ⅰ型27例(29个病灶),颞叶病灶25个;FCDⅡ型10例(11个病灶),颞叶病灶4个,2种类型在病灶分布上差异有统计学意义(P=0.002).合并海马硬化者共14例,其中,FCD Ⅰ型13例,FCDⅡ型1例.优化扫描对于颞叶FCD、海马硬化的显示优于常规扫描,颞叶FCD的阳性率从44.8％ (13/29)提高到65.5％ (19/29),合并海马硬化FCD的阳性率从42.9％( 6/14)提高到85.7％(12/14),差异有统计学意义(颞叶FCD x2=4.167,P=0.031;海马硬化x2=4.167,P=0.031),对额叶FCD病灶的显示2种扫描方式间差异无统计学意义(x2 =0.304,P=1.000).结论 FCD好发于颞叶,其次是额叶,FCDⅠ型好发于颞叶,且更容易合并海马硬化.垂直于海马长轴的全颞叶优化扫描是针对FCD发病特点的优化扫描序列组合,有助于病灶的显示.     

局限性脑皮层发育不良的影像与病理对比分析

目的 探讨局限性脑皮层发育不良(FCD)的影像改变,并与病理改变对比,以提高对FCD的认识.方法 回顾性分析28例经临床病理证实的FCD患者的临床及影像资料,所有患者均行MR检查,其中14例患者行PET检查,将影像改变与病理变化进行对比分析.结果 28例FCD中MRI显示病灶24例(85.7%),4例未显示明显异常,局限性脑皮层增厚及灰、白质界限不清是FCD的主要MR改变,同时脑皮层及皮层下白质可出现多种异常信号,其中皮层下白质内类三角形稍长T2信号指向侧脑室具有一定的特征性.14例行PET检查,其中9例(64.3%)显示病灶局部葡萄糖代谢活性减低.病理改变主要包括脑皮层细胞排列结构紊乱,皮层及皮层下白质内异形神经元细胞及气泡状细胞,部分患者可见皮层下白质髓鞘形成障碍及海绵样坏死灶.结论 高质量的MR图像可以显示大部分FCD病灶,包括脑皮层及皮层下白质的异常信号,是目前术前评价FCD的最佳影像检查.     

长骨纤维结构不良的CT及MRI诊断分析

目的探讨CT及MRI对长骨纤维结构不良的诊断及鉴别诊断价值。方法回顾性分析经病理证实的21例长骨纤维结构不良患者。所有患者均行CT平扫,MRI平扫及增强扫描19例。结果单骨型20例,其中股骨11例,胫骨4例,肱骨3例,尺骨及腓骨各1例;多骨型1例,同时累及同侧股骨及胫骨。CT表现呈磨玻璃样改变11例,囊状膨胀透亮改变8例,丝瓜络样改变2例。对照CT,呈磨玻璃样改变时MRI上T1WI呈等、稍低信号,T2WI呈不均匀等、稍高信号;呈囊状膨胀透亮改变时,T1WI呈低信号,T2WI呈明亮高信号;呈丝瓜络样改变时,T1WI呈不均匀稍低信号,T2WI呈不均匀稍高信号;增强后呈不同程度边缘或片状不均匀强化。结论 CT可显示长骨纤维结构不良的病变范围及细节等,MRI有助于反映病变组织成分,CT结合MRI在纤维结构不良的诊断及鉴别诊断中有重要价值。     

自闭症的常规MRI和1H-MRS表现

目的 探讨常规MR成像和1H MR波谱(MRS)在自闭症诊断中的价值.资料与方法 回顾性分析12例自闭症患者的全脑常规MR扫描及其中8例患者额叶和海马的多体素2D 1H MR波谱扫描表现.结果 常规MR扫描发现3例患者的顶叶白质在FLAIR上出现斑片状高信号.1H MRS分析发现5例患者额叶的NAA/Cho明显下降(NAA/Cho<1),其中1例患者的左侧额叶出现Lac峰;另外有2例患者海马的NAA/Cho出现下降(NAA/Cho<1).结论 常规MR成像和1H MRS可发现部分自闭症患者脑组织异常.     

垂体发育不良的影像学诊断现状

垂体发育不良是垂体功能低下的重要原因之一,影像学检查为诊断垂体发育不良的重要手段,本文综述垂体发育不良的影像学诊断及其在临床工作中的重要意义。     

肝脏局灶性结节增生的MRI分析

目的：探讨肝脏局灶性结节增生（FNH）的MRI表现特点。方法：11例FNH共12个结节行MRI平扫,其中10例10个结节行动态增强。11例均经手术或穿刺活检证实。对病灶的MRI一般和特殊表现进行分析并与病理对照。结果：12个结节中T1WI呈稍低、等信号,T2WI呈稍高、等信号9个;T1WI呈等信号,T2WI呈稍高信号2个;T1WI呈低信号,T2WI呈高信号1个。12个结节中信号不均匀4个,9个结节见疤痕,2个结节见包膜,3个结节周围见胆管受压移位。增强扫描动脉期、门静脉期及延迟期均强化9个,2个结节动脉期强化,延迟期大部分实质低于正常肝组织,2个结节边缘见包膜样强化;6个病灶见疤痕强化,1个结节疤痕无强化,3个结节平扫与增强未见疤痕。结论：绝大多数FNH的MRI表现具有一定的特征性,少部分结节表现不典型,经认真观察与分析,能作出正确诊断。     

MRI对发育性髋脱位软组织病变的诊断价值及新技术的应用趋势

发育性髋关节脱位(DDH)是小儿骨科常见的四肢异常之一,对其骨骼方面的研究已日趋成熟,近年来对关节内外软组织病变的研究逐渐成为热点。MRI因其良好的软组织分辨力,能较好地显示出髋关节内所有临床上重要而其他影像检查难以显示的软组织及软骨结构,已成为DDH诊断及术前评估、预后评价的必要检查方法。同时,随着近年来MR新技术的不断出现,其对提示软骨及肌肉的早期病变具有潜在价值。探讨MRI对DDH软组织病变的诊断价值及一些MR新技术的应用趋势。     

肝脏多发局灶性结节性增生的MRI诊断

目的 评估MR诊断肝脏多发局灶性结节性增生(focal nodular hyperplasia,FNH)的价值.方法 回顾性分析经病理证实的9例多发FNH的MR表现,并与部分病灶病理所见对照.结果 9例诊断均考虑到FNH的可能性,5例第一诊断考虑为FNH,3例第一诊断考虑为肝腺瘤,1例第一诊断考虑为纤维板层型肝癌.9例共31个病灶,T2WI 19个呈稍高信号、12个呈等信号;T1WI上12个呈稍低信号、7个呈等信号、12个呈高信号;反相位成像1个病灶局部信号略有减低.注射Gd-DTPA后,动脉期18个病灶轻度至明显不均匀强化、11个病灶显著均匀强化、1个病灶中度不均匀环形强化、1个病灶未见异常强化;门静脉期和延迟期31个病灶均逐渐呈等信号或稍高信号.共16个病灶出现中央瘢痕,瘢痕延迟期强化.结论 多发FNH有特征性表现,大部分病例MR能正确诊断.