Developmental aspects of type II lissencephaly

We report neuropathological studies of five cases of type II lissencephaly from three fetuses and two infants. This comparative study allowed us to determine the developmental course of the cerebral lesions. Two distinct developmental events seem to generate this type of brain malformation: firstly, an early disturbance in cortex formation, which results both from a disorder of radial migration and a pial barrier disruption; secondly, a late perturbation of cerebral surface organization, resulting in fusion of the cerebral surface. All these features can be related to a primitive meningeal pathology, and more generally, to a neurocristopathy. Accordingly to our observations, this brain malformation appears during both migrational and post-migrational stages and may be considered more like a polymicrogyria than a lissencephaly.     

Diffuse Meningocerebral Angiodysplasia and Renal Agenesis: A Case Report

A third case of meningocerebral angiodysplasia involving the cerebral cortex, with necrosis of both cortex and white matter is described in a neonate with Potter's syndrome. It is suggested that cortical vessel anomalies act as a local intracerebral shunt to produce periventricular infarction. This disorder differs from pure meningeal angiectasis without cerebral infarction and from the classic arteriovenous malformation of the central nervous system with massive systemic shunt, cardiomegaly, and neonatal cardiac failure. Meningocerebral angiodysplasia and renal agenesis appear to form a rare but distinct association.     

混合性脑膜－脑动静脉畸形的血管内栓塞治疗

报告１８例大、中型混合性脑膜－脑动静脉畸形的诊断和血管内治疗。本组均经股动脉或同侧颈动脉置入微导管，采用微钨螺旋圈和氰基丙烯酸酯胶（ＮＢＣＡ，ＩＢＣＡ）或聚乙烯醇颗粒等材料栓塞脑内和脑膜的畸形血管。其中７例经两次栓塞后，畸形血管团消除８０％～９５％；随访３个月至３年，临床头痛及癫痫均得到缓解，未再发生蛛网膜下腔出血。本组资料表明，头痛是脑膜－脑动静脉畸形的重要症状之一；微钨螺旋圈与ＮＢＣＡ或１ＢＣＡ混合栓塞脑内畸形血管团有助于提高栓塞效果；分期栓塞和控制术中与术后的体循环血压可预防或减少栓塞并发症。     

Microvascularization of the intracranial dura mater

Summary The authors have studied the microvascularization of the intracranial dura mater by microradiographic and histologic examination of 73 injected anatomic specimens. There exists a very abundant superficial arterial plexus which also serves to supply the inner table of the cranial vault. This plexus is continuous, even at the walls of the venous sinuses and the dural septa. The arteries are for the most part tortuous. The veins may be satellites of the arteries or, on the contrary, from a plexiform network passing through crevices in the interior of the dural layer. The walls of these veins consist only of an endothelium to be seen within the fibrous layer of the dura. Often, the arteries compress the venous lumen; this dangerous situation probably explains the frequent occurrence of arteriovenous fistulae of the dura mater, known by the name of dural fistulae.

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Microvascularisation de la dure-mère intra-crânienne

Résumé Les auteurs étudient la micro-vascularisation de la dure-mère intra-crânienne grâce à des injections de pièces anatomiques étudiées par microradiographie (73 spécimens) et à l'histologie. Il existe un réseau artériel superficiel anastomotique très riche, qui sert aussi à la vascularisation de la table interne de la voûte du crâne. Ce réseau ne s'interrompt ni au niveau des parois des sinus veineux, ni au niveau des cloisons dure-mériennes. Les artères sont le plus souvent sinueuses. Les veines peuvent être satellites des artères ou au contraire former un réseau plexulaire de fentes à l'intérieur du feuillet dural. Les parois de ces veines ne sont formées que d'un endothélium visible au sein de la couche fibreuse durale. Les artères font souvent impression sur les lumières veineuses; cette situation périlleuse explique probablement la fréquente survenue de fistules artério-veineuses au niveau de la dure-mère, connues sous le nom de fistules durales.

     

非脑膜瘤脑膜异常强化的MRI与病因对比研究

目的：分析不同脑膜病变的强化特征，探讨脑膜强化形式与病因诊断的关系。材料与方法：回顾性分析资料完整的异常脑膜强化２６例，根据脑膜受累层次、范围将强化形式分为四种：（１）弥漫性脑膜强化；（２）弥漫性硬膜强化；（３）弥漫性软膜强化；（４）局限性硬膜强化。依病因不同将脑膜病变分为三组：（１）肿瘤组；（２）炎症组；（３）反应组。结果：２６例中，肿瘤组８例，其中弥漫性硬膜、软膜强化各３例，弥漫性脑膜强化、局限性硬膜强化各１例。炎症组８例，弥漫性软膜强化７例，局限性硬膜强化１例。反应组１０例，８例呈局限性硬膜强化，２例呈弥漫性硬膜强化。结论：不同的脑膜病变可表现不同的强化特征，但离开临床，仅根据强化特征尚难做出定性诊断。     

增强FLAIR T2WI诊断脑膜转移瘤的价值

目的:探讨脑膜转移瘤的MRI表现及增强后FLAIR序列T2WI的诊断价值。方法:回顾性分析20例脑膜转移瘤患者的病例资料,其中硬脑膜转移瘤5例,软脑膜转移瘤15例。所有病例行常规MRI平扫及SE T1WI和FLAIR序列T2WI增强扫描并进行对比分析。结果:MRI平扫检出6例,病灶边界均显示不清;MRI增强扫描检出所有病例,SE-T1WI上病变主要表现为脑膜的线状和/或结节状强化,FLAIR T2WI对软脑膜转移瘤病灶范围的显示更清楚,可鉴别强化的血管与病变。结论:MRI增强扫描是诊断脑膜转移瘤的重要检查方法,增强后FLAIR序列T2WI与SE T1WI同时使用,可提高对软脑膜转移瘤的检出率及诊断准确性。     

散发型脑膜血管瘤病临床病理学分析

目的:探讨散发型脑膜血管瘤病（meningioangiomatosis,MA）的临床病理学特征、诊断及预后,分析血管周围梭形细胞的来源。方法:收集2012年6月至2020年3月首都医科大学宣武医院和福建医科大学附属第一医院诊断的散发型MA病例17例,分析患者的临床资料、影像学特征、病理学特点及预后,通过相关免疫组织化学...     

重点处理静脉端治疗硬脑膜动静脉瘘

目的 探讨治疗硬脑膜动静脉瘘的有效方法。方法 20例硬脑膜动脉瘘患者,其中海绵窦区8例,横窦、乙状窦区6例,小脑幕缘3例,上矢状窦区1例,Galen静脉1例,直窦1例。行引流静脉切断术5例,静脉窦孤立术1例,经静脉途径栓塞14例。结果 临床治愈13例,症状缓解6例,加重1例。影像学检查显示：瘘口完全消失11例;部分消失9例,但血流明显缓慢。术后16例患者获随访,随访时间1个月-4年。结论 重点处理静脉端是治疗硬脑膜动静脉瘘安全有效的方法。     

胃癌转移致脑膜癌病临床分析

目的: 分析16例胃癌致脑膜癌病的临床特征。 方法: 对16例经脑脊液细胞学证实的胃癌致脑膜癌病患者的临床资料进行分析。 结果: 首发症状多为头痛、呕吐,头颅CT或MRI检查多无异常发现,脑脊液检查颅内压均有不同程度增高,脑脊液生化检查蛋白升高者多见,细胞学检查均见癌细胞,形态与原发肿瘤相似,经治疗后31.25%的患者获得缓解。 结论: 对以头痛、呕吐发病,伴颅内压增高及脑膜刺激征,且CT及MRI检查无明显异常者,应高度警惕脑膜癌病的可能,鞘内加全身化疗是有效的治疗手段。