脑膜血管瘤病.docx

脑膜血管瘤病 你见过吗？2015-05-15 10:15来源：丁香园作者：周星驰1986字体大小-|+近日，Neurology 杂志刊登了如下一则病例报道。患儿男性，23 月龄，突发强直阵挛性癫痫发作。腰椎穿刺及常规实验室检查结果未见明显异常。CT（图 1A）显示双侧额部矢状窦旁钙化。脑 MRI 显示病变相应部位信号异常，且异常信号敏感性及增强信号呈现梯度分布，如图 1D 和图 1F 所示。鉴别诊断包括既往感染、血管畸形和肿瘤。活检（图 2）结果支持脑膜血管瘤病的诊断。图 1. CT 和 MRI 平扫显示双额矢状窦旁病变及非对比性轴位 CT 图。（A）提示非结晶高密度矿化伴相应部位低信号敏感性如 MRI（B）所示。（C）T1 平扫显示等信号，而皮质增厚。（D）增强后 T1 显示软脑膜信号强化明显，可能是由于血管增生及轻微非特异性皮质增强改变。皮质下白质信号的变化及脑膜血管瘤特征性改变，仅凭目前检查结果很难理解（E），而后续 T2 液体衰减反转恢复序列（F）结果显著提示该病变。图 2. 脑膜血管瘤组织活检。将 4（A）和 20（B）的脑膜血管瘤活检组织进行苏木精 - 伊红染色，显示螺纹型血管周围脑膜上皮样梭形细胞增殖并伴大量钙化砂粒体。CD31 染色（C）突出了脑膜瘤阳细胞增生周围大量血管包绕。脑膜血管瘤病脑膜血管瘤病在临床上非常罕见，是发生于中枢神经系统的错构性病变，极易误诊为其他神经系统疾病，世界范围内报道仅仅 120 余例，目前脑膜血管瘤病因主要有两种推测：一种认为血管瘤样病变为其基本组织形态，脑膜表面病变为继发性脑膜上皮增生所致；而另一种则关注脑膜上皮增生，认为脑膜血管瘤是脑膜瘤的一种变异型。据第 2 种理论，脑膜血管瘤应属侵袭性脑膜瘤，但其临床表现为良性过程且生长缓慢，故学者多认同其是一种错构性病变。且其病灶多位于大脑颞叶及额叶皮质层并可累及周围脑膜组织，发病年龄从 9 月 -70 岁不等，以幼年至青少年期出现难治性癫痫或顽固性头痛为主要临床表现。也可全无其他症状，本病预后良好，对于难治性癫痫的治疗以尽早手术为主，通过切除病灶治疗癫痫。目前认为，病灶本身就是癫痫灶，手术切除癫痫灶就可控制癫痫发作。本病类似于皮质发育不良，癫痫灶位于病灶周边，手术在切除病灶的同时还要切除其周边的放电皮层。常规术中的脑电监护可以帮助更好的控制癫痫发作，本病例中患者脑膜血管瘤的发生与神经纤维瘤病 2 型相关，且其不表现出其他临床特征。Teaching NeuroImages: Meningioangiomatosis1. Jessie Aw-Zoretic, MB, ChB,2. Delilah Burrowes, MD,3. Nitin Wadhwani, MDand4. Maura Ryan, MD1. From the Departments of Medical Imaging (J.A.-Z., D.B., M.R.) and Pathology (N.W.), Ann & Robert H. Lurie Childrens Hospital of Chicago, IL.1. Correspondence to Dr. Aw-Zoretic:A 23-month-old full-term boy presented with tonic-clonic seizures. Lumbar puncture and routine laboratory tests were unremarkable. CT (figure 1A) showed bifrontal parasagittal calcification. Brain MRI revealed corresponding signal abnormalities with gradient susceptibility (figure 1D) and enhancement (figure 1F). Differential diagnoses included prior infection, vascular malformation, and tumor. Biopsy (figure 2) was consistent with meningioangiomatosis.View larger version: In this window Download as PowerPoint SlideFIGURE 1CT and MRI of bifrontal parasagittal lesion noncontrast axial CT(A) Amorphous high density indicative of mineralization with corresponding subtle low signal on susceptibility MRI (B). (C) Precontrast T1 shows isointense but thickened cortex. (D) Postcontrast T1 shows robust leptomeningeal enhancement, presumably due to vascular proliferation, and subtle nonspecific cortical enhancement. Subcortical white matter signal changes, characteristic of meningioangiomatosis, were initially difficult to appreciate (E) but were more apparent on follow up T2fluid-attenuated inversion recovery image (F).View larger version: In this window Download as PowerPoint SlideFIGURE 2Histology of meningioangiomatosisHematoxylin & eosin staining at 4 (A) and 20 (B) magnification shows whorls of meningothelial-like spindle cells proliferating around blood vessels with numerous calcified psammoma bodies. CD31 staining (C) highlights the numerous blood vessels around which the meningothelial-like cells proliferate.Meningioangiomatosis is a rare epileptogenic lesion involving the meninges and cortex characterized by vascular proliferation and calcifications. Meningioangiomatosis occurs sporadically or in association with neurofibromatosis 2 (NF2).1,2Testing for NF2 proved positive in this patient, despite no other clinical stigmata.Next SectionAUTHOR CONTRIBUTIONSJessie Aw-Zoretic: drafting/revising the manuscript, analysis or interpretation of data, accepts responsibility for conduct of research and final approval, acquisition of data, image processing. Delilah Burrowes: study concept or design, accepts responsibility for conduct of research and final approval, study supervision. Nitin Wadhwani: drafting/revising the manuscript, analysis or interpretation of data, accepts responsibility for conduct of research and final approval, acquisition of data. Maura Ryan: drafting/revising the manuscript, study concept or design, accepts responsibility for conduct of research and final approval.Previous SectionNext SectionSTUDY FUNDINGNo targeted funding reported.Previous SectionNext SectionDISCLOSUREThe authors report no disclosures relevant to the manuscript. Go toNfor full disclosures.Previous SectionNext SectionFOOTNOTES Go toNfor full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article. Download teaching slides:N 2015 American Academy of NeurologyPrevious SectionREFERENCES1. 1.1. JeonTY,2. KimJH,3. SuhYL,4. AhnS,5. YooSY,6. EoH.Sporadic meningioangiomatosis: imaging findings with histopathol