硬脑膜动静脉瘘介入治疗的临床分析

目的：总结硬脑膜动静脉瘘（DAVF）患者的介入治疗特点,以提高疗效。方法：回顾性分析135例DAVF患者的临床资料。结果：治愈74例,好转53例,8例无效,其中1例死于颅内出血。结论：DAVF的临床表现和预后与其静脉引流类型密切相关,颈动脉压迫法及选择性血管内栓塞是安全有效的治疗方法。     

Clear-Cell Meningioma: CT and MR Imaging Findings in Two Cases Involving the Spinal Canal and Cerebellopontine Angle

Clear-cell meningioma is a rare subtype of meningioma which occurs at a younger age and has a higher recurrence rate than other subtypes. We report two cases of clear-cell meningioma, one in the thoracolumbar spinal canal and the other in the cerebellopontine angle. Though the CT and MR imaging findings were not different from those of ordinary meningioma, after surgical removal the condition recurred repeatedly in the patient with spinal canal involvement.     

Meningeal involvement in Behçet's disease: MRI

Behçet's disease is a multisystem disease that involves the central nervous system up to half of cases. Presentation with neurologic symptoms occurs in 5 % of cases and cerebral venous thrombosis is one of its major manifestations. A feature not previously reported is progressive meningeal thickening with involvement of both optic nerves. We report a patient with cerebral venous thrombosis, meningeal thickening and contrast enhancement on MRI. This patient had two other unusual features: positive antineutrophil cytoplasmic antibodies and later development of central diabetes insipidus.     

基于QST分型的颅咽管瘤与第三脑室底脑膜层次的关系

目的探索基于QST分型的、累及第三脑室底的颅咽管瘤与第三脑室底脑膜层次的关系及临床意义。方法回顾性分析2018年1月至2019年10月南方医科大学南方医院神经外科在神经内镜下行肿瘤全切除术治疗累及第三脑室底的原发性颅咽管瘤患者的临床资料(共17例,其中Q型6例,S型3例,T型8例),所有患者术中均留取肿瘤组织标本。正常鞍区标本来源于同期该院行人工引产或自然流产的胎儿(8例)。对以上标本进行苏木素-伊红(HE)和免疫荧光双标染色,应用波形蛋白抗体标记硬脑膜,Ⅰ型胶原蛋白抗体标记蛛网膜,胶质纤维酸性蛋白抗体和层粘连蛋白抗体标记软脑膜,以CK18抗体标记腺垂体,以CK5/6抗体标记颅咽管瘤。观察胎儿脑组织标本的脑膜染色情况以及不同QST分型的颅咽管瘤组织与第三脑室底脑膜的层次关系。结果8例胎儿标本均成功标记硬脑膜、蛛网膜、软脑膜。颅咽管瘤标本HE染色及免疫荧光双标染色结果显示,所有Q型肿瘤(6/6)与第三脑室底之间存在硬脑膜(鞍膈);所有S型肿瘤(3/3)与第三脑室底之间存在蛛网膜和软脑膜;T型肿瘤与第三脑室底之间存在3种病理学形态关系,分别命名为卯榫样、地幔样及护城河样关系,所有T型肿瘤(8/8)与第三脑室底之间有软脑膜分隔,但在肿瘤起源点处,软脑膜可逐渐消失。当肿瘤极度挤压第三脑室空间时,第三脑室室管膜仍可保持完整。结论不同QST分型的颅咽管瘤均可累及第三脑室底,且与第三脑室底之间存在不同的脑膜层次;这些脑膜层次是手术安全切除累及第三脑室底的颅咽管瘤的天然屏障。     

Malignant solitary fibrous tumor of the meninges

Increasing numbers of solitary fibrous tumors (SFTs) in the meninges have been reported since this entity was first recognized. While most cases previously reported were considered to be benign, the malignant potential of extrathoracic SFTs has not been excluded. The authors report a rare case of a meningeal SFT with malignant behavior occurring in a Japanese female patient, initially resected when she was 44 years old and recurring in the same place four times during a 26-year follow-up period. A metastatic tumor to the right lung arose 25 years after the resection of the first meningeal tumor and focal invasion into the cerebellum was also observed with her last (5th) meningeal tumor. Immunohistochemical analysis showed all tumors to be diffusely positive for CD34 and negative for EMA, with a so-called patternless histological pattern, featuring thin collagen fibers between tumor cells. A focal staghorn vascular pattern was also observed. Ki67 (MIB-1) labeling indices and mitosis rates were 3.1±1.2% and less than 1/10 high power fields (HPF) in the first meningeal tumor and 16.1±6.4% and 6/10HPF in the last (5th) one, respectively. Thus, the present case suggests that meningeal SFTs possess malignant potential so that careful long-term follow up is required.     

Intracranial solitary fibrous tumor: Imaging findings

Objective

To study the neuroimaging features of intracranial solitary fibrous tumors (ISFTs).

Materials and methods

Retrospective study of neuroimaging features of 9 consecutive histopathologically proven ISFT cases. Location, size, shape, density, signal intensity and gadolinium uptake were studied at CT and MRI. Data collected from diffusion-weighted imaging (DWI) (3 patients), perfusion imaging and MR spectroscopy (2 patients), and DSA (4 patients) were also analyzed.

Results

The tumors most frequently arose from the intracranial meninges (7/9), while the other lesions were intraventricular. Tumor size ranged from 2.5 to 10 cm (mean = 6.6 cm). They presented multilobular shape in 6/9 patients. Most ISFTs were heterogeneous (7/9) with areas of low T2 signal intensity that strongly enhanced after gadolinium administration (6/8). Erosion of the skull was present in about half of the cases (4/9). Components with decreased apparent diffusion coefficient were seen in 2/3 ISFTs on DWI. Spectroscopy revealed elevated peaks of choline and myo-inositol. MR perfusion showed features of hyperperfusion.

Conclusion

ISFT should be considered in cases of extra-axial, supratentorial, heterogeneous, hypervascular tumor. Areas of low T2 signal intensity that strongly enhance after gadolinium injection are suggestive of this diagnosis. Restricted diffusion and elevated peak of myo-inositol may be additional valuable features.     

Extramedullary hematopoiesis involving the central nervous system and surrounding structures

Extramedullary hematopoiesis in central nervous system is a rare condition that can present in association with hematologic conditions. In this single-center retrospective study from 1988 to 2010, we identified 8 cases of extramedullary hematopoiesis involving the central nervous system or meninges. Most were incidental autopsy findings, usually microscopic collections of cells in the meninges or around blood vessels in the brain of infants (4 cases; age, 10 days to 5 months), in 1 young child (age, 6.5 years with anemia), and 1 elderly adult (age, 81 years with end-stage idiopathic myelofibrosis). Two individuals (both with polycythemia rubra vera) presented with symptomatic intracranial or spinal canal lesions (age, 67 and 68 years). Mixed populations of immature hematopoietic cells were identified by characteristic morphology and appropriate immunohistochemistry. The precise pathogenesis of extramedullary hematopoiesis in central nervous system is unknown. In infants, extramedullary hematopoiesis is unlikely to be pathologic and most likely represents a resolving fetal state. The older children and adults with incidental or symptomatic extramedullary hematopoiesis usually have some underlying hematologic condition, which should be sought if not already recognized.     

Hippocampus/amygdala alterations, loss of heparan sulfates, fractones and ventricle wall reduction in adult BTBR T+ tf/J mice, animal model for autism

Multiple studies converge to implicate alterations of the hippocampus and amygdala in the pathology of autism. We have previously reported anatomical alterations of the meninges, vasculature and fractones, the specialized extracellular matrix (ECM) of the subventricular zone, in the forebrain of adult BTBR T+ tf/J mice, animal model for autism. Here, we used bisbenzidine cell nucleus staining and dual immunofluorescence histochemistry for laminin and N-sulfated heparan sulfate proteoglycans (NS-HSPG) to examine a series of brain sections containing the amygdala and hippocampus in the adult BTBR T+ tf/j mouse. We observed an excessive separation of the two hippocampi, a modified trajectory of the meninges leading to a shrunken choroid plexus in the lateral ventricle, a shorter granular layer of the dentate gyrus, and a reduced size of the amygdala nuclei. The lateral ventricle near the amygdala, and the third ventricle were shrunken. The number and size of fractones, and their immunoreactivity for NS-HSPG, were reduced throughout the third and lateral ventricles walls. Enlarged blood vessels were found at the endopiriform cortex/amygdala interface. These results show anatomical alterations of the hippocampal/amygdala that are associated with defects of the choroid plexus/ventricular system and the ECM in the BTBR T+ TF/J mouse. Similar alterations of the hippocampus/amygdala axis in humans with autism to these observed in BTBR T+ tf/J mice make this animal model highly valuable for the study of autism. Moreover, the meningo/vascular and ECM alterations in BTBR T+ Tf/J mice suggest a possible role of the brain connective tissue in autism.     

脑膜癌病临床与脑脊液细胞学研究

目的：探讨脑膜癌病的临床特征和脑脊液细胞学改变。方法：总结分析11例经脑脊液细胞学检查确诊的脑膜癌病患的临床资料。结果：临床上以颅内高压,脑膜刺激征阳性和颅神经损害为主要表现,头颅CT,MRI检查常为阴性,脑脊液细胞学检查肿瘤细胞检出率高,细胞免疫组化染色对鉴定肿瘤细胞性质有帮助。结论：脑脊液细胞学检查结合细胞免疫组化染色是诊断脑膜癌病最佳手段。     

天幕脑膜瘤的术后护理

目的探讨天幕脑膜瘤患者的术后护理。方法加强患者术后病情观察,监护生命体征的变化,防治并发症,维持良好的营养状态。结果15例患者术后未发生手术相关的感染或死亡。患者出院后经6个月~5年的随访,12例治愈;2例分别于手术后1年、4年复发,行再次手术治愈;1例肿瘤生长活跃,有恶性倾向。结论做好天幕脑膜瘤患者术后的护理,积极防治并发症,是促进患者早日康复的重要环节。