Wiebe S, Munoz D G, Smith S, Lee D H
Department of Clinical Neurological Sciences, University of Western Ontario, London, Canada.
Brain. 1999 Apr;122 ( Pt 4):709-26. doi: 10.1093/brain/122.4.709.
Meningioangiomatosis (MA) is a rare, benign, focal lesion of the leptomeninges and underlying cerebral cortex characterized by leptomeningeal and meningovascular proliferation. It may occur sporadically or in association with neurofibromatosis type 2. Previous reports have emphasized histological and imaging features. Data on the management of these patients are sparse, and electrophysiological features of MA lesions have not been published. We assessed the clinical, electrophysiological, histopathological and imaging features as well as the surgical outcome in MA, and compared MA with and without neurofibromatosis. Seven patients with MA at our centre were investigated and their outcome was assessed. A review of the literature is included. MA exhibits a wide range of clinical, imaging, histopathological and electrophysiological features, making the diagnosis difficult. Sporadic MA cases are not associated with neurofibromatosis and the two disorders are genetically distinct. Medically refractory, localization-related epilepsy is the commonest presentation in sporadic cases, but atypical presentations also occur. Unlike sporadic cases, MA with neurofibromatosis is often found incidentally, does not produce seizures, occurs less frequently (ratio of 1:4), and is multifocal. MRI findings in MA correspond to the histological picture. However, the appearance on imaging is non-specific and may suggest cystic atrophy, angioma and tumours. Several abnormalities have been found in close proximity to MA lesions, i.e. meningioma, oligodendroglioma, arteriovenous malformation, encephalocoel and orbital erosion. In spite of histopathological diversity, MA lesions are either predominantly cellular or vascular. Immunohistochemical results are inconsistent among cases, add little to the diagnosis, and do not support a meningeal origin. Electrocorticographic recordings from the surface and within MA lesions revealed a spectrum of electrophysiological expressions. Intrinsic epileptogenicity of MA lesions was documented in some cases. Epileptogenicity was confined to the perilesional cortex in some patients and it was complex (extralesional, multifocal, generalized) in others. Only 43% of our patients became seizure-free postoperatively compared with 68% previously reported, and >70% of our patients and those in the literature continued to require antiepileptic drugs. This is in keeping with the diverse electrophysiology of MA and suggests a less optimistic postoperative outcome than previously recognized.
脑膜血管外皮细胞瘤（MA）是一种罕见的、良性的软脑膜及脑皮质下局灶性病变，其特征为软脑膜和脑膜血管增生。它可散发性发生，也可与2型神经纤维瘤病相关。既往报道主要强调组织学和影像学特征。关于这些患者治疗的数据较少，且MA病变的电生理特征尚未见报道。我们评估了MA患者的临床、电生理、组织病理学和影像学特征以及手术结果，并比较了伴有和不伴有神经纤维瘤病的MA。对我们中心的7例MA患者进行了研究并评估其结果。文中还包括文献综述。MA具有广泛的临床、影像学、组织病理学和电生理特征，这使得诊断困难。散发性MA病例与神经纤维瘤病无关，这两种疾病在基因上是不同的。药物难治性、与定位相关的癫痫是散发性病例最常见的表现，但也会出现非典型表现。与散发性病例不同，伴有神经纤维瘤病的MA常为偶然发现，不引起癫痫发作，发生率较低（比例为1:4），且为多灶性。MA的MRI表现与组织学图像相符。然而，影像学表现是非特异性的，可能提示囊性萎缩、血管瘤和肿瘤。在MA病变附近发现了几种异常情况，即脑膜瘤、少突胶质细胞瘤、动静脉畸形、脑膨出和眼眶侵蚀。尽管组织病理学存在多样性，但MA病变主要为细胞性或血管性。病例之间免疫组化结果不一致，对诊断帮助不大，且不支持脑膜起源。对MA病变表面及内部进行皮质脑电图记录显示出一系列电生理表现。在某些病例中记录到MA病变具有内在致痫性。在一些患者中，致痫性局限于病变周围皮质，而在另一些患者中则较为复杂（病变外、多灶性、全身性）。我们的患者中只有43%术后无癫痫发作，而此前报道的比例为68%，并且我们的患者及文献中的患者超过70%仍需继续服用抗癫痫药物。这与MA多样的电生理情况相符，提示术后结果不如之前认为的乐观。
