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Abstract：
Primary familial brain calcification (PFBC) is an idiopathic pathology characterized by the development of calcific deposits in the supratentorial region such as telencephalon and diencephalon but also, in more extensive forms, in the cerebellum. Meningiomas are among the most common central nervous system (CNS) tumors generally related to a good prognosis. The simultaneous presence of intracerebral or extra-axial tumors and PFBC represents an exceedingly rare occurrence. A 72-year-old female patient was admitted to our department because of anoculogyric crisis followed by generalized seizures. She performed a brain CT scan which showed widespread hyperdensities in the paraventricular supratentorial region, basal ganglia and at the level of bilateral cerebellar dentate nuclei, characteristics of PFBC. Concomitant left frontal and smaller right temporal extra-axial lesions were identified and then confirmed in a brain MRI. The patient underwent a microsurgical resection of the lesion and subsequent histological examination reported a meningothelial meningioma (WHO Grade I). According to our literature review, this is the first paper that reports the coexistence of both intracranial meningiomas and PFBC. To date, it is not possible to provide an exact correlation between pathogenesis and genetic mechanism underlying this association.
摘要：
原发性家族性脑钙化（PFBC）是一种特发性病理，其特征是在幕上区域，如端脑和间脑，以更广泛的形式，在小脑.脑膜瘤是最常见的中枢神经系统（CNS）肿瘤之一，通常与良好的预后有关。脑内或轴外肿瘤和PFBC的同时存在代表非常罕见的发生。一名72岁的女性患者因失眼危象和全身性癫痫发作而入院。她进行了脑部CT扫描，显示室旁幕上区域广泛的高密度，基底神经节和双侧小脑齿状核水平，PFBC的特点。合并的左额叶和较小的右颞叶轴外病变被发现，然后在脑MRI中被证实。患者接受了病变的显微外科手术切除，随后的组织学检查报告了脑膜上皮脑膜瘤（WHOI级）。根据我们的文献综述，这是第一篇报道颅内脑膜瘤和PFBC共存的论文。迄今为止,不可能提供发病机制和遗传机制之间的确切相关性。
