基底节钙化的鉴别诊断  

Differential diagnosis of basal ganglia calcification

作　　者：褚雪颖 邢小平[1] 王鸥[1] 魏妍平[1] 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院内分泌科国家卫生和计划生育委员会内分泌重点实验室,北京100730

出　　处：《中华骨质疏松和骨矿盐疾病杂志》2017年第2期170-178,共9页Chinese Journal Of Osteoporosis And Bone Mineral Research

基　　金：国家自然科学基金(81270873;81100559);国家临床重点专科建设项目(WBYZ2011-873)

摘　　要：基底节钙化(basal ganglia calcification,BGC)是临床常见CT表现,在医院就诊的一般患者中行CT扫描可高达20%发现纹状体、苍白球及齿状核等区域不同程度钙化。双侧对称的纹状体-苍白球-齿状核钙化又被称为Fahr's综合征,临床表现从无症状到各种运动障碍及神经精神表现等均有报道。多种原发或继发性病因可导致Fahr's综合征,其中最常见的有特发性BGC、甲状旁腺功能减退等,另外一些代谢性疾病如线粒体肌病、自身免疫性疾病如系统性红斑狼疮、感染、颅内肿瘤及一些先天性综合征等均可有类似表现,而预后及治疗方式各不相同。因此Fahr's综合征的鉴别诊断显得尤为重要。本文对BGC的病理生理,及生理性及各种不同病因病理性BGC的发病机制、临床及影像学表现进行综述。Basal ganglia calcification （BGC） of with or without dentate nucleus, thalamus, and white matter involved is commonly seen in CT scans, which can be detected in up to 20% patients. Bilateral strio-pallido-dentate calcinosis is also called Fahr＇s syndrome. Clinically, it was reported from asymptomatic to conditions of movement and/or neuropsychiatric disorders. Various diseases can present as Fahr＇s syndrome, including idiopathic basal ganglia calcification （ IB- GC）, hypoparathyroidism, metabolic disorders like certain forms of mitochondrial encephalopathies, autoimmune diseases like systemic lupus erythematosus （ SLE ）, infection, tumor and some congenital syndromes, of which the therapy and prognosis will be totally different. Thus the differential diagnosis of Fahr＇ syndrome appears to be of great importance. This article will review the pathology, mechanism and clinical as well as imaging features of different causes of BGC.

关 键 词：基底节钙化 Fahr's综合征 Fahr's病 病因 

分 类 号：R445.3[医药卫生—影像医学与核医学]