Fahr’s病CT表现与临床.docVIP

Fahr’s病CT表现与临床 　　[摘要] 目的:探讨Fahr’s病的CT表现和临床资料,以更好地认识Fahr’s病。方法:回顾性分析9例临床确诊为Fahr’s病的临床和影像学资料,全部病例均行颅脑CT扫描。结果:全部病例发病年龄为15～70岁,血清钙、磷均在正常范围,CT表现为颅内广泛对称性钙化,主要累及基底节、小脑齿状核、皮质下和丘脑。临床表现最常见有帕金森综合征,其次有精神障碍、记忆力下降、智力低下、癫痫、舞蹈症等。 结论:根据CT表现、临床表现、实验室检查和家族病史,可以对Fahr’s病作出正确的诊断,CT是Fahr’s病诊断最有价值的影像学检查,预防与对症治疗是目前主要治疗目的。 　　[关键词] Fahr’s病,颅内钙化,CT,帕金森综合征 　　 [Abstract] Objective To evaluate the characteristics of clinical imaging of Fahr’s disease for further understanding of it.MethodsClinical data and radiographic findings in 9 cases with clinical confirmed Fahr’s disease were retrospectively analyzed. CT scan was performed in all the patients.ResultsThe age of all cases were from 15 years to 70 years, the serum calcium and phosphorum were in the normal range. The features of CT showed the bilateral diffuse symmetric intracranial calcinosis, involved in the basal ganglia, dentate nucleus, subcortical nuclei and thalamus. The Clinical presentation included parkinsonism, mental disorder, memory decline, intelligence retardation, epilepsy and chorea, the parkinsonism was the most common manifestation.ConclusionsFahr’s disease could be diagnosed according to the radiological features, clinical manifestation, laboratory examination, and the family history. CT was the most valuable examination to Fahr’s disease.Prevention and symptomatic treatment were the main goals of treatment at present. 　　[key words] Fahr’s disease, Intracranial calcinosis, CT, Parkinsonism 　　 　　Fahr’s病又称家族性脑血管亚铁钙沉着症,在1850年Delacour首次报道,1930年Karl Theodor Fahr加以描述,指的是单发的或家族性特发性基底节钙化,与多种神经学和精神病学异常存在密切的相关性。本文搜集我院9例Fahr’s病,结合文献资料,对Fahr’s病进行探讨。 　　 　　资料与方法 　　 　　1. 收集本院2001年至2009年9例,纳入标准:头颅CT表现为颅内广泛对称性钙化,血清钙、磷,尿钙在正常范围,有或无相关家族史,无感染、中毒、代谢方面病因。男4例,女5例,其中15岁1例;20～30岁(2例);36岁1例;40～50岁(2例),60岁1例,70岁1例,平均年龄41.1岁。临床表现有幻听(1例)、被害妄想(1例)、行为紊乱(3例)、言语紊乱(3例)、记忆力下降(3例),癫痫(3例),智力低下(1例),颈部不自主运动(舞蹈症1例),其中一例无任何临床症状,出现脑膜炎后做CT扫描发现。6例至少有1位直系亲属有颅内广泛钙化病史及神经学或(和)精神学障碍病史,3例无明显相关家族史。全部病例由资深的神经专科和影像医师研究后诊断为Fahr’s病。 　　2.检查方法:所有患者采用GE 1800I 或飞利浦Brillia