Abstract
Objectives: To study the clinical features and diagnostic value of CT in Fahr’s disease. Methods: We retrospectively analyzed skull images of 8,000 cases and observed the incidence and imaging characteristics of Fahr’s disease. Results: Fahr’s disease were found in 6 patients (6/4000=0.075%)whose ages ranged from 16 to 62 years (average age:38.5±6.4years). We found that 6 Fahr’s disease had been incidentally detected in 4 men(4/6=66.7%) and 2 women(2/6=33.4%); serum calcium：2.13～2.42 mmol/L，serum phosphor：1.0～1.6 mmol/L.The most common CT feature of Fahr’s disease is the presence of small bilateral intracranial calcifications which are usually restricted to the bilateral globus pallidus, putamen, caudate nucleus, thalamus, dentate nucleus and white matter of the cerebral hemispheres. Conclusions: Combined with clinical manifestations and imaging features, we can make a clear diagnosis of the Fahr’s disease.
Highlights
We found that 6 Fahr’s disease had been incidentally detected in 4 men(4/6=66.7%) and 2 women(2/6=33.4%); serum calcium:2.13~2.42 mmol/L,serum phosphor:1.0~ 1.6 mmol/L.The most common CT feature of Fahr’s disease is the presence of small bilateral intracranial calcifications which are usually restricted to the bilateral globus pallidus, putamen, caudate nucleus, thalamus, dentate nucleus and white matter of the cerebral hemispheres. .Conclusions:Combined with clinical manifestations and imaging features, we can make a clear diagnosis of the Fahr’s disease
需与 Fahr’s 病相鉴别疾病主要有甲状旁腺机能减退,假性甲状旁腺机能减退,结节性硬化等,甲状旁腺机 能减退及假性甲状旁腺机能减退疾病均表现为双侧基底节区、皮层下白质及小脑齿状核对称性广泛性钙化, 与 Fahr’s 病较难区分,但两者均有钙、磷代谢异常,即血钙低、血磷高,且假性甲状旁腺机能减退双手 X 线表现突出,第 3-5 掌骨短,第 1-4 指末节指骨短,有时有骨膜下骨质的吸收改变。结节性硬化多位于侧 脑室体部旁即前角边缘突入脑室的结节,可有钙化,临床有典型的智力低下、癫痫、皮质腺瘤 Vogt 三联征, 故结合临床病史及典型的 CT 表现,可对 Fahr’s 病做出明确诊断。
Summary
胡卫东 1 ,项立 2 ,王秀荣 1 ,朱记超 1 ,谢尚煌 1 ,梁文彬 1 ,申云霞 1 深圳市龙岗中心医院 1 医学影像科,[2] 内二科,广东深圳 518116 通讯作者:胡卫东,Email: clzh_2004@163.com 【摘要】目的 研究 Fahr’s 病的临床特点及其 CT 诊断价值。方法 回顾性分析 8000 例头颅图片,观察 Fahr’s 病发生率及影 像学特点。结果 共发现 6 例 Fahr’s 病,发生率(6/8000=0.075%);年龄:16~62 岁,平均年龄(38.5±6.4)岁;其中男 4 例,女 2 例; 血清钙:2.13~2.42 mmol/L,血清磷:1.0~1.6 mmol/L;CT 主要特点是颅内小对称性的钙化灶,主要集中在双侧苍白球、 尾状核、丘脑、齿状核和大脑半球白质区。结论 结合临床表现和影像特征可对该病做出明确诊断。 【关键词】 Fahr’s 病;断层摄影;X线计算机 Fahr’s 病由 Fahr 首次提出,又称铁钙沉积症(ferrocalcinosis),作为一种罕见疾病临床开始对其进行 研究和报道,影像方面的研究较少,且主要集中在国外,国内亦有相关报道,对国人 CT 图片进行回顾性 分析研究其发病率报道较少[1~10],本文通过对 6 例 Fahr’s 病的临床表现及 CT 特征进行分析来探讨其影像 诊断价值。
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