Varoglu Asuman Orhan
Departments of Neurology, Medical Faculty, University of Ataturk, Erzurum, Turkey.
Eurasian J Med. 2008 Aug;40(2):85-7.
Fahr's syndrome is a rare neurological condition in which calcification of vessels in the basal ganglia and cerebellum usually occurs. Impaired glucose tolerance (IGT) may be present, causing multiple organ complications by means of atherosclerosis. We report a case of Fahr's syndrome associated with hypoparathyroidism and IGT. We present the case of a 52-year-old man with Fahr's syndrome and IGT. The patient's neurological examination was normal except for bilateral tremor. Laboratory investigations showed that the level of serum Ca (calcium) was 5.9 mg/dl, the parathormone (PTH) level was 1.95 pg/ml (normal level 15-65 pg/ml) and the phosphate (P) level was 5.45 mg/dl (normal level 2-6 mg/dl). Bilateral and symmetric calcifications in the basal ganglia, the centrum semiovale, and the cerebellum were demonstrated with a computerized scan of the head (CT) and magnetic resonance imaging (MRI). Therefore, the patient was diagnosed with Fahr's syndrome. An endocrinologist was consulted, and treatment was started according to the suggestions of the endocrinologist. Here we report a case of Fahr's syndrome associated with hypoparathyroidism and IGT. Atherosclerosis due to IGT may contribute to the calcification of brain vessels. Further investigations will be needed to clarify the pathogenesis of Fahr's syndrome.
Fahr综合征是一种罕见的神经系统疾病，通常会出现基底神经节和小脑血管钙化。可能存在糖耐量受损（IGT），通过动脉粥样硬化导致多器官并发症。我们报告一例与甲状旁腺功能减退和IGT相关的Fahr综合征病例。我们介绍一名患有Fahr综合征和IGT的52岁男性病例。除双侧震颤外，患者的神经系统检查正常。实验室检查显示血清钙（Ca）水平为5.9mg/dl，甲状旁腺激素（PTH）水平为1.95pg/ml（正常水平15 - 65pg/ml），磷酸盐（P）水平为5.45mg/dl（正常水平2 - 6mg/dl）。头部计算机断层扫描（CT）和磁共振成像（MRI）显示基底神经节、半卵圆中心和小脑有双侧对称性钙化。因此，该患者被诊断为Fahr综合征。咨询了内分泌科医生，并根据内分泌科医生的建议开始治疗。在此我们报告一例与甲状旁腺功能减退和IGT相关的Fahr综合征病例。IGT引起的动脉粥样硬化可能导致脑血管钙化。需要进一步研究以阐明Fahr综合征的发病机制。
