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Fahr综合征即纹状体—苍白球齿状核钙化,临床表现有进行性痴呆、锥体外系统症状和癲痫发作。主要病理变化是双侧基底节区对称性钙化。随着CT的广泛应用,临床上钙化的检出率已有相应的提高。本文报道两个家族(一家系同胞兄弟,一家系同胞姐弟)的Fahr综合征。
Fahr syndrome, the striatum - globus pallidum calcification, clinical manifestations of progressive dementia, extra-pyramidal system symptoms and seizures. The main pathological changes are bilateral basal ganglia symmetry calcification. With the extensive use of CT, the clinical detection rate of calcification has been increased accordingly. This article reports Fahr syndrome in two families (one sibling, one sibling).