Fahr病的临床表现与影像学诊断

The Clinical Manifestation and Imaging Diagnosis of Fahr Disease

目的探讨Fahr病的临床表现和影像学的诊断价值。方法回顾性分析1999年-2008年就诊的11例经Fahr病的不同临床表现和CT、MRI及SPECT资料。结果所有患者都接受CT检查,9例MRI检查,8例SPECT扫描。CT可清晰地显示钙化病灶的大小范围;在显示钙化方面,MRI不如CT敏感,但MRI能显示病灶内和周围胶质增生、囊变与脱髓鞘的范围及程度;亦能较清晰的显示小脑齿状核的病变情况;SPECT能早期监测到双侧基底节和双侧大脑皮质的局部脑血流灌注下降程度。结论Fahr病临床表现各异,易误诊。CT、MRI和SPECT对其诊断具有协同作用。

Objective To investigate the clinical manifestation,radiographic characteristic and the diagnosic value of CT,MRI and SPECT for Fahr disease.Methods Eleven cases who were clinically proved as Fahr disease were analyzed retrospectively from 1999 to 2008.Their clinical manifestations and the results of CT,MRI and SPECT finding were compared.Results All patients received CT,and nine underwent MRI and eight underwent SPECT.CT could clearly display the size and scope of the calcified lesions which was more sensitive than MRI,but MRI could show the range and extent of the gliosis and cystification and demyelination by utilization of gradient echo sequense,and MRI could also evaluate the lesions in the dentate nucleus because it overcame the volume effect arised from the cranial bone.Furthermore,SPECT could early monitor the regional cerebral blood flow of the bilateral basal ganglia and bilateral cerebral cortex which generally decreased.Conclusion In the absense of specific clinical manifestations,Fahr disease is often misdiagnosed.CT,MRI and SPECT are synergistic in diagnosis of Fahr disease.