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目的 探讨Fahr病的临床特点和CT特征。方法 回顾性分析了7例Fahr病患者的临床和CT资料,并结合文献进行讨论。结果(1)2例有家族遗传史,5例为散发;(2)临床表现为癫痫、精神障碍、智力低下或痴呆、震颤麻痹等,无神经系统定位体征;(3)病程较长,平均12年;(4)CT表现为基底节区两侧对称(6例)或单侧(l例)钙化灶,可伴有丘脑、小脑齿状核或大脑灰白质交界区钙化;(5)血清钙、磷正常。结论CT是诊断Fahr病的主要检查方法。
Objective To investigate the clinical features and CT features of Fahr disease. Methods The clinical and CT data of 7 patients with Fahr disease were retrospectively analyzed and discussed in the literature. (2) clinical manifestations of epilepsy, mental disorders, mental retardation or dementia, paralysis, such as paralysis, no signs of neurological localization; (3) a longer duration, the average 12 years; (4) CT showed bilateral symmetry (6 cases) or unilateral (1 case) calcification in the basal ganglia, which may be associated with calcification of the thalamic, cerebellar dentate nuclei or the cerebral gray matter junction; (5) Calcium, phosphorus normal. Conclusion CT is the main method of diagnosis of Fahr disease.