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Fahr’s综合征,又称对称性大脑钙化综合征,即纹状体苍白球齿状核钙化。相当少见,儿童患者更为罕见。国内未见报道,现将我们遇到的一例报告如下。患儿男,8岁;第一胎,足月须产。于3年前始无诱因的出现发作性全身抽搐伴意识丧失,数分钟后自行缓解。曾用苯妥英钠治疗,开始尚有效,以后药量虽不断增加,效果逐差。近2月来抽搐频发,每日数十次,于1992年3月16日来我院就诊。查体:发育正常,言语、智力和情感均正常,神经系统及内科查体无阳性体征。血钙浓度为1.2mmol/L,血磷浓度为2.6mmol/L;脑电图和脑地形图为重度异常;头颅及泌尿系统X光片未见异常。头颅CT示双侧基底节、额颞叶皮层下对称性斑、块状钙化,CT值110～114Hu(见图片)。约予活性钙,血钙浓度达正常水平后,抽搐好转,又加用苯妥英钠,2个月后复诊,症状完全控制。
Fahr’s syndrome, also known as symmetrical cerebral calcification syndrome, ie, striatum globus pallidum calcification. Very rare, children are more rare. There is no report in our country. Now we report the following one. Children male, 8 years old; first child, full-term need to produce. Three years ago, no incentive for the onset of generalized convulsions associated with loss of consciousness, relieve itself after a few minutes. Have used phenytoin treatment, the beginning is still valid, although the drug continues to increase, the effect of poor. Twitch frequent in recent 2 months, dozens of times a day, in March 16, 1992 to our hospital. Physical examination: normal development, speech, intelligence and emotion are normal, nervous system and medical examination no positive signs. Blood calcium concentration of 1.2mmol / L, phosphorus concentration of 2.6mmol / L; EEG and brain topography was severe abnormalities; no abnormalities in the skull and urinary system X-ray. Head CT showed bilateral basal ganglia, frontal temporal cortex symmetrical plaque, massive calcification, CT value 110 ~ 114Hu (see picture). About active calcium, serum calcium concentration up to normal levels, convulsive improvement, plus use of phenytoin, 2 months after referral, the symptoms completely controlled.