32例苯丙酮尿症脑电图分析

苯丙酮尿症(PKU)是一种先天性代谢性疾病,因缺乏苯丙氨酸羟化酶而发病。患儿多表现智力低下、抽搐、发黄、鼠尿味等。脑电图(EEG)均为广泛异常改变,无局灶改变。血苯丙氨酸(PHE)浓度增高及抽搐者,EEG可有明显改变。因此PKU之EEG改变特点可为病情的估计,疗效的观察及预后提供参考。

AN EEG ANALYSIS OF 32 CASES OF P K U

Phenylketonuris (P K U) is congenital disease of metabolism. The causeis laek of phenylalanine hydroxylase. The clrnical manifestations of ill chil-dren are mental deficiency, spasm, yellow hair, urine with a smell of amouse's and so on. The EEG showed changes of different degrees. Amongthe 32 cases, 22 were males and 10 were females. The youngest age was 4months and the eldest age was 14 years. Abnormal rate of EEG was 43. 8%.The EEG showed hypsarrhythmia, paroxysmal disordered spike-and-slow wavecomplex and slow background activity. All were extensive abnormal changeswithout any local change. The ill children were accompanied with seriousmental deficiency, increase of phenylalanine in concentration and spasm.Their EEG showed obvious changes, and therefore the EEG patterns canprovide a reference for the observation of the patien'cotsndition, theircurative effect and prognosis.