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出生至6岁早期治疗的苯丙酮尿症患者的脑电图发育情况

EEG development in early treated PKU patients from birth to 6 years of age.

作者信息

Pietz J, Lütcke A, Sontheimer D, Benninger C, Pietz B, Batzler U, Heusser A

机构信息

Ruprecht-Karls Universität Heidelberg, Kinderklinik, Federal Republic of Germany.

出版信息

Eur J Pediatr. 1990;149 Suppl 1:S28-33. doi: 10.1007/BF02126296.

Abstract

In 126 early treated PKU patients (type I and type II) a close EEG follow up was performed from birth up to 6 years of age. A total of 1465 EEGs were performed before and after onset of dietary treatment and on 11 more subsequent occasions. The composition of the background activity was normal up to 6 years when only a small number of the children (19) showed no dominant alpha activity. The frequency of epileptiform activity of generalised as well as focal type was low in the first 2 years of life, but afterwards slightly enhanced in comparison to normal control groups. Other findings like generalised theta paroxysms or focal slow waves were rarely observed. Under a standardised protein load at 6 months (52 patients) and at 5 years of age (42 patients) a moderate generalised slowing of the background activity but no other abnormalities were noted.

摘要

对126例早期接受治疗的苯丙酮尿症患者(I型和II型)进行了从出生到6岁的密切脑电图随访。在饮食治疗开始前后以及随后的11次随访中,共进行了1465次脑电图检查。在6岁之前,背景活动的组成正常,只有少数儿童(19名)没有优势α活动。全身性和局灶性癫痫样活动的频率在生命的前两年较低,但与正常对照组相比,之后略有增加。很少观察到其他发现,如全身性θ发作或局灶性慢波。在6个月(52例患者)和5岁(42例患者)时进行标准化蛋白质负荷试验,发现背景活动有中度全身性减慢,但未发现其他异常。

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