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婴儿期癫痫性进行性脑病(韦斯特综合征和伦诺克斯 - 加斯东综合征)(作者译)

[Epileptic evolutive encephalopathies in infants (West tsyndromee and Lennox-Gastaut syndrom) (author's transl)].

作者信息

Pinsard N

出版信息

Rev Electroencephalogr Neurophysiol Clin. 1981 Dec;11(3-4):419-24. doi: 10.1016/s0370-4475(81)80080-9.

DOI:10.1016/s0370-4475(81)80080-9
PMID:7345500
Abstract

Electroclinical aspects of the West syndrome are studied in their atypical forms: clinical and EEG characteristics, partial forms, Aicardi syndrome. Etiological investigations (especially CT scan) are necessary to distinguish the more frequent 'secondary forms' from the 'primary forms'. The etiological factors are: (i) Prenatal causes (hereditary affections, chromosome-related etiology, fetal etiology; (ii) Perinatal causes (prematurity, traumatic delivery); (iii) Postnatal causes (infectious diseases). The evolutive aspects and prognosis are different for 'primary' and 'secondary' forms. The time of commencement of the mode of therapy and its duration are important factors in relation to the prognosis of 'primary forms'. A pre-existing encephalopathy obviously leads to a very poor prognosis. A typical Lennox-Gastaut syndrome can be observed in infants. However, atypical forms are more frequent. These too have a poor prognosis because they are usually secondary to cerebral lesions.

摘要

研究了West综合征非典型形式的临床电生理方面:临床和脑电图特征、部分形式、Aicardi综合征。病因学调查(尤其是CT扫描)对于区分较常见的“继发性形式”和“原发性形式”很有必要。病因包括:(i)产前原因(遗传性疾病、染色体相关病因、胎儿病因);(ii)围产期原因(早产、分娩创伤);(iii)产后原因(传染病)。“原发性”和“继发性”形式的演变情况和预后不同。治疗方式开始的时间及其持续时间是与“原发性形式”预后相关的重要因素。先前存在的脑病显然预后很差。婴儿中可观察到典型的Lennox-Gastaut综合征。然而,非典型形式更为常见。这些形式的预后也很差,因为它们通常继发于脑部病变。

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