Rapin I
Saul R. Korey Department of Neurology, Department of Pediatrics, Albert Einstein College of Medicine, Bronx, NY, USA.
Semin Pediatr Neurol. 1995 Dec;2(4):278-85. doi: 10.1016/s1071-9091(95)80007-7.
At least a third of autistic toddlers regress in language, sociability, play, and often cognition. Many fewer children undergo a similar, unexplained regression after language is fully developed (disintegrative disorder [DD]). Epilepsy or a paroxysmal electroencephalogram (EEG) with/without clinical seizures, including electrical status epilepticus in slow wave sleep (ESES), may be associated, in occasional children, with either selective loss of language (Landau-Kleffner syndrome [LKS]) or with pervasive autistic regression. Fluctuation in language and behavior deficits should raise the suspicion of epilepsy. Review of the literature and of the author's experience suggests that epilepsy probably plays a relatively minor, although non-negligible, pathogenetic role in autistic regression. Multidisciplinary, possibly multi-institutional, longitudinal studies that encompass the regression are needed to sharpen diagnostic criteria to devise more effective therapies.
至少三分之一的自闭症幼儿在语言、社交能力、玩耍以及认知方面出现退化。在语言完全发育后经历类似的、原因不明的退化(瓦解性障碍[DD])的儿童则少得多。癫痫或伴有/不伴有临床发作的阵发性脑电图(EEG),包括慢波睡眠期电持续状态(ESES),在少数儿童中可能与选择性语言丧失( Landau-Kleffner综合征[LKS])或广泛性自闭症退化有关。语言和行为缺陷的波动应引起对癫痫的怀疑。文献回顾和作者的经验表明,癫痫在自闭症退化中可能起着相对较小但不可忽视的致病作用。需要开展多学科、可能是多机构的纵向研究,涵盖退化情况,以完善诊断标准并制定更有效的治疗方法。