Verrotti A, Domizio S, Sabatino G, Morgese G
Department of Pediatrics, University of Chieti, Italy.
Childs Nerv Syst. 1996 Sep;12(9):530-3. doi: 10.1007/BF00261606.
The authors describe the electroclinical aspects and long-term evolution of five (in one male and four female patients) cases of early infantile epileptic encephalopathy in the neonatal period, the most common seizures were tonic spasms (either generalized or lateralized) that, very often, occurred in series. All newborns showed the typical suppression-burst pattern. The neurological status (initially normal) progressively deteriorated in the subsequent months, and all infants suffered from severe mental insufficiency and daily intractable seizures. Four out of the five children developed hypsarrythmia and one, Lennox-Gastaut syndrome. The authors discuss the main problems related to the suppression-burst pattern and the long-term prognosis of this abnormality.
作者描述了5例(1例男性和4例女性患者)新生儿期早发性婴儿癫痫性脑病的电临床特征及长期演变情况,最常见的发作类型为强直性痉挛(全身性或局限性),且常常连续发作。所有新生儿均表现出典型的抑制-爆发模式。神经状态(最初正常)在随后几个月逐渐恶化,所有婴儿均患有严重智力缺陷及每日难治性癫痫发作。5名儿童中有4名出现高峰节律紊乱,1名出现Lennox-Gastaut综合征。作者讨论了与抑制-爆发模式相关的主要问题以及这种异常情况的长期预后。