Praline Julien, Hommet Caroline, Barthez Marie-Anne, Brault Florence, Perrier Danièle, Passage Grégoire Du, Lucas Brigitte, Bonnard Julie, Billard Catherine, Toffol Bertrand De, Autret Alain
Neurology Department, University Hospital, Tours Neuropediatric Department, Bicetre Hospital, Le Kremlin Bicetre, France.
Epilepsia. 2003 Nov;44(11):1434-40. doi: 10.1046/j.1528-1157.2003.08403.x.
The aim of this study was to determine the clinical, social, and/or professional and cognitive outcomes in adulthood of the continuous spike-waves during slow sleep (CSWS) and Landau-Kleffner syndromes, which are two rare epileptic syndromes occurring in children.
We enrolled seven young adults, five who had a CSWS syndrome, and two, a Landau-Kleffner syndrome in childhood. We evaluated their intellectual level as well as their oral and written language and executive functions.
This study confirmed that the epilepsy associated with these syndromes has a good prognosis. Only one patient still had active epilepsy. However, the neuropsychological disorders particular to each syndrome persisted. Only two patients had followed a normal pathway in school. Three of the five patients with a CSWS syndrome during childhood remained globally and nonselectively mentally deficient. We found no evidence of the persistence of a dysexecutive syndrome in this study group. The intellectual functions of the two patients with Landau-Kleffner syndrome were normal; however, their everyday lives were disrupted by severe, disabling language disturbances. We discuss the role of some prognostic factors such as the location of the interictal electric focus and the age at onset of CSWS.
These two epileptic syndromes of childhood are very similar in many respects, but their clinical outcomes in adulthood are different.
本研究旨在确定儿童期出现的两种罕见癫痫综合征——慢波睡眠期持续棘慢波（CSWS）综合征和Landau-Kleffner综合征在成年期的临床、社会和/或职业及认知结局。
我们招募了7名年轻成年人，其中5名曾患CSWS综合征，2名儿童期曾患Landau-Kleffner综合征。我们评估了他们的智力水平、口语和书面语言以及执行功能。
本研究证实，与这些综合征相关的癫痫预后良好。只有1例患者仍有活动性癫痫。然而，每种综合征特有的神经心理障碍仍然存在。只有2名患者在学业上遵循了正常路径。5名儿童期患CSWS综合征的患者中有3名总体上存在非选择性智力缺陷。在该研究组中，我们没有发现持续存在执行功能障碍综合征的证据。2名Landau-Kleffner综合征患者的智力功能正常；然而，他们的日常生活因严重的、致残性语言障碍而受到干扰。我们讨论了一些预后因素的作用，如发作间期电灶的位置和CSWS的发病年龄。
这两种儿童癫痫综合征在许多方面非常相似，但它们在成年期的临床结局不同。
