Grote C L, Van Slyke P, Hoeppner J A
Department of Psychology, Rush-Presbyterian-St Luke's Medical Center, Chicago, Illinois 60612, USA.
Brain. 1999 Mar;122 ( Pt 3):561-6. doi: 10.1093/brain/122.3.561.
Landau-Kleffner syndrome is an acquired epileptic aphasia occurring in normal children who lose previously acquired speech and language abilities. Although some children recover some of these abilities, many children with Landau-Kleffner syndrome have significant language impairments that persist. Multiple subpial transection is a surgical technique that has been proposed as an appropriate treatment for Landau-Kleffner syndrome in that it is designed to eliminate the capacity of cortical tissue to generate seizures or subclinical epileptiform activity, while preserving the cortical functions subserved by that tissue. We report on the speech and language outcome of 14 children who underwent multiple subpial transection for treatment of Landau-Kleffner syndrome. Eleven children demonstrated significant postoperative improvement on measures of receptive or expressive vocabulary. Results indicate that early diagnosis and treatment optimize outcome, and that gains in language function are most likely to be seen years, rather than months, after surgery. Since an appropriate control group was not available, and that the best predictor of postoperative improvements in language function was that of length of time since surgery, these data might best be used as a benchmark against other Landau-Kleffner syndrome outcome studies. We conclude that multiple subpial transection may be useful in allowing for a restoration of speech and language abilities in children diagnosed with Landau-Kleffner syndrome.
Landau-Kleffner综合征是一种获得性癫痫性失语症，发生于原本语言能力正常的儿童，这些儿童会丧失先前已获得的言语和语言能力。尽管一些儿童恢复了部分此类能力，但许多患有Landau-Kleffner综合征的儿童仍存在持续的严重语言障碍。多处软膜下横切术是一种外科技术，已被提议作为治疗Landau-Kleffner综合征的合适方法，因为该技术旨在消除皮质组织产生癫痫发作或亚临床癫痫样活动的能力，同时保留该组织所维持的皮质功能。我们报告了14名接受多处软膜下横切术治疗Landau-Kleffner综合征的儿童的言语和语言结果。11名儿童在接受性或表达性词汇量测量方面术后有显著改善。结果表明，早期诊断和治疗可优化预后，且语言功能的改善最有可能在术后数年而非数月出现。由于没有合适的对照组，且术后语言功能改善的最佳预测指标是手术时间长短，这些数据最好用作与其他Landau-Kleffner综合征预后研究对比的基准。我们得出结论，多处软膜下横切术可能有助于恢复被诊断为Landau-Kleffner综合征儿童的言语和语言能力。
