Morrell F, Whisler W W, Smith M C, Hoeppner T J, de Toledo-Morrell L, Pierre-Louis S J, Kanner A M, Buelow J M, Ristanovic R, Bergen D
Department of Neurological Science, Rush-Presbyterian-St Luke's Medical Center, Chicago, IL 60612, USA.
Brain. 1995 Dec;118 ( Pt 6):1529-46. doi: 10.1093/brain/118.6.1529.
Landau-Kleffner syndrome (LKS) is an acquired epileptic aphasia occurring in childhood and associated with a generally poor prognosis for recovery of speech. It is thought to be the result of an epileptogenic lesion arising in speech cortex during a critical period of development. Utilizing a new surgical technique designed to eliminate the capacity of cortical tissue to generate seizures while preserving the normal cortical physiological function, we have treated 14 children with aphasia, seizures and a severely abnormal EEG by multiple subpial transection of the epileptogenic cortex. Seven of the 14 patients (50%) have recovered age-appropriate speech, are in regular classes in school and no longer require speech therapy. Four of the 14 (29%) have shown marked improvement, are speaking and understanding verbal instruction but are still receiving speech therapy. Thus, 11 of the 14 (79%), none of whom had used language to communicate for at least 2 years, are now speaking--a rate of sustained improvement considered unusual in this disorder. This study documents the value of a treatment modality not previously used in LKS. Success depends on selection of cases having severe epileptogenic abnormality that can be demonstrated to be unilateral in origin despite a bilateral electrographic manifestation.
Landau-Kleffner综合征（LKS）是一种发生于儿童期的获得性癫痫性失语症，通常言语恢复预后较差。它被认为是在发育关键期言语皮质出现致痫性病变的结果。利用一种新的手术技术，该技术旨在消除皮质组织产生癫痫发作的能力，同时保留正常的皮质生理功能，我们通过对致痫皮质进行多处软膜下横切术，治疗了14名患有失语症、癫痫发作且脑电图严重异常的儿童。14名患者中有7名（50%）恢复了与年龄相符的言语能力，在学校正常上课，不再需要言语治疗。14名中有4名（29%）有明显改善，能说话并理解言语指令，但仍在接受言语治疗。因此，14名中有11名（79%），其中没有人使用语言进行交流至少达2年，现在能够说话了——这种持续改善的比率在这种疾病中被认为是不寻常的。本研究证明了一种以前未在LKS中使用的治疗方式的价值。成功取决于选择具有严重致痫异常的病例，尽管脑电图表现为双侧，但可证明其起源为单侧。
