Duda Piotr, Duda Natalia, Kostelecka Katarzyna, Woliński Filip, Góra Joanna, Granat Michał, Bryliński Łukasz, Teresińska Barbara, Karpiński Robert, Czyżewski Wojciech, Baj Jacek
Department of Correct, Clinical and Imaging Anatomy, Medical University of Lublin, ul. Jaczewskiego 4, 20-090 Lublin, Poland.
Department of Forensic Medicine, Medical University of Lublin, Jaczewskiego 8b, 20-090 Lublin, Poland.
J Clin Med. 2024 Dec 13;13(24):7580. doi: 10.3390/jcm13247580.
As speech-related symptoms of Landau-Kleffner syndrome (LKS) are often refractory to pharmacotherapy, and resective surgery is rarely available due to the involvement of the vital cortex, multiple subpial transection (MST) was suggested to improve patient outcome and preserve cortical functions. Here, we analyze the reports about MST use in LKS, regarding its impact on seizures, language, behavior, EEG, cognition, and reported adverse effects. In conditions like LKS, surgery is not a popular treatment option and presumably should be considered sooner. Candidates for MST should be selected carefully, optimally with the unilateral onset of epileptic activity. Laterality can be assessed using a methohexital suppression test (MHXT), electrical intracarotid amobarbital test, or magnetoencephalography. After MST, a significant percentage of LKS patients present seizure-free status, normalization of EEG patterns, and rapid behavior improvement. Data comprising language outcomes are mixed, with improvement reported in 23.8-100% of cases, and no superiority was found in the only study comparing MST with a non-surgical group. Cognitive outcomes are not well described. The risk linked to MST is described as low, with cerebral edema and transient neurological deficits being the most common complications. MST successfully improves seizure, EEG, and behavioral outcomes in LKS patients. However, its beneficial impact on language and cognition is not well proven. It is generally a safe neurological operation.
由于Landau-Kleffner综合征（LKS）的言语相关症状通常对药物治疗无效，且由于重要皮质受累很少能进行切除性手术，因此有人建议采用多处软膜下横切术（MST）来改善患者预后并保留皮质功能。在此，我们分析了关于MST用于LKS的报告，涉及它对癫痫发作、语言、行为、脑电图、认知以及报告的不良反应的影响。在LKS这样的病症中，手术并非常用的治疗选择，大概应尽早考虑。MST的候选者应仔细挑选，最好是癫痫活动单侧起病的患者。可使用美索比妥抑制试验（MHXT）、颈动脉内注射阿米妥试验或脑磁图来评估脑半球优势。MST术后，相当比例的LKS患者呈现无癫痫发作状态、脑电图模式正常化以及行为迅速改善。关于语言结果的数据不一，报告显示23.8%至100%的病例有改善，且在唯一一项比较MST与非手术组的研究中未发现MST有优势。认知结果描述得并不充分。与MST相关的风险被描述为较低，脑水肿和短暂性神经功能缺损是最常见的并发症。MST成功改善了LKS患者的癫痫发作、脑电图和行为结果。然而，其对语言和认知的有益影响尚未得到充分证实。它总体上是一种安全的神经外科手术。
