Department of Neurology, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Buenos Aires, Argentina.
Department of Neurology, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Buenos Aires, Argentina.
Seizure. 2014 Feb;23(2):98-104. doi: 10.1016/j.seizure.2013.09.016. Epub 2013 Oct 8.
The aim of the study was to retrospectively analyze the electroclinical features, etiology, treatment, and prognosis of 29 patients with Landau-Kleffner syndrome (LKS) with a long-term follow-up.
Inclusion criteria were a diagnosis of LKS with: (1) acquired aphasia or verbal auditory aphasia; (2) with or without focal seizures, secondarily generalized tonic-clonic seizures, absences, or atonic seizures.
Mean follow-up was 12 years. All cases except six had seizures. Before the onset of aphasia, developmental language and behavioral disturbances were present in 19 and 14 patients, respectively. All patients had verbal auditory agnosia. Aphasia was severe in 24 patients and moderate in five. Nonlinguistic cognitive dysfunctions were moderate in 14 patients. Behavioral disturbances were found in 16 patients. During the continuous spike-and-wave discharges during slow sleep phase, the spike-wave index was >85% in 15, 50-85% in eight, and 30-50% in four. In two patients, the EEG recording showed occasional bilateral spikes, without continuous spike-and-wave discharges during slow sleep. In this phase, the awake EEG recording showed more frequent interictal abnormalities, predominantly in the temporal regions. Eight patients recovered language completely, but the remaining patients continue to have language deficits of different degrees.
Landau-Kleffner syndrome is an epileptic encephalopathy characterized by acquired verbal auditory aphasia and seizures in most of the patients associated with continuous or almost continuous spike-and-wave discharges during slow wave sleep. The most commonly used treatments were clobazam, ethosuximide, sulthiame. High-dose steroids were also administered. Adequate and early management may avoid language and cognitive deterioration.
本研究旨在回顾性分析 29 例具有长期随访的 Landau-Kleffner 综合征（LKS）患者的临床电生理特征、病因、治疗和预后。
纳入标准为诊断为 LKS 并符合以下条件：（1）获得性失语或言语听觉性失语；（2）伴有或不伴有局灶性发作、继发性全面强直阵挛发作、失神发作或弛缓性发作。
平均随访时间为 12 年。除 6 例外，所有病例均有癫痫发作。在失语症发作前，19 例患者存在发育性语言和行为障碍，14 例患者存在语言和行为障碍。所有患者均存在言语听觉性失认症。24 例患者失语症严重，5 例患者中度。14 例患者存在非语言认知功能障碍。16 例患者存在行为障碍。在慢波睡眠期间棘慢波放电时，棘波指数＞85%的患者有 15 例，50%-85%的患者有 8 例，30%-50%的患者有 4 例。在 2 例患者中，脑电图记录显示偶尔出现双侧棘波，而在慢波睡眠期间无连续棘慢波放电。在这一阶段，清醒脑电图记录显示更频繁的发作间期异常，主要位于颞叶区域。8 例患者完全恢复语言功能，但其余患者继续存在不同程度的语言缺陷。
Landau-Kleffner 综合征是一种癫痫性脑病，其特征为获得性言语听觉性失语和大多数患者的癫痫发作，伴有慢波睡眠期间连续或几乎连续的棘慢波放电。最常使用的治疗药物为氯巴占、乙琥胺、硫喷妥钠。也给予了大剂量类固醇。充分和早期的治疗可以避免语言和认知功能恶化。
