Smith Michael C, Hoeppner Thomas J
Departmernt of Neurosciences, Rush University Medical Center, Chicago, IL 60612-3833, USA.
J Clin Neurophysiol. 2003 Nov-Dec;20(6):462-72. doi: 10.1097/00004691-200311000-00008.
Landau-Kleffner syndrome (LKS) and the syndrome of continuous spikes and waves during slow wave sleep (CSWS) are two points on the spectrum of functional childhood epileptic encephalopathies. They are characterized by a severe paroxysmal EEG disturbance that may permanently alter the critical synaptogenesis by strengthening synaptic contacts that should have been naturally "pruned." The much more common benign epilepsy with centrotemporal spikes is also related to LKS and CSWS by a common pathophysiology. Although prognosis in LKS and CSWS for seizure control is good, cognitive function declines and permanent neuropsychologic dysfunction is seen in many cases. This permanent damage is most evident in those patients who had early-onset EEG abnormality and a prolonged active phase of continuous spike-and-wave discharges during sleep. If the active phase of paroxysmal activity persists for over 2 to 3 years, even successful treatment does not resolve neuropsychologic sequelae. In LKS, the paroxysmal activity permanently affects the posterior temporal area and results in auditory agnosia and language deficits; in CSWS, the frontal lobes are more involved and other cognitive disturbances predominate. Aggressive treatment should include high-dose antiepileptic drugs, corticosteroids, and surgery in specific cases.
Landau-Kleffner综合征（LKS）和慢波睡眠期持续棘慢波综合征（CSWS）是儿童功能性癫痫性脑病谱系中的两个类型。它们的特征是严重的阵发性脑电图紊乱，这种紊乱可能通过强化本应自然“修剪”的突触联系而永久性地改变关键的突触形成。更为常见的伴有中央颞区棘波的良性癫痫，通过共同的病理生理学机制也与LKS和CSWS相关。尽管LKS和CSWS在控制癫痫发作方面预后良好，但在许多病例中会出现认知功能下降和永久性神经心理功能障碍。这种永久性损伤在那些早期出现脑电图异常且睡眠期间持续棘慢波放电活跃期延长的患者中最为明显。如果阵发性活动的活跃期持续超过2至3年，即使治疗成功也无法解决神经心理后遗症。在LKS中，阵发性活动永久性地影响颞叶后部区域，导致听觉失认和语言缺陷；在CSWS中，额叶受累更明显，其他认知障碍更为突出。积极的治疗应包括高剂量抗癫痫药物、皮质类固醇，以及在特定情况下进行手术。
