Otsubo H, Chitoku S, Ochi A, Jay V, Rutka J T, Smith M L, Elliott I M, Snead O C
Department of Paediatrics, Division of Neurology, The Hospital for Sick Children and University of Toronto, Ontario, Canada.
Neurology. 2001 Aug 28;57(4):590-6. doi: 10.1212/wnl.57.4.590.
To describe the diagnosis, treatment, and outcomes in children with malignant rolandic-sylvian epilepsy (MRSE), defined as a form of epilepsy characterized by sensorimotor seizures, medical refractoriness, normal MRI, frontocentrotemporal EEG spikes, rolandic-sylvian spike sources on magnetoencephalography (MEG), and cognitive problems.
A retrospective chart analysis of seven patients who had shown these characteristics and undergone extensive diagnostic testing, including MEG and intracranial video-EEG was performed.
Interictal scalp EEG spikes were seen over the frontocentrotemporal regions bilaterally (6) and unilaterally (1). MEG showed spike sources in the perisylvian region in two patients (both bilateral) and in the perirolandic fissure in five (two bilateral). Three patients required bilateral subdural strips to lateralize seizures before electrocorticography. Final electrocorticograms showed an ictal onset zone around the rolandic (four cases) and rolandic-sylvian regions (three cases). Six patients showed neuropsychological deficits. After cortical excision and multiple subpial transection, three were seizure free and four had seizures rarely (30 months' mean follow-up). No child had a permanent deficit in sensorimotor or cognitive functions, although two showed exacerbation of preexisting attentional deficits. Tissue analysis showed definite evidence of neuronal migration disorders (3) and gliosis (2).
MEG was helpful for localizing both malignant rolandic-sylvian neuronal activities and functional cortex. Successive neuropsychological assessments are necessary to detect cognitive deterioration and indicate remedial programming. If, after careful observation over at least 5 years, surgery is considered to control refractory seizures, intracranial video-EEG is needed to localize the epileptogenic zone.
描述恶性罗兰-西尔维安癫痫（MRSE）患儿的诊断、治疗及预后情况，MRSE被定义为一种以感觉运动性发作、药物难治性、MRI正常、额中央颞区脑电图尖波、脑磁图（MEG）显示罗兰-西尔维安尖波源以及认知问题为特征的癫痫形式。
对7例具有这些特征并接受了包括MEG和颅内视频脑电图在内的广泛诊断检查的患者进行回顾性病历分析。
双侧（6例）和单侧（1例）额中央颞区可见发作间期头皮脑电图尖波。MEG显示2例患者（均为双侧）的尖波源位于外侧裂周围，5例（2例双侧）位于中央沟周围。3例患者在进行皮质脑电图检查前需要双侧硬膜下条形电极来确定癫痫发作的侧别。最终的皮质脑电图显示发作起始区位于中央沟（4例）和中央沟-外侧裂区域（3例）。6例患者存在神经心理学缺陷。在进行皮质切除和多次软膜下横切术后，3例无癫痫发作，4例很少发作（平均随访30个月）。尽管有2例患者原有注意力缺陷加重，但没有患儿出现感觉运动或认知功能的永久性缺陷。组织分析显示有明确的神经元迁移障碍（3例）和胶质增生（2例）的证据。
MEG有助于定位恶性罗兰-西尔维安神经元活动和功能皮层。需要进行连续的神经心理学评估以检测认知恶化并指导补救性干预计划。如果在至少5年的仔细观察后考虑进行手术以控制难治性癫痫发作，则需要颅内视频脑电图来定位致痫区。
