Shevell M I, Rosenblatt B, Watters G V, O'Gorman A M, Montes J L
Division of Pediatric Neurology, Montreal Children's Hospital, Quebec, Canada.
Pediatr Neurol. 1996 Jan;14(1):31-5. doi: 10.1016/0887-8994(95)00252-9.
Benign epilepsy of childhood with rolandic spikes (BECRS) is an electroclinical entity that is the most common primary partial epilepsy syndrome of childhood. Typically presenting between the ages of 3 and 13 years, it is characterized by a well-recognized seizure pattern arising in a normal child with EEG findings restricted to rolandic/centrotemporal regions. Seizure control is usually easily achieved and prognosis is believed to be uniformly good. Some authors have suggested that individuals fitting the electroclinical parameters of this entity need not undergo neuroimaging due to the benign evolution of this disorder. Five patients presenting over a 13-year period with peribuccal seizures, normal neurologic examinations, and EEG data initially suggestive of BECRS found to have focal lesions on neuroimaging are summarized. Independent bilateral centrotemporal epileptiform abnormalities were seen in 3 patients. Imaging studies (CT, MRI, or both) documented a mass lesion in all in variable locations. Histologic examination documented a low-grade astrocytoma in 3 patients and a cavernous angioma in another. The fifth patient refused treatment or biopsy. Careful retrospective review of the clinical features of these patients reveals variable atypical features in each. Therefore, despite an electroclinical phenotype initially suggestive of the BECRS presentation, the presence of atypical clinical features raises the possibility of an underlying structural lesion and thus a negative neuroimaging study may in some patients be essential to the definitive accurate diagnosis of BECRS.
儿童罗兰多棘波良性癫痫(BECRS)是一种电临床实体,是儿童最常见的原发性局灶性癫痫综合征。通常在3至13岁之间发病,其特征是在正常儿童中出现一种公认的发作模式,脑电图表现局限于罗兰多/中央颞区。癫痫控制通常很容易实现,且预后普遍良好。一些作者认为,符合该实体电临床参数的个体由于该疾病的良性发展,无需进行神经影像学检查。本文总结了13年间出现的5例患者,这些患者有口周发作、神经系统检查正常,脑电图数据最初提示为BECRS,但神经影像学检查发现有局灶性病变。3例患者可见独立的双侧中央颞区癫痫样异常。影像学检查(CT、MRI或两者)在所有患者中均记录到不同部位的占位性病变。组织学检查证实3例患者为低级别星形细胞瘤,另1例为海绵状血管瘤。第五例患者拒绝治疗或活检。对这些患者临床特征的仔细回顾性分析发现,每例患者都有不同的非典型特征。因此,尽管电临床表型最初提示为BECRS表现,但非典型临床特征的存在增加了潜在结构性病变的可能性,因此在某些患者中,神经影像学检查结果为阴性对于准确诊断BECRS可能至关重要。