Bass N, Wyllie E, Comair Y, Kotagal P, Ruggieri P, Holthausen H
Department of Neurology, Cleveland Clinic Foundation, Ohio 44195-5221, USA.
J Pediatr. 1995 Apr;126(4):537-44. doi: 10.1016/s0022-3476(95)70346-2.
Some types of seizures in children may be difficult to recognize; the diagnosis of seizures arising near the mesial posterior frontal supplementary sensorimotor area (SSMA) may be especially challenging. Such seizures have been well described in adults, but few pediatric cases have been reported even though onset is typically in childhood.
We studied 11 children and adolescents with SSMA seizures diagnosed by prolonged video electroencephalography (EEG).
Mean age at onset was 5.8 years, and diagnosis by video EEG was made at a mean age of 12 years. Children had at least borderline intelligence and few abnormal findings on neurologic examination. Seizures were typically brief, frequent, and predominantly nocturnal, with bilateral tonic posturing, gross proximal limb movements, and preserved consciousness. Magnetic resonance imaging showed normal findings or a superior or mesial posterior frontal tumor or cortical dysplasia. Routine EEG findings were usually normal, but prolonged EEG showed epileptiform discharges over the vertex. Seizure onset in the region of the SSMA was confirmed by subdural EEG in six patients who were studied for epilepsy surgery. Five patients had seizure-free outcome or worthwhile improvement after operation. Two nonoperated patients are seizure-free on antiepileptic medication, and three are undergoing surgical evaluation.
Diagnosis of SSMA seizures in children may be challenging because of unusual symptoms (bilateral tonic limb involvement with preserved consciousness) and frequently normal routine EEG findings. Video EEG may be necessary for diagnosis, and magnetic resonance imaging may reveal a focal lesion. Increased recognition of this seizure type in pediatric patients should lead to earlier diagnosis for optimal medical or surgical treatment.
儿童的某些类型癫痫发作可能难以识别；起源于额叶内侧后辅助运动感觉区（SSMA）附近的癫痫发作的诊断可能尤其具有挑战性。此类癫痫发作在成人中已有详尽描述，但尽管通常在儿童期发病，儿科病例报道却很少。
我们研究了11例经长时间视频脑电图（EEG）诊断为SSMA癫痫发作的儿童和青少年。
发病的平均年龄为5.8岁，视频EEG诊断时的平均年龄为12岁。儿童至少有临界智力，神经系统检查几乎没有异常发现。癫痫发作通常短暂、频繁，且主要在夜间发作，表现为双侧强直性姿势、近端肢体大幅度运动，意识保留。磁共振成像显示正常结果或额叶上或内侧后部肿瘤或皮质发育异常。常规EEG结果通常正常，但长时间EEG显示头顶有癫痫样放电。6例因癫痫手术接受研究的患者经硬膜下EEG证实癫痫发作起源于SSMA区域。5例患者术后无癫痫发作或有显著改善。2例未手术患者服用抗癫痫药物后无癫痫发作，3例正在接受手术评估。
由于症状不寻常（双侧强直性肢体受累且意识保留）且常规EEG结果常正常，儿童SSMA癫痫发作的诊断可能具有挑战性。诊断可能需要视频EEG，磁共振成像可能显示局灶性病变。提高对儿科患者这种癫痫发作类型的认识应能实现更早诊断，以便进行最佳药物或手术治疗。
