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目的探讨家族性颞叶癫痫(FTLE)的临床和脑电图(EEG)特点。方法收集6个 FTLE的家系资料,通过详细的调查,建立较完善的家系谱,并对受累者的临床资料、EEG进行分析总结。结果6个家系共78名家族成员,其中受累者20例,每个家系2-6例受累者不等,2代发病居多。发病年龄0．5-27岁,平均(13．7±10．5)岁。平均发作频率(6．7±8．9)次/月。表现为复杂部分性发作13 例次(76．5％),继发全身强直一阵挛性发作12例次(70．6％),单纯部分性发作4例次(23．5％),强直发作和全身强直一阵挛性发作各1例次;无法分类3例。20例受累者中3例出现复杂视幻觉,1例出现听幻觉。受累者中13例进行发作间期EEG检查,仅1例示颞叶局灶痫性放电(7．7％),2例痫性异常放电, 余未见明显异常;10例进行MRI检查,仅1例示右额颞发育异常(10．0％)。18例受累者疗效观察,3例发作自然缓解,另15例接受抗癫痫药物(AEDs)治疗,14例有效(4例发作控制),仅1例无效。结论 FTLE家庭受累者临床症状呈多样性,发作间期EEG大部分未见局灶异常放电,MRI检查未见异常。 FTLE的正确诊断主要依据患者临床发作特征和家系谱,AEDs治疗疗效良好。
Objective To investigate the clinical and electroencephalographic (EEG) features of familial temporal lobe epilepsy (FTLE). Methods Six pedigree data of FTLE were collected. Based on detailed investigation, a more complete pedigree was established, and the clinical data and EEG of affected persons were analyzed and summarized. Results A total of 78 family members were found in 6 pedigrees, of which 20 were affected, ranging from 2-6 cases in each pedigree. The incidence of the second generation was mostly. Age of onset 0.5-27 years old, with an average (13.7 ± 10.5) years. The average seizure frequency (6.7 ± 8.9) times / month. 13 cases (76.5%) had complex partial seizures, 12 cases (70.6%) had tetanic tetanus secondary to tetanic episodes, 4 cases (23.5%) had partial partial seizures, And generalized tonic-clonic seizures in 1 case; can not be classified in 3 cases. In 20 cases, 3 cases showed complicated visual hallucinations and 1 case had auditory hallucinations. Of the 13 affected patients, EEG was performed in the interictal period. Only 1 patient showed epileptic discharge in the temporal lobe (7.7%) and 2 patients showed abnormal epileptiform discharge. Right frontotemporal development abnormalities (10.0%). Eighteen patients were treated with euthyroidism, three patients with spontaneous remission, another 15 with antiepileptic drugs (AEDs), 14 were effective (4 with seizure control) and only 1 patient was ineffective. Conclusion The clinical symptoms of FTLE family were diverse. Most of the EEG during the interictal period showed no abnormal electric discharge, and MRI showed no abnormality. The correct diagnosis of FTLE mainly based on clinical characteristics and pedigree patients, AEDs treatment of good effect.