6例儿童持续性部分性癫癎患者的病因学及临床分析

目的 探讨儿童持续性部分性癫癎（EPC）患者的病因学及临床特点，促进对EPC的认识。方法 回顾性分析6例有EPC发作的儿童癫癎患者的临床特点及诊疗过程，并比较不同病因所致患者的临床及实验室特点和预后差异。结果 6例患儿中男1例、女5例，起病年龄在1岁7个月至9岁，2例诊断为Rasmussen脑炎（RE），1例为局灶性皮层发育不良（FCD），1例为POLG基因突变所致Alpers综合征，1例为Angelman综合征（AS），1例为结核性脑膜炎，后两例患者EPC发作分别有癫癎持续状态所致急性脑病和颅脑手术的诱因，余4例患儿EPC发作为自然病程进展。所有患者具有除EPC以外的局灶性癫癎发作，症状学包括自动症、双侧非对称性强直、偏转、复杂运动、植物神经症状等，部分伴有意识障碍。EPC持续时间为数天或数月。所有患儿头颅磁共振（MRI）均有异常发现，包括局部异常信号、皮层肿胀、弥漫或一侧脑萎缩、局部皮层增厚、层状坏死等。3例患者行头颅PET/CT扫描并发现局部高代谢或高代谢与低代谢并存。所有患儿脑电图（EEG）均有异常，异常电活动分布分别为脑区性、半球性或弥漫性，EPC发作时EEG改变部分可识别、部分不易辨认。所有患者EPC发作对于抗癫癎药物治疗均不敏感，AS患儿的EPC相对自限；FCD患儿行癫癎病灶切除术，术后发作控制，未遗留神经功能障碍；1例诊断为RE的患儿，行功能性大脑半球切除术，术后无发作，遗留明显功能障碍。Alpers综合征患者预后最差。结论 EPC是特殊癫癎发作类型，儿童患者的病因以免疫炎症及代谢性病因为主，治疗方案的选择、疗效及预后取决于病因。

Etiology and clinical features of epilepsia partialis continua: an analysis of six cases

Objective To investigate the etiology and clinical features of epilepsia partialis continua (EPC) in children.Methods A retrospective analysis was performed for the clinical features, diagnosis and treatment of six children with EPC, and the clinical and laboratory features and prognosis were compared between the children with different etiologies.Results There were five girls and one boy, with an onset age ranging from one year and seven months to nine years. Two were diagnosed with Rasmussen encephalitis, one was diagnosed with focal cortical dysplasia, one was diagnosed with Alpers syndrome caused by POLG gene mutation, one was diagnosed with Angelman syndrome, and one was diagnosed with tuberculous meningitis. The latter two children had the predisposing factors for acute encephalopathy induced by status epilepticus and craniocerebral operation during the onset of EPC, while the other four children had natural progression of EPC. All the children had focal seizures except EPC, and symptoms included automatism, bilateral asymmetric tonic seizure, deflection, complex motor, and autonomic symptoms, with disturbance of consciousness in some children. EPC often lasted for several days or even several months. All children had abnormalities on head MRI, including local abnormal signal, cortex swelling, diffusive brain atrophy or brain atrophy at one side, local cortex thickening, and cortical necrosis. Head PET/CT scan was performed for three children and found local hypermetabolism or co-existence of hypermetabolism and hypometabolism. All the children had abnormalities on electroencephalography (EEG), with cerebral, hemispheric, or diffusive distribution of abnormal electrical activities, and during the onset of EPC, some EEG changes were recognizable and some were difficult to identify. All the children with EPC were not sensitive to antiepileptic drugs. EPC was relatively self-limiting in the child with Angelman syndrome. The child with focal cortical dysplasia underwent resection of epileptic foci and had good postoperative control, without neurological dysfunction. The child with Rasmussen encephalitis underwent functional hemispherectomy and had no attack after surgery, with neurological dysfunction. The child with Alpers syndrome had the worst prognosis.Conclusions EPC is a special type of epileptic seizures. Immune inflammation and metabolic etiologies are the main causes of EPC in children, and the selection of treatment regimens, treatment outcome, and prognosis depend on etiology.