以癫痫为首发表现的神经白塞病

The clinical features of neuro-Behcet disease with epileptic seizure as the primary symptom

目的：分析以癫痫为首发表现的神经白塞病患者的临床及影像学特点。方法回顾性分析4例以癫痫发作为首要神经系统症状的神经白塞病患者临床及影像学资料。结果1例患者因反复关节疼痛入住皮肤科，1例因视物模糊入住眼科，住院过程中均因发生抽搐会诊后转入神经内科，2例患者因癫痫发作急诊入住神经内科。4例患者头颅CT均未见异常，2例患者头颅MRI示脑干异常加权像信号，2例MRI示侧脑室体旁、半卵圆中心多发对称性病灶，病灶均呈T1加权像低信号，T2加权像、Flair和弥散加权成像（DWI）高信号，但4例患者头颅MRI皮质均未见异常信号。脑脊液检查3例患者细胞数增加，1例患者正常；2例患者脑脊液蛋白水平正常，2例患者增高。视频脑电图1例可见尖、棘波，余3例均未见异常。结论神经白塞病以癫痫发作为首要表现者少见，头颅CT及MRI为诊断神经系统疾病常用的影像学检查方法，头颅MRI未发现致痫灶，但临床均以全面强直-阵挛发作为主要表现，可能与神经白塞病导致皮层异常放电有关。

Objective To analyze the clinical features and image characteristic of neuro-Behcet disease with epileptic seizure as the primary symptom. Methods The data of 4 patients with neuro-Behcet disease with epileptic seizure as the first symptom were collected and retrospectively analyzed. Results One patient had recurrent joint pain in the department of dermatology, and 1 patient had blurred vision in the department of ophthalmology. They were shifted to department of neurology because of epileptic seizure. Head CT of four patients were normal. There were abnormal signal in brainstem in 2 cases, and in the other 2 cases, there were symmetry abnormal signals in periventricle, centrum semiovale. The abnormal signal was low in T1 weighted image and high in T2 weighted image, flair, diffuse weighing imaging , but there were no abnormal signals in cortex in these 4 cases. In CSF examination, 3 patients′cell number was high, 1 patient′s cell number was normal, 2 patients′protein levels were high, and the other 2 patients′protein levels were normal. One patient had spike wave and sharp wave in video-EEG, and the other 3 patients were normal. Conclusions Epileptic seizure is rare in neuro-Behcet disease, and CT and MRI is frequently-used in diagnosing nervous system disease. There are no abnormal signals in cortex, but there are generalized tonic-clonic seizure, which may relate with the abnormal discharge of cortex.