35例Kojewnikow综合征临床-脑电-影像特征与影响预后的相关因素分析

发布时间：2018-05-26 16:37

本文选题：癫痫持续状态(SE) + 连续部分性癫痫持续状态(EPC)　；参考：《吉林大学》2017年硕士论文

【摘要】：癫痫持续状态(SE)是神经内科最为常见的危急重症,随着大家对癫痫的认识的加深,癫痫持续状态的定义也在不断完善中,2001年国际抗癫痫联盟提出了新的癫痫持续状态的定义:“一次癫痫发作(包括各种类型癫痫发作)持续时间大大超过大多数这种发作类型患者的发作时间,发作仍没有停止的临床征象,或反复的癫痫发作,在发作间期中枢神经系统的功能没有恢复到正常基线状态”。持续的癫痫发作常合并感染、电解质紊乱、酸碱平衡失调、多器官功能衰竭,导致难治性癫痫的发生,增加患者致残率和死亡率。全面性癫痫持续状态已得到临床医生高度重视,但对局灶性癫痫持续状态认识还不够深入,一方面导致该疾病在早期没有得到及时诊断和积极治疗,患者常常留下严重的神经功能障碍,如癫痫发作后神经功能缺损(PEND);另一方面也可能存在过治疗,导致呼吸抑制等并发症。其中,连续部分性癫痫持续状态(epilepsia partialis continua,EPC),又称Kojewnikow综合征(KS),作为局灶性癫痫持续状态的一种亚型越来越受到人们的关注。目的:分析总结Kojewnikow综合征患者临床、脑电、影像学特征,分析影响预后的相关因素,为Kojewnikow综合征的预防、诊断、治疗提供经验。方法:回顾性分析于2013年9月1至2017年2月1日期间于我院(吉林大学中日联谊医院)就诊的门诊及住院的局灶性癫痫持续状态病例,排除资料极不完善的病历,进一步结合其临床症状及脑电图改变,将符合2001年癫痫诊断及分类标准,且符合Thomas[1]提出的KS两大特点的病例纳入研究。详细阅读病历资料,对部分患者进行电话跟踪随访,分别记录患者的性别、发病年龄、病因、诱因、发作累及部位、发作时合并症状、脑电图改变、影像学检查、一次发作持续时间、发作控制时间、治疗方法、抗癫痫药物及预后情况,总结KS的临床、影像、脑电图特征,并分析发病年龄、病因、诱因、脑电图、影像学特征、意识状态、一次发作持续时间、发作控制时间、发作频率特点及抗癫痫治疗对KS预后的影响。结果:1、临床特征:男女比例为1.69:1,平均年龄(51.9±21.55)岁。以41-60岁中年组及大于60岁老年组最常见,各占37.1%;病因以急性症状性及慢性症状性最常见,各占了34.3%;感染及不规律应用抗癫痫药物是KS最常见的诱因;最常见的累及部位是面部、上肢及面部,各占20%,累及近端肢体多于远端肢体,左侧多于右侧。发作期间9例(25.7%)合并其他类型癫痫发作;23例(65.7%)合并不同程度的意识障碍,其余12例(34.3%)发作期间意识清楚;26例(74.3%)表现为持续发作,9例(25.7%)表现为丛集发作。2、脑电图特征:发作间期脑电图:30例(85.7%)表现异常,其中10例(28.6%)为双侧异常,12例(34.3%)为右侧异常,8例(22.6%)为左侧异常,异常脑电图主要以慢波为主(57.1%),局灶性慢波多见;部分行发作期脑电图,以肌阵挛对侧出现周期性癫痫样放电(PLEDs)常见。3、影像学特征:影像检查异常者31例(88.6%),正常4例(11.4%),其中仅皮层异常者23例(65.7%),主要为额、顶皮层异常,仅皮下结构异常者3例(8.6%),皮层及皮下结构均异常者5例(14.3%)。右侧异常更常见。对比临床表现、脑电图,60%具有相关性影像学改变,以额、顶叶皮质受累最常见。4、治疗及预后:大部分患者发作期应用抗癫痫药物,小部分病例自行好转或去除病因后好转,22例(62.9%)患者联合2种以上的抗癫痫药物,其中最常见丙戊酸钠静脉给药,其次是苯巴比妥肌注;除2例死亡及1例出院外,10例(31.2%)合并PEND,最常见的PEND是TODD麻痹。5、影响预后的相关因素分析:性别、发病年龄、病因、脑电图、影像学检查、一次发作持续时间、发作频率特点、抗癫痫治疗与是否合并PEND无关(P0.05),意识状态、发作控制时间与是否合并PEND相关(P0.05)。结论:1、KS易发生于中老年癫痫患者中,感染及不规律应用抗癫痫药物是KS最常见的诱因,面部、上肢最常累及。额、顶叶皮层异常是KS危险因素,发作间期脑电图改变以局灶性慢波最常见,提示脑局部功能异常。2、KS治疗中大多数患者需要联合2种以上抗癫痫药物。部分KS遗留PEND,最常见的是TODD麻痹。3、意识状态、发作控制时间可能影响预后,积极控制KS发作是改善预后的关键。
[Abstract]:The status epilepticus (SE) is the most common critical critical in the Department of Neurology. With the deepening of understanding of epilepsy, the definition of the status of epilepsy is constantly improved. In 2001, the international antiepileptic alliance proposed a new definition of the status of the status of epilepsy: "a seizure (including various types of epileptic seizures) has a great duration of duration. In most cases of this type of attack, the seizures still have no stopping clinical signs, or repeated seizures, and the function of the central nervous system is not restored to normal baseline in the interval of the seizure. Persistent seizures often merge infection, electrolyte disorder, acid-base balance disorder, multiple organ failure, resulting in difficult treatment. The occurrence of sexual epilepsy increases the rate of disability and mortality. The status of comprehensive epilepsy has been highly valued by clinicians, but the understanding of the persistent state of focal epilepsy is not enough. On the one hand, the disease has not been diagnosed and actively treated in the early stage, and the patients often leave serious neurological dysfunction, such as epilepsy. The post seizure nerve function defect (PEND), on the other hand, may also lead to complications such as respiratory depression, such as the continuous partial status epilepticus (epilepsia partialis continua, EPC), and Kojewnikow syndrome (KS). A subtype of focal status epilepticus is becoming more and more concerned. Summarize the clinical, electroencephalogram and imaging features of Kojewnikow syndrome patients, analyze the related factors affecting the prognosis, and provide experience for the prevention, diagnosis and treatment of Kojewnikow syndrome. Methods: retrospective analysis of the focal epilepsy in the outpatient and hospitalized patients in our hospital (Jilin University Sino Japan Friendship Hospital) from 1 to February 1, 2017 September 2013. Persistent cases, excluding the very incomplete data, further combined with the clinical symptoms and electroencephalogram changes, will conform to the 2001 epileptic diagnosis and classification standards, and conform to the Thomas[1] KS two characteristics of the case included in the study. Read the medical records in detail, follow up the patients by telephone follow-up and record the patient's sex, respectively. Age, cause, cause, cause, part of attack, combined symptoms, electroencephalogram change, imaging examination, one attack duration, time of seizure control, treatment, antiepileptic drugs and prognosis, summarize the clinical, imaging, Electroencephalogram Characteristics of KS, and analyze the age, cause, inducement, electroencephalogram, imaging features and meaning of the onset of disease. Status, duration of one attack, time of seizure control, frequency characteristics of seizures and the effect of antiepileptic therapy on KS prognosis. Results: 1, clinical features: the proportion of men and women was 1.69:1, the average age was (51.9 + 21.55) years, the most common in the 41-60 year old group and the older group older than 60 years, each accounted for 37.1%; the cause of the cause was acute symptomatic and chronic symptomatic most frequent. See, each accounted for 34.3%; infection and irregular use of antiepileptic drugs was the most common cause of KS; the most common areas involved were facial, upper and face, each accounted for 20%, involving the proximal extremities more than the distal extremities, and the left side more than the right. 9 cases (25.7%) combined with other types of epileptic seizures during the seizure; 23 cases (65.7%) combined different degrees of consciousness disorder, The other 12 cases (34.3%) had a clear consciousness during the seizure; 26 (74.3%) showed continuous seizures, 9 (25.7%) showed cluster attack.2, electroencephalogram (EEG) characteristics: interictal electroencephalogram (EEG): 30 cases (85.7%) were abnormal, 10 (28.6%) was bilateral abnormality, 12 (34.3%) was right abnormality, 8 cases (22.6%) were left abnormality, and abnormal electroencephalogram mainly was slow wave. Main (57.1%), focal slow waves were most common; part of the episodes of electroencephalogram (PLEDs) was common.3 in the contralateral myoclonus, imaging features: 31 cases (88.6%) with abnormal imaging examination (88.6%) and 4 normal cases (11.4%), of which there were only 23 cases (65.7%) with only cortical abnormalities, and 3 cases (8.6%), only hypodermic structures (8.6%), and skin. There were 5 cases (14.3%) with abnormal layer and subcutaneous structure. Abnormal right side was more common. Comparison of clinical manifestations, electroencephalogram, 60% associated imaging changes, the most common.4, treatment and prognosis in frontal cortex and parietal cortex: most of the patients were treated with antiepileptic drugs, small cases improved or removed the cause, and 22 cases (62.9%) were combined. 2 or more antiepileptic drugs, the most common valproate intravenous drug, followed by phenobarbital intramuscular injection; 2 cases of death and 1 cases of discharge, 10 cases (31.2%) combined with PEND, the most common PEND is TODD paralysis.5, affecting the prognosis of related factors analysis: sex, age, etiology, electroencephalogram, imaging examination, one attack duration, The characteristics of attack frequency, anti epileptic treatment and whether or not combining PEND (P0.05), consciousness state, seizure control time and PEND associated with (P0.05). Conclusion: 1, KS occurs easily in middle-aged and elderly epileptic patients, infection and irregular application of antiepileptic drugs are the most common cause of KS, the most frequently involved in the face, upper extremity. The abnormal of the parietal cortex is the KS danger. Risk factors, electroencephalogram changes in interictal period are the most common focal slow wave, suggesting that the local function of brain is abnormal.2, most of the patients in the KS treatment need to combine more than 2 antiepileptic drugs. Part of the KS remains PEND, the most common is the TODD paralytic.3, the state of consciousness, the seizure control time may affect the prognosis, and active control of KS seizures is the key to improving prognosis. Key.
【学位授予单位】：吉林大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R742.1

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