急性病毒性脑炎并发癫(癎)持续状态的临床研究

目的 探讨急性病毒性脑炎并发癫(癎)持续状态和难治性癫(癎)持续状态的临床特征及预后影响因素.方法 回顾分析26例急性病毒性脑炎并发癫(癎)持续状态患者癫(癎)持续状态出现的时间,以及脑脊液、影像学、脑电图变化特点和预后相关影响因素.结果 26例患者中15例(57.69%)进展为难治性癫(癎)持续状态,与非难治性癫(癎)持续状态患者相比,12例(12/15)于急性病毒性脑炎发病后10d内出现癫(癎)持续状态;14例(14/15)脑脊液压力升高;7例(7/15)急性期呈弥漫性脑水肿;14例(14/15)脑电图检查显示普遍重度异常;7例(7/15)同时应用≥5种抗癫痫药物;15例患者均伴有并发症,其中14例(14/15)需呼吸机辅助呼吸;住院时间明显延长[(446±336)d];5例死亡.6例难治性癫(癎)持续状态患者随访2～10年,2例呈植物状态生存;3例为难治性癫(癎)伴智力减退;1例发作终止,遗留轻度记忆力减退.结论 急性病毒性脑炎是导致癫(癎)持续状态的常见原因,其中近半数患者可进展为难治性癫(癎)持续状态.危险因素包括疾病早期即出现癫(癎)持续状态、脑脊液压力升高、急性期脑水肿、脑电图异常等.难治性癫(癎)持续状态患者病死率高,预后不良.     

癫癎持续状态与脑电图

癫癎持续状态（SE）是神经科常见急症之一，若不能尽早终止发作，可导致神经元永久性损害和患者死亡。除基础病因、年龄等因素外，癫癎持续状态持续时间是影响预后的重要因素。根据癫癎持续状态的实验研究及临床观察发现，癫癎发作持续时间〉30min，即会演变得难以控制。     

癫(癎)持续状态与脑电图

癫(癎)持续状态(SE)是神经科常见急症之一,若不能尽早终止发作,可导致神经元永久性损害和患者死亡[1-4].除基础病因、年龄等因素外,癫(癎)持续状态持续时间是影响预后的重要因素.根据癫(癎)持续状态的实验研究及临床观察发现,癫(癎)发作持续时间>30 min,即会演变得难以控制[5].     

小儿病毒性脑炎合并癫(癎)为持续状态的护理体会

本院自1999-2007年收治38例病毒性脑炎合并癫(癎)持续状态的患儿,现将护理体会介绍如下. 1 临床资料 38名患儿,为本院自1999-2007年收治的诊断为病毒性脑炎合并癫(癎)持续状态(SE)的住院患儿,女14例,男24例,年龄2个月～14岁.其中不典型表现的4例,均为1岁以内的婴儿.     

以难治性癫(癎)持续状态为突出表现的急性坏死性脑炎2例脑组织活检报告

单纯疱疹病毒性脑炎(HSE)又称急性坏死性脑炎,是由单纯疱疹病毒(HSV)所引起的一种急性中枢神经系统感染.未经治疗的HSE病死率高达70%以上.基本病理改变为不对称的脑组织水肿、软化和出血坏死,主要的受累部位为额叶底部、颞叶底部、颞角及枕叶等部位.     

癫(癎)患者出现新发作类型的临床研究

目的 探讨癫(癎)患者新发作类型出现的比例及可能原因,为正确诊断、治疗和预防癫(癎)提供依据.方法 回顾性研究1074例癫(癎)患者的临床资料,分析癫(癎)患者新发作类型的发生率、可能原因、脑电图和神经影像学结果、家族史及神经系统检查.采用独立样本秩和检验比较有或无新发作类型出现的癫(癎)患者的发病年龄及病程差异.X2检验比较有或无新发作类型出现的癫(癎)患者的性别、家族史、神经系统检查以及脑电图和神经影像学出现异常比例的差异.结果 1074例患者中231例(21.5%)出现新的发作类型,其中部分性发作患者584例,132例(22.6%)出现新的发作类型;全面性发作患者490例,99例(20.2%)出现新的发作类型.1074例癫(癎)患者中脑电图异常者576例(53.6%),影像学异常237例(22.1%).231例出现新发作类型的癫(癎)患者中123例(53.2%)出现脑电图异常,75例(32.5%)影像学异常.有41例患者可找到出现新发作类型的可能原因.有和无新发作类型的癫(癎)患者的首次发病年龄差异无统计学意义,平均病程(Z=2.024)、神经系统体格检查和影像学检查结果有异常的比例(χ~2=23.836、18.511)差异均有统计学意义(P＜0.05).结论 一部分出现新发作类型的患者可找到原因,分析新发作类型的出现、原因对癫(癎)的诊断、治疗和预防有指导意义.     

癫癎持续状态的护理体会

我科自2007-01～2009-01共救治癫癎持续状态患者23例，效果理想，现将我们在这方面的护理体会报告如下。 1临床资料 1．1一般资料 本组23例癫癎持续状态患者，男16例，女7例，年龄8～63岁。诱因为停服、漏服或更换抗癫癎药物、发热、劳累。     

非惊厥性癫癎持续状态

非惊厥性癫癎持续状态（NCSE）系临床常见但易忽视的癫癎持续状态（SE）发作类型,据估计,占所有癫癎持续状态的20％～50％。2004年,英国癫癎研究基金会（ERF）将非惊厥性癫癎持续状态定义为：由于持续性癫癎样脑电活动导致的一系列非惊厥临床征象。     

A girl with Cardio-facio-cutaneous syndrome complicated with status epilepticus and acute encephalopathy

We report a six-year-old girl with Cardio-facio-cutaneous (CFC) syndrome who developed acute encephalopathy after the recurrence of status epilepticus. While epileptic encephalopathy and severe epilepsy have been mentioned as frequent complications of the CFC syndrome, no previous reports have shown a case of the CFC syndrome complicated with acute encephalopathy. Here we discuss the possibility for the linkage between the development of acute encephalopathy and CFC syndrome which is generally susceptible to seizures or epilepsy.     

Nonconvulsive status epilepticus

Nonconvulsive status epilepticus (NCSE) is a heterogeneous disorder with multiple subtypes. Although attempts have been made to define and classify this disorder, there is yet no universally accepted definition or classification that encompasses all subtypes or electroclinical scenarios. Developing such a classification scheme is becoming increasingly important, because NCSE is more common than previously thought, with a bimodal peak, in children and the elderly. Recent studies have also shown a high incidence of NCSE in the critically ill. Although strong epidemiological data are lacking, NCSE constitutes about 25-50% of all cases of status epilepticus. For the purposes of this review, we propose an etiological classification for NCSE including NCSE in metabolic disorders, NCSE in coma, NCSE in acute cerebral lesions, and NCSE in those with preexisting epilepsy with or without epileptic encephalopathy. NCSE is still underrecognized, yet potentially fatal if untreated. Diagnosis can be established using an electroencephalogram (EEG) in most cases, sometimes requiring continuous monitoring. However, in comatose patients, diagnosis can be difficult, and the EEG can show a variety of rhythmic or periodic patterns, some of which are of unclear significance. Although some subtypes of NCSE are easily treatable, such as absence status epilepticus, others do not respond well to treatment, and debate exists over how aggressively clinicians should treat NCSE. In particular, the appropriate treatment of NCSE in patients who are critically ill and/or comatose is not well established, and large-scale trials are needed. Overall, further work is needed to better define NCSE, to determine which EEG patterns represent NCSE, and to establish treatment paradigms for different subtypes of NCSE.     

Infections in status epilepticus: A retrospective 5-year cohort study

PurposeStatus epilepticus (SE) has attracted renewed interest lately, and efforts are made to optimize every treatment stage. For refractory SE, optimal supporting care involves mechanical ventilation and intensive care unit (ICU) admission. Infections often complicate SE and recently a single-centre observational study demonstrated an association between infections and poor short-term outcome of SE in a cohort of severely ill patients. We have here attempted to replicate those findings in a different cohort.MethodWe performed a retrospective observational study and included all patients with a diagnosis of SE during 2008–2012 at a Swedish tertiary referral centre.ResultsThe cohort consisted of 103 patients (53% female, 47% male, median age 62 years, range 19–87 years). In house mortality was less than 2 and 70% of the patients’ were discharged home. The most common aetiologies of SE were uncontrolled epilepsy (37%) and brain tumours (16%). A total of 39 patients suffered infections during their stay. Presence of infection was associated with mechanical ventilation (OR 3.344, 95% CI 1.44–7.79) as well as not being discharged home (OR2.705, 95% CI 1.14–6.44), and duration of SE was significantly longer in patients with infection (median 1 day vs. 2.5 days, p < 0.001).ConclusionWe conclude that the previously described association between infections, a longer SE duration, and an unfavourable outcome of SE seems valid also in SE of less severe aetiology.     

以癫痫持续状态起病的桥本脑病1例分析

目的 探讨以癫痫持续状态为首发表现的桥本脑病的临床特征。方法 回顾性分析1例以癫痫持续状态为首发表现的桥本脑病患者的临床资料。结果 临床呈癫痫持续状态,入院时神志不清,间断抽搐伴瞳孔散大,间歇期仍昏迷,余无明显局灶定位体征; 血气pH值6.807,乳酸24 mmol/L,头颅CT、MRI检查均未见异常,2次腰穿脑脊液常规、生化均正常,自身免疫性脑炎抗体8项均阴性,甲状腺功能检测提示轻度甲减,抗甲状腺球蛋白抗体和抗甲状腺过氧化物酶抗体升高,诊断桥本脑病,给予丙种球蛋白及甲强龙冲击治疗后病情迅速并明显好转,用药次日患者神志转清楚,癫痫持续状态得到有效控制。结论 桥本脑病临床以癫痫持续状态为首发者少见,故遇到不明原因的脑病患者,需注意甲状腺功能及相关抗体检查,以尽快明确诊断,尽早治疗,最大程度地改善其预后。     

Safety and efficacy of intravenous valproate in pediatric status epilepticus and acute repetitive seizures

Summary: Purpose: To evaluate the safety and efficacy of intravenous valproate (VPA) loading in children with status epilepticus (SE) or acute repetitive seizures. Methods: Retrospective review was performed on 40 pediatric patients with intravenous VPA loading. Patients were classified into two groups: SE (n = 18) and acute repetitive seizures (n = 22). Thirty‐one patients were VPA naïve and received a full loading dose of 25 mg/kg; nine had subtherapeutic plasma VPA levels and received a partial loading dose. Average infusion rate was 2.8 mg/kg/min. Heart rate and blood pressure were measured before, during, and after infusion. Results: Intravenous VPA loading stopped seizures in 18 patients with SE within 20 min. All 18 patients regained baseline mental status within 1 h of seizure cessation. Among 22 patients with acute repetitive seizures, only one had further seizures after VPA infusion. One patient in the SE group complained of transient tremors. No significant changes in blood pressure or heart rate were found in either group. Postinfusion plasma VPA levels ranged from 51 to 138 μg/ml (mean ± SD = 88 ± 21.5 μg/ml). Conclusions: Intravenous VPA loading is safe and effective for treating acute seizure emergencies in children.     

Convulsive status epilepticus: clinical profile in a developing country

PURPOSE: In developing countries optimal care of status epilepticus (SE) is associated with major barriers, particularly transportation. METHODS: A prospective study of SE was performed between 1994 and 1996 to determine the clinical profile, response to treatment and outcome, Glasgow Outcome Scale (GOS). RESULTS: Of the 85 patients admitted, the mean age was 33 years (8-75 years), 16% <16 years of age. The mean duration of SE before admission was 18.02 h (1-72 h). Only 23 (28%) patients, all locals, presented within <3 h of onset. Etiology included acute symptomatic (54%), remote symptomatic (7%), cryptogenic (19%), and established epilepsy (20%). Central nervous system infections accounted for 24 (28%) of the etiologies. Seventy-five (88%) patients responded to first-line drugs and 10 (12%) required second-line drugs. The mean duration of SE was significantly long in nonresponders (Mean +/- SD: 32.6 +/- 20.11 vs. 15.2 +/- 18.32, p < 0.006). Duration (p < 0.01; OR 1.04, 95% CI 1.01-1.07) and acute symptomatic etiology (p < 0.038; OR 10.38, 95% CI 1.13-95.09) were the independent predictors of no-response to first-line drugs. Of the nine deaths (10.5%), eight were in acute symptomatic group. Predictors of mortality included female sex (p < 0.017, OR 13.41, 95% CI 1.59-115.38) and lack of response to first-line drugs (p < 0.0001, OR 230.27, 95% CI 8.78-6037.19). Longer duration was associated with poor GOS 1-4 (p = 0.001). Of the 37 patients with <6 h, 81% had GOC5 outcome. CONCLUSION: This study suggests that longer duration of SE and acute symptomatic etiology are independent predictors of lack of response to first-line drugs. Failure to respond to first-line drugs and duration predict the outcome.     

Long-term survival in patients with status epilepticus: A tertiary referral center study

Purpose:   To determine long-term survival in patients with status epilepticus (SE).
Methods:   We prospectively followed patients admitted for the first (69.6%) or recursive episode of SE between January 1, 1989 and December 31, 1997 at the Institute of Neurology, Belgrade, Serbia, until death or study termination (December 31, 2006). Data were obtained for cause of death; etiology of SE—acute symptomatic (AS), progressive symptomatic (PS), remote symptomatic (RS), and idiopathic/cryptogenic (I/C); presence of epilepsy; and reoccurrence of SE. Standardized mortality rate (SMR), survival, and regression analysis were used.
Results:   A total of 120 of 750 patients with an episode of SE (15.9%) died in the 30-day period following SE. Data for 207 of 630 (32.8%) surviving patients (35.7% with initial SE) were available at the end of follow-up [median 12 years; 95% confidence interval (CI) 11.1–12.8]. SMR was significantly increased (SMR = 1.81; 95% CI 1.32–2.41). There were 46 deaths (22.2%): 15 of 65 in the AS, 20 of 29 in the PS, 6 of 29 in the RS, and 5 of 75 in the I/C groups. Five-year survival rate was lowest in the PS (45%) compared to AS (91%), RS (87%), and I/C (99%) groups. The following characteristics increased long-term risk for mortality: older age [Exp(B) 1.05, 95% CI 1.029–1.072], PS and AS etiology [Exp(B) 15.6, 95% CI 5.8–41.6; 3.3, 95% CI 1.2–9.1], presence of epilepsy [Exp(B) 2.3, 95% CI 1.2–4.3], and initial SE [Exp(B) 2.4, 95% CI 1.4–4.4].
Discussion:   Approximately one of five patients die within 12 years after an episode of SE. Symptomatic SE (PS and AS), initial SE, age, and presence of epilepsy are associated with long-term increased risk of death.     

Prediction and assessment of acute encephalopathy syndromes immediately after febrile status epilepticus

ObjectiveThis study aimed to predict occurrence of acute encephalopathy syndromes (AES) immediately after febrile status epilepticus in children and to explore the usefulness of electroencephalogram (EEG) in the early diagnosis of AES.MethodsWe reviewed data from 120 children who had febrile status epilepticus lasting >30 min and were admitted to our hospital between 2012 and 2019. AES with reduced diffusion on brain magnetic resonance imaging was diagnosed in 11 of these patients. EEG and serum cytokines were analyzed in AES patients. Clinical symptoms and laboratory data were compared between AES and non-AES patients. Logistic regression analysis was used to identify early predictors of AES.ResultsMultivariate logistic regression identified serum creatinine as a risk factor for developing AES. A scoring model to predict AES in the post-ictal phase that included serum creatinine, sodium, aspartate aminotransferase, and glucose was developed, and a score of 2 or more predicted AES with sensitivity of 90.9% and specificity of 71.6%. Post-ictus EEG revealed non-convulsive status epilepticus in four of the seven AES patients.ConclusionChildren with febrile status epilepticus may be at risk of developing severe AES with reduced diffusion. Post-ictus EEG and laboratory data can predict the occurrence of severe AES.