伴有睡眠中电持续状态癫痫患儿的临床表现及脑电图特点

目的探讨伴有睡眠中癫痫性电持续状态(ESES)的不同癫痫综合征患儿的临床表现及脑电图特点。方法通过长程脑电图监测,对9例合并ESES的癫痫患儿进行神经心理测试、临床资料及脑电图资料的回顾性分析。结果 9例合并ESES的癫痫患儿中,伴中央颞区棘波的儿童良性癫痫5例,韦氏儿童智力量表测定示,无言语及精神发育倒退,药物治疗效果较好;不典型儿童良性部分性癫痫2例,韦氏量表示,认知功能轻中度受损,药物治疗效果欠佳;Lennox-Gastaut综合征2例,均有多种形式的癫痫发作,精神运动发育落后,未能配合韦氏测定,多种抗癫痫药物联合应用无效。结论 ESES不是一个独立的癫痫综合征,而是包括了一系列不同病因、不同临床表现及不同预后的多种癫痫综合征。ESES,是引起神经心理损伤的主要原因,早期识别这种特殊的脑电图现象,早期干预,是改善神经心理损伤的关键。     

外伤性脑静脉窦血栓形成

目的 探讨外伤性颅内静脉窦血栓形成（CVST）的特点及治疗策略。方法 对11例外伤所致CVST的诊断、治疗进行回顾性分析。结果 11例CVST患者4例继发于颅骨未骨折的闭合性脑损伤，7例继发于颅骨线形骨折；以头部胀痛、视力下降、癫痫发作为其主要临床表现；头颅MRI及MRV和脑血管造影检查为该病提供确诊依据；11例患者中4例行抗凝治疗，7例行血管内治疗；治疗后6例患者痊愈，4例好转，1例无效，无严重并发症发生。本组中9例病人接受门诊和电话随访6个月至3年，疗效稳定，无一例复发。结论 颅脑损伤后不明原因颅内压增高，尤其是并发颅骨骨折时，应警惕CVST发生，尽早行神经影像学检查以明确诊断，血管内治疗协同抗凝治疗可提高其疗效。     

颞叶切除治疗频发性颞叶癫痫二例报告

颞叶切除治疗频发性颞叶癫痫二例报告昆明市人民医院神经外科惠金明，孙敏，马钢，李俊彦本文报道２例癫痫频繁发作、药物控制无效的颞叶癫痫病人，经常规脑电图检查定位并经神经放射学检查和病理检查发现相应颞叶有局灶性病变，其中１例为脑血管畸形，颞叶切除术后癫痫发...     

下丘脑错构瘤

目的：报告５例下丘脑错构瘤的临床表现、诊断及治疗。方法：分析５例下丘脑错构瘤的病史，临床表现及诊断、治疗情况，结合文献加以分析讨论。结果：５例皆在儿童早期发病，均有性早熟，其中３例有痴笑样癫痫，其它可有癫痫大发作，行为异常或智力减退，有２例手术治疗取得了较好疗效。病理可见神经元结构，电镜显示神经元内有分泌颗粒。结论：下丘脑错构瘤可以手术治疗，药物治疗对性早熟有效，而对痴笑样癫痫无效     

抗 N-甲基-D-天冬氨酸受体脑炎3例临床分析

目的：总结抗NMDA受体脑炎的临床表现、诊断、治疗及预后等特点,以期提高对该病的认识。方法分析3例抗NMDA受体脑炎患者的临床表现、实验室检查、影像学检查、对免疫治疗的反应及预后。结果3例患者均表现有显著的精神症状,难治性癫痫和自主神经功能障碍,部分患者伴有运动障碍;脑脊液及血清抗 NMDA 受体阳性;免疫治疗有效。结论无精神病史的患者出现不明原因的精神症状伴痫性发作、记忆丧失、意识水平降低、运动障碍甚至出现中枢性通气不足,特别是伴有卵巢畸胎瘤者应警惕抗NMDA受体脑炎,及时诊断和治疗有助于抗NMDA受体脑炎患者的恢复。     

阵发性交感神经过度兴奋2例报道及相关文献复习

目的 探讨阵发性交感神经过度兴奋(paroxysmal sympathetic hyperactivity,PSH)的临床特征、诊断和治疗。方法 对2例PSH患者的病程、临床表现及辅助检查及诊疗过程进行分析。结果 2例患者中1例是脑干出血患者,另1例是大面积脑梗死患者。2例患者均以阵发性躁动、高热、大汗、血压升高、心动过速、呼吸急促及肌张力障碍等为主要临床表现。发作间期格拉斯哥昏迷评分分别为5和15分。2例患者均行脑电图检查,均未见癫痫波。神经影像学检查提示额叶、颞叶、顶叶、脑干等部位损伤。23例抗癫痫药物治疗无效; β受体阻滞剂和加巴喷丁联合应用对PSH有较好疗效。结论 不同病因、不同程度脑干出血与脑梗死均可导致PSH。PSH易被误诊为癫痫,抗癫痫治疗无效。β受体阻滞剂和加巴喷丁联合治疗有效。     

癫痫免疫性病因研究进展北大核心CSCD

免疫性癫痫在癫痫患者中占比不低,癫痫的免疫性病因应受到更多重视。自身免疫相关性癫痫(autoimmune-associated epilepsy,AAE)指自身免疫性脑炎(autoimmune encephalitis,AIE)后遗留的慢性癫痫发作,AIE患者神经特异性抗体(neural specific autoantibodies,NSAbs)类型不同,发生AAE的比例也不同,早期免疫治疗是预防AAE发生的重要预防措施。在不明原因癫痫患者中检出NSAbs同样提示病因为免疫性,这类患者常表现频繁发作、药物难治等特点。近期学者提出多种预测不明原因癫痫患者NSAbs阳性的评分系统,但其有效性仍需进一步研究证实。免疫是癫痫的重要病因,及早诊断和治疗能更好地改善患者的预后,临床医师应加强癫痫免疫性病因的筛查,务求早诊断、早治疗。     

误诊为耐药性癫痫的胰岛素瘤4例临床报告

目的了解胰岛素瘤非痫性发作的临床特征及与耐药性癫痫的鉴别。方法回顾性研究4例发作间期EEG和头部MRI正常,抗癫痫药物治疗无效而误诊为耐药性癫痫,经术后病理证实为胰岛素瘤非痫性发作病例,分析其临床特征和误诊原因,探索其鉴别要点。结果胰岛素瘤非痫性发作多出现在空腹或进食前,发作前后有交感神经兴奋症状,临床表现多样化是重要的鉴别要点；早期常规血糖检查诊断价值有限。结论胰岛素瘤所致的非痫性发作极易误诊为耐药性癫痫,发作期血糖、血清胰岛素和胰岛素释放指数等实验室检查可为诊断提供依据；B超和CT是重要的影像学检查手段,影像学检查阴性而其它检查支持胰岛素瘤时需行病理学检查,明确诊断是胰岛素瘤所致非痫性发作治疗的关键。     

儿童睡眠中癫痫电持续状态患儿的临床及脑电图特征

目的探讨儿童睡眠中癫痫性电持续状态(ESES)的临床和脑电图变化特征。方法通过长程视频脑电图(VEEG)监测,对35例合并ESES的癫痫患儿进行临床资料及脑电图资料的回顾性分析。结果35例患儿的VEEG,NREM期均见ESES现象,放电以Rolandic区为主。发病后精神行为异常25例(71.4%),智力障碍19例(54.2%),语言障碍22例(62.8%)。经抗癫痫药治疗后,26例有效,临床发作完全控制或明显减少;7例无效者采用甲泼尼龙冲击治疗后,在控制临床发作和痫样放电方面有良好的效果;2例无效。结论 ESES是涉及多种癫痫综合征的特殊EEG,NREM睡眠期持续放电是引起神经心理损伤的主要原因。而长程视频脑电图是诊断ESES的有效工具,便于早期采取干预措施,是改善神经心理损伤的关键。     

系统性红斑狼疮脑病27例报告（附1例尸检资料）

报告２７例系统性红斑狼疮脑病，占本院收容系统性红斑狼疮病人有神经精神损害人数的５６．２％。主要表现为癫痫发作，精神障碍，昏迷、偏瘫等。结合临床和尸检资料，对其病机、诊断、鉴别诊断及治疗进行讨论。指出凡遇有原因不明的神经精神障碍的中青年女性，应当想到本病。     

Crises épileptiques révélant des anomalies du métabolisme phosphocalcique

Hypocalcemia due to hypoparathyroidism produces a broad spectrum of clinical manifestations, but overt symptoms may be sparse. One unusual presentation is onset or aggravation of epilepsy in adolescence revealing hypoparathyroidism. This situation can lead to delayed diagnosis, with inefficacity of the antiepileptic drugs. We report five cases of adolescence-onset epilepsy with unsuccessful antiepileptic therapy, even with gradually increasing dose. Physical examination revealed signs of hypocalcemia, confirmed biologically. Full testing disclosed the origin of the seizures: hypoparathyroidism in three patients and pseudohypoparathyroidism in the other two. In four of five patients, computed tomography showed calcification of the basal ganglia, defining Fahr's syndrome. The patients were treated with oral calcium and active vitamin D (1-alphahydroxy vitamin D3). Seizure frequency progressively decreased and serum calcium levels returned to normal. These cases illustrate the importance of the physical examination and of routine serum calcium assay in patients with new-onset epileptic seizures in order to detect hypocalcemia secondary to hypoparathyroidism.     

Epileptic seizures as the sole manifestation of neuro-Behçet's disease: Complete control under interferon-alpha treatment

Behçet's disease (BD) is a multisystemic disease of unknown etiopathogenesis with various clinical features including manifestations from central nervous system involvement. We report the case of a patient presented with a 20-year history of BD and a 10-year history of epileptic seizures refractory to various antiepileptic drugs. Under systemic treatment with interferon-α 2a (IFN-α) a complete remission of the cutaneous manifestations and a seizure-free state were achieved. The impressive therapeutic response of both the seizures and the non-neurological manifestations to IFN-α was also observed upon re-administration of this cytokine subsequent to a severe BD relapse. In view of this response and the lack of any other obvious etiology of the seizures in our patient, it seems reasonable to consider them as being the sole manifestation of neuro-BD. The patient is presently completing a 40-month seizure-free follow-up, despite withdrawal of all antiepileptic drugs for the last 35 months. Further studies on large numbers of patients are now warranted to define the therapeutic efficacy and safety of IFN-α in neuro-BD and particularly in neuro-BD-related epileptic seizures.     

PET-CT联合神经导航在难治性癫痫术中致痫灶定位中的应用

目的探讨PET-CT在难治性癫痫手术致痫灶定位应用中的准确性。方法回顾性分析80例2016-03—2018-05在郑州大学第二附属医院治疗的难治性癫痫患者,所有患者均已明确口服抗癫痫药物效果较差,经磁共振检查均为阴性,经24 h视频脑电监测结果回示有明显异常,根据是否行PET-CT检查分组,其中40例行PET-CT检查的患者设定为观察组,使用神经外科导航融合PET-CT影像学数据行术前定位癫痫灶;未行PET-CT检查的40例患者设定为对照组。2组患者术中均使用皮层脑电图监测定位致痫灶,并通过手术切除癫痫灶,观察术后癫痫症状的缓解情况。结果观察组治疗效果明显优于对照组(P<0.05);且观察组术后癫痫缓解情况、术中损伤明显优于对照组(P<0.05)。结论PET-CT联合神经外科导航可优化难治性癫痫手术的过程,在整个治疗过程起到关键作用。     

Electroconvulsive therapy in patients with epilepsy

There are scant published data to guide the clinician about safe and effective use of electroconvulsive therapy (ECT) in epileptic patients who suffer from psychiatric disorders. In this report, we describe our experience treating 43 epileptic patients with ECT. Seven of the patients may have had spontaneous seizures during the course of treatments, although the possibility of pseudoseizures or nonictal phenomena seemed quite likely in several of these cases. For the majority of patients, adequate seizures could be obtained during ECT despite concomitant treatment with antiepileptic medications, although dose reductions were required in a few cases. Most patients enjoyed moderate to marked reductions in psychiatric symptoms with ECT, and one patient seemed to have a marked reduction in spontaneous seizure frequency for several weeks after completion of the ECT course. We conclude that most epileptic patients can be treated with ECT without dose adjustment in antiepileptic medications and provide general recommendations for safe use of ECT in this population.     

抗癫痫药物对癫痫患者甲状腺激素水平影响的研究

目的 研究癫痫患者甲状腺激素水平和抗癫痫药物对其影响以及与疗效之间的关系。方法 测定已确诊的45例未服用过抗癫痫药物的癫痫患者血清甲状腺激素水平并与30例健康对照组进行比较。再经卡马西平、苯妥英钠、丙戊酸钠三种抗癫痫药物分组单药治疗3个月、6个月、年后观察甲状腺激素水平的变化及与疗效之间的关系。结果 未服用抗癫痫药物的新诊断癫痫患者游离甲状腺素（FT4）水平显著低于健康对照组,经苯妥英钠、卡马西平分别治疗3个月、6个月、1年后T4、FT4、FT3显著低于治疗前水平,TSH无显著性变化。经丙戊酸钠治疗后的不同时间段各甲状腺激素水平与治疗前比较无显著性差异（P＞0．05）。甲状腺激素水平的变化与化疗效之间似无相关性。结论 癫痫的反复发作虽未经抗癫痫药物治疗已存在FT4水平的降低。苯妥英钠、卡马西平可明显造成癫痫患者的亚临床甲状腺功能降低（T4、FT4、FT3下降）,丙戊酸钠对患者甲状腺激素水平无显著影响。甲状腺激素水平的变化与疗效之间无相关性。     

Treatment of acquired epileptic aphasia with the ketogenic diet

Acquired epileptic aphasia remains a poorly understood entity, which is frequently difficult to treat. Previously described treatment modalities have included antiepileptic drugs, corticosteroids, intravenous immunoglobulin, and multiple subpial transections. We describe three patients with acquired epileptic aphasia refractory to traditional treatments who were successfully treated with the ketogenic diet. All three patients had lasting improvement of their language, behavior, and seizures for 26, 24, and 12 months, respectively. This is the first reported series of patients with acquired epileptic aphasia successfully treated with the ketogenic diet, and suggests a new therapeutic alternative for patients with this often difficult-to-treat disorder.     

热性惊厥史与青少年癫痫病因关系临床分析

目的 分析有热性惊厥史的青少年癫痫患者临床特点和治疗情况,探讨热性惊厥史与青少年癫痫之间的关系。方法 对发病年龄〈18岁的青少年癫痫患者的病因进行回顾分析,总结单纯性和复杂性热性惊厥患者癫痫发作特点、辅助检查和服药情况。结果 符合癫痫诊断标准的4595例患者中发病年龄〈18岁者2699例占58.74%,其中原发性癫痫1573例占58.28%、继发性癫痫1126例占41.72%。青少年继发性癫痫以热性惊厥（297例占26.38%）为主要病因,其中单纯性热性惊厥200例、复杂性热性惊厥97例,均以部分性发作为主（χ^2=4.933,P=0.026）。有热性惊厥史患者神经影像学（χ^2=38.083,P=0.000）和神经电生理学（χ^2=4.469,P=0.035）异常检出率明显增加;其中服药率约为66.67%（198/297）,服用传统抗癫痫药物者占70.20%（139/198）。结论 青少年继发性癫痫病因较为复杂,以热性惊厥为主要病因。无论单纯性或复杂性热性惊厥均以部分性发作常见,仍以传统抗癫痫药物为主,约1/3患者未服药或未正规服药。     

Prognosis of patients with seizures occurring in the first 2 years

The aim of this study is to determine the prognosis of patients with seizure onset from 1 to 24 months of age in respect to epilepsy, developmental outcome, and neurological status. It also aims to determine predictive factors regarding an unfavorable prognosis. Seventy-five patients were retrospectively analyzed. Univariate analysis revealed the following findings: (1) mental retardation at initial admission, abnormal neurological finding, infantile spasm, use of more than 1 antiepileptic drug, epileptic activity on electroencephalography (EEG) of neonatal seizure, and perinatal anoxia were significant risk factors with regard to developmental outcome; (2) mental retardation at initial admission, abnormal neurological finding, infantile spasm, use of more than 1 antiepileptic drug, epileptic activity on EEG, symptomatic etiology, history of neonatal seizure, and perinatal anoxia were significant risk factors regarding neurological status; and (3) mental retardation at initial admission, neurological abnormality, infantile spasm, use of more than 1 antiepileptic drug, epileptic activity on EEG, status epilepticus, symptomatic etiology, seizure frequency of more than once per week, history of perinatal anoxia, and neonatal seizure were significant risk factors regarding epilepsy prognosis. In addition, multivariate analysis revealed that neurological abnormality and use of more than 1 antiepileptic drug were significant for developmental outcome, that epileptic activity on EEG and use of more than 1 antiepileptic drug were significant for neurological status, and that perinatal anoxia, infantile spasm, and status epilepticus were significant for epilepsy prognosis. These findings suggest that neurological abnormality, use of more than 1 antiepileptic drug, infantile spasm, status epilepticus, and perinatal anoxia are unfavorable predictive risk factors regarding the prognosis of patients with seizures that have an onset from 1 to 24 months of age.     

Antiepileptic Drug Intoxication: Factors and Their Significance

A retrospective chart review (1979-1985) was performed to identify probable causes of intoxication with antiepileptic drugs (AEDs). We identified 141 patients meeting clinical and laboratory criteria for intoxication and 17 with clinical signs but with serum concentrations within the therapeutic range. The majority were epileptic patients; almost half were treated with monotherapy, most with phenytoin (PHT). The causes of intoxication in the epileptic patients were iatrogenic (41%), inappropriate dose self-adjustment (34%), suicide attempt (18%), inappropriate caretaker dose adjustment (9%), accidental ingestion (8%), unrecognized drug interaction (6%), and association with intercurrent illness (2%). Twenty-two patients had more than one probable cause of intoxication. In nonepileptic patients the causes were suicide attempt (50%), accidental ingestion (27%), and iatrogenic (23%). Most patients had signs of ocularmotor and vestibulocerebellar dysfunction. Rarely described manifestations of intoxication, such as seizures or choreoathethosis, were observed in a few patients. The average hospital stay was 6.9 days; there was no mortality, and all patients recovered fully. We conclude that AED intoxication is a major but preventable cause of morbidity and that suicide attempts are an important and underrecognized contributor in both epileptic and non-epileptic patients.     

24小时动态脑电图监测对不典型癫痫的诊断价值

目的 探讨24小时动态脑电图（AEEG）监测对不典型癫痫的诊断价值。方法 对21例临床上疑似癫痫，但发作不典型的患者作24小时AEEG检测，并结合临床进行观察。结果 21例常规脑电图（REEG）均未见痫样放电，而AEEG可检测到多次阵发棘波，尖波，棘慢波综合，尖慢波综合等痫样放电，并经抗癫痫药均获得控制，故可诊断为癫痫。结论 24小时AEEG监测能帮助临床上诊断不典型的癫痫患者。