电视录像脑电图在癫痫及其他发作性疾病中的诊断价值探讨

目的：评价电视脑电图（Video-EEG)监测在癫痫及其他发作性疾病临床诊断中的应用价值。方法：对216例具有各种发作性症状的患者进行连续24h的包括清醒、睡眠及诱发试验的Video-EEG监测。结果：216例患者中130例（60．2％）监测到临床发作，其中53例伴有发作期痫样放电，证实为癫痫性发作；73例发作期及发作间期均无痫样放电，为非癫痫性发作。216例患者中共80例监测到了痫样放电，其中64例通过发作期的脑电－临床表现和（或）发作间期的EEG特征，结合有关病史资料确定了癫痫的发作类型，27例监测后发作分类得到了修正。结论：Video-EEG可提高痫样放电的检出率，有助于癫痫发作与非癫痫发作的鉴别及癫痫的分型。     

240例睡眠/清醒不同发作时间癫痫患者24小时动态脑电图分析

目的:了解睡眠/清醒不同发作时间癫痫患者24小时动态脑电图特点。方法:回顾性分析240例癫痫患者的24小时动态脑电图资料。结果:仅于睡眠中发作的癫痫患者AEEG上仅在睡眠中放电的比例高于清醒期和睡眠/ 清醒均发作患者,额叶癫痫患者于睡眠中发作者以及AEEG上单纯夜间放电者均明显多于颞叶癫痫患者,差异具有显著性(P<0.01)。结论:额叶癫痫患者及临床上仅于睡眠中发作患者睡眠中放电明显高于其他癫痫患者,对此类睡眠中高发人群进行AEEG检查有助于提高诊断率。     

835例癫痫及发作性疾病动态脑电图分析

目的分析癫痫及发作性疾病患者的动态脑电图(ambulatory EEG monitoring,AEEG),探讨其在诊断和鉴别诊断的中的价值。方法对2010年7月~2014年6月间,本院住院、门诊中临床诊断为癫痫475例及发作性疾病360例患者进行动态脑电图监测并分析。结果癫痫组动态脑电图(AEEG)与常规脑电图(routing-EEG,REEG)比较痫样放电检出率和异常率均有显著差异(P0.01)。癫痫组与发作性疾病组比较痫样放电检出率和异常率均有显著差异(P0.01)。痫样放电多见于睡眠期,以非快速眼动睡眠Ⅰ-Ⅱ期(NREMⅠ-Ⅱ期)为主。在脑电图记录过程中,癫痫组有临床发作36例:5例仅在发作期可见痫样放电,发作间期未见痫样放电;31例两期均可见痫样放电。发作性疾病组有临床发作44例:2例仅发作期可见痫样放电,发作间期未见痫样放电;4例两期均可见痫样放电;其余均未见痫样放电,占86.4%。结论癫痫及发作性疾病患者AEEG检查痫样放电检出率优于REEG检查;在临床症状类似癫痫的发作性疾病中,AEEG及REEG痫样放电检出率只为8.6%及3.6%。因此,发作性疾病需要行AEEG检查,帮助鉴别诊断和确诊。     

视频脑电图监测对癫痫的诊断价值

目的探讨视频脑电图(Video-EEG)对癫痫的诊断价值.方法对252例发作性疾病患者进行连续12～24小时监测,其中包括清醒、睡眠及诱发试验,分析临床发作和异常放电的关系,异常放电出现的时相,癫痫分型与异常放电的关系,临床发作前脑电图的改变以及临床发作间期和发作期异常脑电图的不同表现.结果252例监测到临床发作142例,其中同时伴异常放电者为111例;252例检出异常放电187例,其中出现于睡眠期者146例;确诊的111例中103例确定了发作类型,其中25例修正了发作类型;确诊的111例监测到发作前脑电图的异常改变,表现为背景脑电波频率和波幅的改变,或者出现痫样放电;111例不同发作类型癫痫患者发作间期和发作期有不同的异常脑电图表现.结论视频脑电图可提高痫样放电的检出率,有助于癫痫的诊断及分型,有利于观察癫痫患者发作间期及发作期脑电图的表现.     

15例夜间睡眠期行为异常患者的脑电图及多导睡眠图分析

目的通过对夜间睡眠期行为异常患者的脑电图及多导睡眠图分析,鉴别诊断额叶癫痫及REM期睡眠行为异常(RBD),并结合文献对其临床表现、电生理特点进行总结。方法对15例夜间睡眠期行为异常患者,均行长程录像脑电图(Video-EEG)及多导睡眠监测(PSG)。并依据国际睡眠障碍分型修订版(ICSD-R)及国际抗癫痫联盟(ILAE)所制定的癫痫综合征分类标准,给予鉴别诊断及相应治疗,随访观察预后。结果其中9例为额叶癫痫患者,其发作间期脑电图,额区、额中线区可见异常放电者7例;发作期脑电图,额区、额中线区可见节律性演变者2例,伴有大量肌肉及动作伪差,行为异常多发作于非快速眼动睡眠Ⅰ～Ⅱ期,睡眠结构中,总睡眠时间缩短、睡眠Ⅲ～Ⅳ期、REM睡眠期缩短、Ⅰ～Ⅱ期睡眠时间延长。6例为RBD患者:发作间期及发作期脑电图正常;PSG记录中,发作均位于REM期,并持续出现肌电活动增加,总睡眠时间、睡眠Ⅰ～Ⅱ期、REM睡眠期均缩短。结论额叶癫痫及RBD的临床症状复杂,容易误诊、延诊,故借助神经电生理检查手段(长程脑电图结合PSG)可以提高疾病的诊断率。     

75例额叶癫痫的临床特点及发作期发作间期脑电图分析

目的回顾性分析额叶癫痫(FLE)的临床及发作期和发作间期脑电图特点,为临床早期识别和治疗提供依据。方法详细整理75例FLE患者的临床资料,发作期及发作间期脑电图改变和影像学表现,并进行总结分析。结果 (1)临床表现:一种发作类型25例(33%);两种或两种以上发作类型50例(67%);发作时意识清醒者20例(27%);丛集性发作27例(36%);夜间发作36例(48%)。(2)脑电图表现:1发作间期脑电:50例FLE行长程视频脑电监测中异常者47例(94%);2发作期脑电:监测到临床发作22例;3睡眠期痫性放电阳性率高于清醒期(P0.01)。(3)影像学表现:影像学检查异常且病灶位于额叶者36例(54%);非额叶病灶者5例(7.6%);神经影像学未见异常者25例(33%)。结论 FLE临床表现复杂多样,运动症状常见;意识恢复快,多无发作后状态;FLE多于夜间发作,发作时间短暂,呈丛集性发作;FLE脑电图特异性差,长程视频脑电较普通脑电易于检测出痫性放电,睡眠期脑电图阳性率高于清醒期;FLE脑电显示多无侧别提示。     

额叶癫痫发作录像脑电图特点分析

目的通过录像脑电图观察额叶癫痫的临床特征、发作期及发作间歇期的脑电图特点。方法使用录像脑电图（ｖｉｄｅｏＥＥＧ,ＶＥＥＧ）对５０例确诊为额叶癫痫的患者进行常规及长时间记录,对其中１７例（２０次）癫痫发作的临床表现及脑电图所见进行分析。结果额叶癫痫常见的发作形式有：姿势性发作,具有额叶癫痫特点的自动症;复杂部分性发作时伴发声、偏转或表情的变化,有时出现发作性情绪改变或强迫思维等少见症状。其发作特点为：持续时间短,发作相对较频繁,无明显发作后意识障碍。发作间歇期脑电图有时可无阳性所见,典型的临床发作及发作时ＶＥＥＧ记录到的额部爆发性节律有助于诊断。本组９例儿童期起病的额叶癫痫患者,以夜间频繁的躯体自动症发作为主,检查均未发现相应的脑器质性病变。脑电图睡眠描记可见频繁的额部导联癫痫样放电。临床治疗观察预后良好,提示可能为一组儿童原发性部分发作型癫痫综合征。结论额叶癫痫是一组较为特征性的癫痫综合征,临床并不少见,及时而正确的诊断有助于治疗。     

动态脑电图对癫痫的诊断价值

目的探讨动态脑电图(ambulatory electroencephalogram,AEEG)对癫痫(epilepsy,EP)的诊断价值。方法应用奥地利EMS16道脑电记录仪对54例临床诊断为癫痫(常规脑电图均未发现痫样放电)的患者,进行动态脑电图监测。结果①经AEEG检查有痫样放电者44例,阳性率81.5%;②有临床发作者37例(68.5%),其中30例(55.6%)同时出现痫性放电;3例(5.6%)发作期无痫样放电,发作间期脑电图显示痫样放电;4例(7.4%)发作期和发作间期均没有痫样放电;③17例(31.5%)未监测到临床发作,其中11例(20.3%)有痫样放电;6例(1.1%)无痫样放电。结论AEEG监测对癫痫的诊断具有重要价值。     

额叶癫痫的特点及手术治疗

目的 :分析额叶癫痫的临床特征、发作期及发作间期脑电图特点 ,探讨额叶癫痫手术治疗。方法 :应用视频脑电图对 9例额叶癫痫患者进行长程监测 ,并对其中 6例记录颅内脑电图。分析癫痫发作的临床表现及脑电图特点 ,定位致痫灶 ,行手术切除。结果 :额叶癫痫的发作特点为 :发作频繁而短暂 ,以睡眠期发作为主 ,常见过度运动 ,姿势性强直 ,发声等发作症状。发作期可见棘波节律 ,广泛低幅快活动 ,节律性慢波等特征性脑电活动。颅内电极记录可清晰显示异常脑电活动的发作起源及扩散情况 ,有助于定位致痫灶。手术切除病灶及致痫灶 ,效果满意。结论 :额叶癫痫是一组具有特征性的癫痫综合征 ,颅内电极记录有助于揭示其脑电活动变化。对于难治疗性额叶癫痫 ,准确定位致痫灶是手术成功的关键。     

长程视频脑电图在癫癇诊断与鉴别诊断中的作用

目的评价长程视频脑电图对癫癇和非癫癇发作性疾病的诊断价值。方法对因发作性事件就诊并拟诊为癫癇或可疑癫癇的279例患者的长程视频脑电图资料进行回顾分析。结果 279例患者中122例(43.73%)长程视频脑电图监测到临床发作,其中84例可见发作期同步样放电而确诊为癫癇发作,38例发作期无同步样放电而确诊为非癫癇发作;157例(56.27%)未监测到临床发作,其中102例可见发作间期样放电。监测到发作期或发作间期样放电的188例患者中97例明确癫癇发作类型,其中75例进一步确定为癫癇综合征。结论长程视频脑电图在癫癇的诊断、分型及其与非癫癇发作性疾病的鉴别诊断中具有重要临床价值。     

夜发性额叶癫痫三家系临床和遗传学特征

目的 分析夜发性额叶癫痫3个家系的临床、脑电图和遗传学特征.方法 在3个夜发性额叶癫痫家系患者及部分亲属中收集临床、脑电图及神经影像学等资料,并采用测序方法筛查烟碱型乙酰胆碱受体(nAChR)α4、β2和α2亚单位编码基因(CHRNA4、CHRNB2和CHRNA2).结果 3个家系中有6例患者(其中男5例),平均年龄(20.5±11.5)岁,平均发病年龄(7.3±5.5)岁,临床表现为夜发性额叶发作,具体发作类型包括姿势性发作2例,躯体自动症发作4例,最多每夜发作6次.发作间期、发作期视频脑电图2例患者表现为正常或动作伪差,2例表现为前部导联慢波节律,3例出现前部导联棘波、棘慢波及尖波.神经系统及神经影像检查未见异常.抗癫痫药物治疗反应良好.CHRNA4、CHRNB2和CHRNA2部分序列(包含跨膜区1～3)筛查未见突变.结论 夜发性额叶癫痫是一种遗传异质性癫痫综合征.     

Paroxysmal motor disorders of sleep: the clinical spectrum and differentiation from epilepsy

The diagnosis of paroxysmal events in sleep represents a significant challenge for the clinician, with the distinction of nocturnal epilepsy from nonepileptic sleep disorders often the primary concern. Diagnostic error or uncertainty is not uncommon in this situation, particularly with respect to nocturnal frontal lobe epilepsy (NFLE), which has a variable and often unusual presentation. Such errors can be minimized if the range of nonepileptic disorders with motor activity in sleep is fully appreciated. Here we review these disorders, before discussing the important clinical and electrographic features that allow their accurate differentiation from seizures. Particular emphasis is placed on the differentiation of nocturnal frontal lobe epilepsy from non-rapid eye movement (NREM) arousal disorders and other parasomnias. The value of recording episodes with video EEG polysomnography is discussed.     

The Sleep Manifestations of Frontal Lobe Epilepsy

Frontal lobe seizures have a tendency to occur from sleep, and in some cases occur exclusively (or almost exclusively) from sleep; these individuals are said to have nocturnal frontal lobe epilepsy (NFLE). NFLE can be difficult to distinguish clinically from various other sleep disorders, particularly parasomnias, which also present with paroxysmal motor activity in sleep. Here, the manifestations of frontal lobe epilepsy are reviewed in detail, with particular reference to the influence of sleep and the characteristics of NFLE. Key aspects of differential diagnosis are also considered, and the underlying mechanisms involved in NFLE discussed.     

Distinguishing sleep disorders from seizures: diagnosing bumps in the night

BACKGROUND: Abnormal paroxysmal events in sleep may be parasomnias or epileptic seizures. In nocturnal frontal lobe epilepsy (NFLE), the unusual seizure features often lead to diagnostic confusion with nonepileptic parasomnias; video-electroencephalography monitoring is usually required to make the diagnosis. OBJECTIVE: To examine the reliability of the clinical history in diagnosing NFLE, using the Frontal Lobe Epilepsy and Parasomnias (FLEP) scale. DESIGN: The FLEP scale, comprising specific questions reflecting the diagnostic features of NFLE and parasomnias, was developed by an expert panel following review of the literature. It was then validated in a sample of individuals with firmly diagnosed nocturnal events. SETTING: Patients were recruited after appropriate diagnostic workup in tertiary sleep and epilepsy referral centers in Melbourne, Australia. PARTICIPANTS: Sixty-two patients (45 males) [corrected] with paroxysmal nocturnal events. Intervention Two independent interviews were conducted in each case, with the patient and a witness, by researchers blinded to the diagnosis. MAIN OUTCOME MEASURE: The diagnosis obtained from scores on the FLEP scale was compared with the confirmed diagnosis in each patient. RESULTS: Nocturnal frontal lobe epilepsy was correctly diagnosed from the FLEP score in 31 of 31 patients, with a sensitivity of 1.0 (95% confidence interval [CI], 0.85-1.00), specificity of 0.90 (95% CI, 0.73-0.97), positive predictive value of 0.91 (95% CI, 0.75-0.97), and negative predictive value of 1.00 (95% CI, 0.85-1.00). CONCLUSIONS: A diagnosis of NFLE can be made reliably using the clinical features identified in the FLEP scale. This may reduce the requirement for tertiary referral and extensive inpatient monitoring.     

Parasomnias and nocturnal frontal lobe epilepsy (NFLE): lights and shadows--controversial points in the differential diagnosis

Nocturnal frontal lobe epilepsy (NFLE) is characterized by seizures with complex, often bizarre, violent behaviour arising only or mainly during sleep. These unusual seizures and their occurrence during sleep are often accompanied by normal EEG tracings and neuroradiological findings, making it difficult to distinguish NFLE seizures from other non-epileptic nocturnal paroxysmal events, namely parasomnias. NFLE was described for the first time in 1981, but, as its epileptic origin was controversial, the condition was called nocturnal paroxysmal dystonia. Even though many aspects of parasomnias and NFLE have been clarified in the last two decades, the problem of differential diagnosis remains a challenge for clinicians. This paper discusses some controversial points still under debate. The difficulties in distinguishing nocturnal epileptic seizures from parasomnias reflect just one aspect of the intriguing issue of the pathophysiological relationships between all types of paroxysmal motor behaviours during sleep.     

Intracerebral recordings of nocturnal hyperkinetic seizures: demonstration of a longer duration of the pre-seizure sleep spindle.

OBJECTIVE: Nocturnal frontal lobe epilepsy (NFLE) seizures occur primarily during non-rapid eye movement sleep stage 2. We observed in several patients rhythms of same localization and frequency as sleep spindles, immediately preceding and sometimes continuing at seizure onsets. We aimed to study the link between sleep spindles and seizure onsets. METHODS: We used intracerebral stereo-EEG ictal recordings of two MRI-negative patients with clinically defined NFLE. For each of the six studied seizures, sustained activity in the frontal sleep spindle frequency (12Hz) was observed around seizure onset. The duration of this pre-seizure sleep spindle was compared to that of the 10 preceding sleep spindles. RESULTS: The pre-seizure sleep spindles were clearly of longer duration than the "interictal" sleep spindles for all seizures. This sustained pre-seizure 12Hz activity could be differentiated from normal awakenings, and showed no spatial relation to the ictal onset. CONCLUSIONS: We demonstrated a functional alteration of the sleep spindle-generating thalamocortical loop concomitant with the seizure onsets. This defect may also be involved in seizure generation. SIGNIFICANCE: A thalamic participation in NFLE pathogenesis is likely in our two patients. The study of additional patients will allow to evaluate the role of the thalamocortical circuits in NFLE.     

The FLEP scale in diagnosing nocturnal frontal lobe epilepsy, NREM and REM parasomnias: data from a tertiary sleep and epilepsy unit

Purpose: To test the usefulness of the FLEP scale in diagnosing nocturnal frontal lobe epilepsy (NFLE), arousal parasomnias, and REM sleep behavior disorder (RBD). Methods: The FLEP scale was applied to 71 subjects (60 male; 11 female; aged 54 ± 21) referred to an outpatient's sleep and epilepsy unit for diagnostic assessment of nocturnal motor‐behavioral episodes, which turned to be arousal parasomnias (11 subjects), NFLE (14 subjects), or idiopathic RBD (46 subjects), based on the findings of in‐lab full night video polysomnography with extended EEG montages. Results: The sensitivity of the scale as a diagnostic test for NFLE was 71.4%, the specificity 100%, the positive predictive value 100%, and the negative predictive value 91.1%. The FLEP scale gave an incorrect diagnosis in 4/71 (5.6%) of the cases, namely NFLE patients with episodes of nocturnal wandering, and uncertain diagnostic indications in 22/71 subjects (30.9%). Conclusions: The FLEP scale shows high positive and negative predictive values in diagnosing NFLE versus arousal parasomnias and RBD. However, the scale is associated with a real risk of misdiagnosis in some patients and gives uncertain indications in about one‐third of cases, mainly RBD. Our investigation highlights the inadequacy of some of the items in the scale. The item investigating wandering, as presently formulated, may be unable to distinguish nocturnal wandering from sleepwalking. The items about “recall” and “clustering” of the events throughout the night may increase the likelihood of mistaking RBD for seizures. Further testing of the reliability of the FLEP scale items appears to be needed.     

Autosomal dominant nocturnal frontal lobe epilepsy and mild memory impairment associated with CHRNB2 mutation I312M in the neuronal nicotinic acetylcholine receptor

Certain paroxysmal nocturnal behaviors have been established as features of nocturnal frontal lobe epilepsy (NFLE). Despite insight into its genetics, the majority of patients with NFLE are not linked to a known mutation and clinical diagnosis remains a challenge. We describe a family presenting with stereotyped nocturnal arousals from non-rapid eye movement sleep, bilateral hand posturing, and pelvic thrusting in the mother, but subtle motor activity in the daughter, and minimal or no epileptiform EEG discharges. Despite normal IQ, there were moderate and severe verbal memory deficits in the mother and daughter, respectively. Genetic testing revealed the CHRNB2 mutation I312M in transmembrane region 3 (M3) of the neuronal nicotinic acetylcholine receptor. Phenotypic similarities in unrelated families suggest the determining role of this mutation in NFLE, whereas different inter- and intrafamilial cognitive profiles point to other factors. The absence of clear motor features of NFLE in the daughter emphasizes the shortcomings of current clinical criteria and the potential for genetic testing to further guide clinical diagnostic criteria.     

Distinctive polysomnographic traits in nocturnal frontal lobe epilepsy

Purpose: To describe the polysomnographic features and distribution of epileptic motor events, in relation to conventional sleep measures and cyclic alternating pattern (CAP) parameters, in 40 untreated patients with nocturnal frontal lobe epilepsy (NFLE). Methods: We analyzed the basal polysomnographic recordings of 40 patients (20 male and 20 female; mean age: 31 ± 10 years) with a diagnosis of nocturnal frontal lobe epilepsy. Conventional sleep measures and CAP parameters were assessed. Polysomnographic recordings were subdivided in sleep cycles. The distribution of the epileptic motor events (including minor motor events, paroxysmal arousals, tonic‐dystonic, or hyperkinetic seizures and epileptic nocturnal wandering) was analyzed throughout: total sleep time, non–rapid eye movement (NREM) and REM sleep, light sleep (S1 + S2), slow wave sleep (SWS), each sleep cycle, CAP or non‐CAP sleep, phase A and phase B of CAP. Only clear epileptic motor events supported by video–polysomnographic evidence were taken into consideration. Polysomnographic findings of patients with NFLE were compared with those of 24 age‐ and gender‐balanced healthy subjects without sleep complaints. Key Findings: Compared to controls, patients with NFLE showed a significant increase in wake after sleep onset, SWS duration, and REM latency, whereas REM sleep duration was significantly lower in NFLE patients. The patients with NFLE showed a significant increase of CAP time, CAP rate (72% vs. 32% in control group), CAP cycles, and mean duration of a CAP sequence. These findings were associated with a significant enhancement of all subtypes of the A phases of CAP (mainly subtype A1). A total of 139 epileptic motor events supported by video‐polysomnographic evidence were counted: 98% of all seizures occurred in NREM sleep and 72% of NREM seizures emerged from SWS, the latter being particularly collected in the first sleep cycles and decreasing in frequency together with the progressive decline of deep sleep. Ninety percent of total NREM seizures occurred during a CAP sequence, and CAP‐related seizures occurred in association with a phase A. Significance: Significant polysomnographic alterations seem to emerge in patients with NFLE (increased REM latency, epileptic fragmentation of SWS, and increase of CAP rate). The analysis of seizure distribution showed that most epileptic events occurred in SWS, with predominance in the first sleep cycle and decreasing in frequency together with the homeostatic decline of SWS across the night. Within the NREM sleep, CAP is a manifestation of unstable sleep and represents a powerful predisposing condition for the occurrence of nocturnal motor seizures, which arise in concomitance with a phase A.