荧光屏诱发癫痫发作国例报告

目的：回顾性分析4例光敏性癫痫病人的资料，以提高对荧光屏诱发癫痫发作的认识。方法：对2000年1月至2001年12月来院求诊的4例光敏性癫痫病人的临床资料进行分析，结果：4例中2例仅由转换电视频道诱发作，1例看电视中诱发发作，另1例玩计算机游戏诱发发作。4例中2例为全身强直阵挛发作（GTCS），1例为部分性发作继发全身发作，1例为失神及部分性发作。结论：荧光屏诱发癫痫可以有多种发作类型，不仅有GTCS，还可以有部分发作及失神。     

脑电地形图在癫痫外科治疗上的应用

近来,我院对14名难治性癫痫行脑电地形图(BEAM)检查及手术治疗,效果满意。结合资料,进行讨论。临床资料一般资料男11例,女3例,年龄8～46岁,平均24岁。病程3个月～32年。发作类型:全身强直一阵挛性发作(GTCS)9例,复杂性部分发作(CPS)3例,单纯性部分发作(SPS)加CPS加GTCSl例,另1例为GTCS加单纯失神。所有病例均行常规EEG、CT及BEAM检查。其中4例行MRI检查。方法:按国际10/20法安放电极。时间常数     

感觉刺激对癫痫发作的抑制作用

为了证实感觉刺激抑制癫痫发作的临床现象,作者对19例全身性发作癫痫病人采用声音刺激抑制由过度换气所诱发的失神发作脑电活动,139次发作中79次抑制成功。本组包括病人19例,男5,女14;3～38岁(平均16岁);发作类型:8例失神发作,4例肌阵挛发作,2例合并前两种发作,4例全身强直发作,1例不典型失神发作。过度换气(每次5分钟,共三次,每次间隔3分钟)诱发失神发作。第一次刺激在发作     

丙戊酸钠治疗顽固性癫痫时的药物动力学与药效

作者对20名药物未能有效控制的各型顽固性癫痫病人进行了丙戊酸钠(VPA)的药物动力学及毒性研究.20名病人中,10名为成人,10名为儿童,除1例外都正在服用多种抗癫痫药物.成人癫痫病期达7-37年,儿童达2-10年.成人癫痫类型为失神及全身强直—阵挛发作3例、单纯失神发作1例、复杂部份性及失神发作1例、复杂部份性发作5例;儿童为失神发作1例、失神及全身强直—阵挛发作2例、非典型失神、全身强直—阵挛及无张力性混合发作4例、家族性进行性肌阵挛癫痫1例、继发性全身性部份发作1例、复杂性部份性伴无张力性发作1例、每位病人除进行一般检查,化验,血浆抗癫痫药物浓度测定外;并记录脑电图变化及测定VPA的高峰血浆浓度与半衰期.上述项目进行完毕     

癫痫外科治疗的探讨(附32例临床资料)

我院于1987年11月至1991年6月末,对药物治疗无效的32例癫痫患者施手术治疗,现结合临床资料讨论如下。临床资料一般资料:男性22例,女性10例。年龄4～54岁,平均年龄22.9岁。发作类型和频率:全身强直一阵挛性发作(GTCS)21例,复杂部分发作(CPS)8例,单纯部分发作(SPS)2例,GTCS+单纯失神1例。每日1～20次7例,每隔3～5日发作1次9例,每月至少2次5例,每月发作1～2次者4例,次数不等者7例。病史最长者32年。抗痉药物治疗均无效,其发作次数及持续时间都增频延长。神经系统检查:肢体轻偏瘫5例,右侧鼻唇沟浅2例。智力低下6例。呆傻、生活不能     

自身诱发部分性癫痫

有些癫痫病人可自己诱导发作。这些大多数为光敏性原发性全身发作病人,是通过间歇性光刺激,少数为模式或闭眼刺激所诱发。自身诱导的部分性发作极为罕见。作者报道1例43岁男性病人,右利,无神经病家族史和明显的     

神经外科导航系统在癫痫外科中的应用

我院 1 998年 1 0月至 1 999年 4月运用德国博伊莱公司生产 Brain SCAN神经外科导航系统 ,完成癫痫外科手术治疗 8例 ,现报告如下。材料与方法一、临床资料一般资料 :本组 8例 ,男 5例。女 3例。年龄 1 4～ 39岁 ,平均 2 6.8岁。癫痫病史 3～ 1 8年 ,平均 1 0年。复杂局限性发作 5例 ,全身强直阵挛发作 1例 ,单纯失神性发作 1例 ,失张力性发作 1例。均严格服用抗癫痫药 2年以上 ,发作次数仍在每月一次以上 ,并已影响学习和工作者。神经心理学测试智商均在70以上。辅助检查 :8例均多次行常规 EEG(包括各种诱发试验 ,药物诱发 ,配合特殊电…     

多种术式治疗顽固性癫痫的长期随访

我院自 1 992年 1 0月至 1 998年 5月 ,采用多种术式治疗顽固性癫痫 4 6例。经长期随访 ,取得了较好的效果 ,现报告如下 :资料与方法1 .一般资料　本组男 34例 ,女 1 2例 ,年龄 8～4 4岁 ,平均 2 8岁 ,病程 4～ 1 5年 ,平均 9.5年。每个病人服用过 4种以上不同抗癫痫药 ,如苯妥英钠、卡马西平、苯巴比妥、丙戊酸钠或氯硝安定 ,且病程后期同时服用 2～ 3种抗癫痫药仍不能控制癫痫发作。服药状态下发作频率 ,为数次 /天～ 2次 /月。单纯部分性发作 6例 ,复杂部分性发作 5例 ,部分性发作发展至全身性发作 7例 ,强直 -阵挛性发作 2 4例 ,失神发…     

埋藏式颅内电极在癫痫外科中应用

癫痫手术治疗前精确定位 ,可提高手术效果 ,现将我院应用埋藏式颅内电极 ,对 19例顽固性癫痫定位 ,总结分析如下。一、资料与方法自 1993年 9月至 1998年 2月 ,对 19例癫痫患者采用埋藏式颅内电极 ,进行术前痫灶定位应用 ,其中男 11例 ,女 8例 ,平均年龄 2 4 ( 14～ 4 2岁 ) ,病程平均 11年 ( 4～ 17年 ) ,均有抗癫药物治疗不能控制癫痫发作。临床发作形式 :全身强直 -阵挛性发作7例 ,失神伴全身强直 -阵挛性发作 3例 ,部分性发作发展至全身性发作 4例 ,复杂部分性发作 5例。全部病例均行多次头皮 -蝶骨ＥＥＧ检查 ,部分行剥夺睡眠或长程Ｅ…     

颅内CT钙化与癫痫及其手术治疗

我院从 1983年 4月至 1999年 3月期间 ,收治颅脑ＣＴ扫描证实钙化病灶的癫痫病人 ,共 2 6例 ,经行手术治疗效果满意 ,现将结果报告如下 :临床资料一、一般资料 :本组 2 6例 ,男、女各 13例。年龄5～ 4 0岁 ,平均 19岁 ,15岁以下者 7例 ,16岁以上者 19例。癫痫病程 7天至 2 0年 ,平均 7年 ,其中 1年以内者 5例 ,1年 1例 ,2年以上 5例 ,5年以上 15例。癫痫发作类型 :局限 (部分 )发作至强直—阵挛性发作 9例 ,复杂部分发作至强直—阵挛性发作 5例 ,复杂部分性发作 2例 ,强直—阵挛性发作 10例。癫痫发作频率 :“失神样”发作患者 ,每日多达 80…     

Seizure Risk in Offspring of Parents with Generalized Versus Partial Epilepsy

Genetic factors are commonly assumed to play a more important role in generalized than in partial epilepsy. This study tested this hypothesis by comparing risks of unprovoked seizures in offspring of individuals with generalized versus partial epilepsy. Overall, seizure incidence was no higher in offspring of persons with generalized epilepsy than in offspring of those with partial epilepsy. The number of affected offspring was about three times that expected from population incidence rates, regardless of whether the parent had partial or generalized epilepsy. For the subgroup of generalized cases with absence seizures, however, seizure incidence in offspring was about three times as high as for partial cases. The higher incidence in offspring of absence cases was only partly explained by a higher proportion of absence than partial cases with two factors associated with high risk in relatives, namely early age at onset and idiopathic epilepsy. Offspring of absence cases had higher risk than offspring of other cases not only for absence seizures, but for other seizure types as well, suggesting that absence epilepsy is not genetically distinct from other seizure types of epilepsy. These results suggest that the higher incidence sometimes observed in relatives of patients with generalized epilepsy is due to a small proportion of generalized cases with extremely high familial risks--most generalized epilepsies are no more likely than partial epilepsies to have a genetic basis.     

Felbamate for refractory absence seizures

We treated 10 patients (aged 5–37 years) with medically refractory absence seizures with felbamate (FBM). Average duration of absence seizures was 16.2 years. All patients continued to have absence seizures at maximally tolerated doses of valproic acid and/or ethosuximide. At maximal FBM dosage (45 mg/kg/day or 3,600 mg/day), there was at least a 65% reduction in absence seizure frequency in eight patients; in four, there was a > 95% reduction. Mean duration of FBM therapy is 16.2 months (range, 5–31 months). Nine patients remain on FBM. Two patients are on FBM monotherapy; seven others are on FBM and one or two other antiepileptic drugs. FBM was discontinued in one patient who developed severe insomnia followed by a return to baseline absence seizure frequency after an initial good response, and an increase in generalized tonic-clonic seizures (GTCS). Three other patients also had a history of GTCS; in one there has been no significant change, whereas two have not had a GTCS during FBM therapy. Eight patients continue to have a clinically meaningful reduced absence seizure frequency, and eight patients reported fewer adverse effects with their current regimen including FBM. Tachyphylaxis to the antiabsence efficacy was observed in four cases. These preliminary findings suggest that FBM is useful in treating absence seizures.     

Some clinical and EEG aspects of benign juvenile myoclonic epilepsy

Twelve patients with benign juvenile myoclonic epilepsy (BJME) representing 4% of our population of epileptics (n = 275) are presented. Only two patients (17%) had myoclonic jerks as the only seizure type. Seven (58%) had generalized tonic-clonic seizures (GTCS) and myoclonus. Three patients (25%) had absence seizures (AS), GTCS, and myoclonic jerks. Electroencephalographic evidence of photosensitivity was found in four (33%). Auditory precipitation of seizures was found in one patient. As is the case with other primary generalized epilepsies, the onset of BJME seems to be age specific. In our series the mean age of onset in years was 4.3 for AS, 14.75 for myoclonic jerks, and 16.4 for GTCS. It took an average of 8.5 years from the onset of BJME (range, 2-20 years) and 6.5 years from the onset of GTCS (range, 2 months-6 years) until the condition was properly recognized. Five patients experienced at least one episode of myoclonic status epilepticus. Generalized, paroxysmal, symmetric polyspike and slow wave discharges are the typical EEG finding. These complexes, however, showed considerable interpatient variability. Sleep deprivation proved to be the most valuable activating procedure. Valproic acid monotherapy effectively controlled myoclonic jerks as well as associated GTCS in most patients.     

Adolescent Onset of Idiopathic Photosensitive Occipital Epilepsy After Remission of Benign Rolandic Epilepsy

Summary: Purpose: We describe 2 girls, aged 19 years, who experienced a rolandic seizure at ages 4 and 5, respectively, together with the interictal EEG features of benign rolandic epilepsy (BRE). In adolescence both patients developed photosensitive occipital seizures accompanied by spontaneous and photic-induced occipital EEG paroxysms.
Methods: We have been following 33 patients with a history of BRE, between ages 12 and 28 years (mean 17 years). Twenty-one of these patients had experienced their last rolandic seizure before the age of 10 years and 9 of them had been without treatment since age 11 or earlier. In 2 of these 9 patients, other types of seizures recurred after remission of BRE. Clinical, EEG, and evoked potential findings on these 2 patients are presented.
Results: After having experienced BRE, both patients suffered partial seizures from age 12, with elementary visual hallucinations, visual blurring, slow head turning, cephalic pain, epigastric discomfort, unresponsiveness, and vomiting. Seizure onset was related to watching TV or exposure to bright light. EEG showed interictal occipital spikes, and a photoparoxysmal response limited to the occipital lobes. Visual evoked potentials were greatly increased in amplitude. One patient had two visual attacks only and remained seizure free after 4 years of follow-up, while the other had seizures controlled by an association of valproate and carbamazepine.
Conclusions: Clinical and neurophysiological characteristics suggest that these two patients may have presented different age-related expressions within the spectrum of a benign seizure susceptibility syndrome rather than sharply distinct epilepsy syndromes.     

Electroclinical features of epilepsy in patients with juvenile type dentatorubral-pallidoluysian atrophy

Purpose: To clarify the electroclinical characteristics of epileptic seizures in patients with juvenile type dentatorubral‐pallidoluysian atrophy (DRPLA). Methods: Seventeen patients with juvenile type DRPLA confirmed by genetic analysis were studied retrospectively. The clinical records of all 17  patients and the ictal video electroencephalography (EEG) recordings from 12 of the 17 patients were reviewed. Results: Electroclinical studies in 12 patients identified 11 habitual seizures in 6 patients as partial seizures on ictal video EEG recordings. Clinical manifestations composed mainly of versions of the head and loss of consciousness. These partial seizures were persistently recorded throughout the clinical course. Brief generalized seizures (atypical absence and myoclonic seizure) were observed in 6 of 12 patients at the early stage. In contrast, generalized tonic–clonic seizures (GTCS) were recorded in four advanced stage patients who were almost bedridden. Semiological studies in 17 patients showed that the prevalence of partial seizures was significantly higher in patients with younger epilepsy onset (below 10 years of age; χ² test, p < 0.05) and that the age of epilepsy onset was significantly lower in patients with partial seizures than in those without partial seizures (Mann‐Whitney U test, p = 0.02). However, the number of CAG repeats and age at clinical onset were not significantly different between two groups. Discussion: Partial seizure is one of the common epileptic features in juvenile type DRPLA, especially in patients with younger epilepsy onset. Seizure types may be affected in an age‐dependent manner and change evolutionally during progression of the clinical stage.     

Postictal generalized electroencephalographic suppression is associated with generalized seizures

Postictal generalized electroencephalographic suppression (PGES) may be involved in sudden unexpected death in epilepsy (SUDEP). We examined whether the occurrence of PGES depends on seizure type and whether PGES occurs more frequently in people with epilepsy who died suddenly. EEG recordings of people with pharmacoresistant focal epilepsies who died from SUDEP after presurgical video/EEG telemetry were compared with recordings of living controls. To test if PGES depends on seizure type, EEG recordings of people with temporal lobe epilepsy who had complex partial seizures (CPS) and secondarily generalized tonic-clonic seizures (GTCS) were reviewed. A total of 122 seizures in 57 individuals have been included. PGES was observed in 15% of all seizures in 26% of all individuals. Secondarily GTCS were significantly associated with PGES. Neither presence nor duration of PGES differed between the SUDEP and control groups. In conclusion, PGES is facilitated by secondarily GTCS, but does not seem to be an independent risk factor for SUDEP.     

Clinical genetic study in juvenile myoclonic epilepsy

PurposeTo evaluate clinical features of probands with juvenile myoclonic epilepsy (JME) and affected members of their families in order to study clinical genetics of JME.MethodThirteen unrelated families with at least two members with history of seizures were identified; clinical and genealogic data were collected from JME probands and family members.ResultsAll probands had myoclonic and generalized tonic–clonic seizures (GTCS), while absences occurred in 25% of them. The average age of seizure onset was 13 years. Totally 22 members from 13 families had history of seizures with average age of seizure onset at 18 years. Ten family members had JME, three had epilepsy with GTCS, two had juvenile absence epilepsy, one had adult onset myoclonic epilepsy and six of the affected individuals had unclassified type of epilepsy. In five families, JME was the solely clinical feature. JME dominated among siblings, while phenotypic heterogeneity was observed in second and third degree relatives. In three multi-generation families, members with adult onset genetic generalized epilepsies (GGE) were identified.ConclusionWe found phenotypic heterogeneity regarding epilepsy type and age of seizure onset. Using pedigree analysis, we found no evidence for preferential maternal or any other distinctive inheritance pattern. Further study is needed to confirm and clarify the results.     

Postictal breathing pattern distinguishes epileptic from nonepileptic convulsive seizures

PURPOSE: To examine postictal breathing pattern in generalized convulsive nonepileptic seizures (GCNES) and generalized tonic-clonic seizures (GTCS) and evaluate this feature as a discriminating sign. METHODS: We reviewed the postictal breathing pattern seizures in 23 generalized tonic-clonic seizures in 15 consecutive patients with epilepsy and 24 convulsive nonepileptic seizures in 16 consecutive patients with pure psychogenic seizures. We also analyzed 21 frontal lobe hypermotor seizures (FLHS) in 9 patients with frontal lobe epilepsy. RESULTS: The breathing after GTCS was deep with prolonged inspiratory and expiratory phases, regular, and loud (except for two short seizures). The breathing after GCNES was characterized by increased respiratory rate or hyperpnea with short inspiratory and expiratory phases, as can be noted after exercise. The breathing was often irregular, with brief pauses. The altered breathing lasted longer after GTCS. The two groups differed significantly in loudness of postictal respiration, postictal snoring (only with GTCS), respiratory rate (faster for the GCNES group), and duration of altered breathing (longer after GTCS) (p < 0.00001 for all features). FLHS shared postictal breathing features of GCNES, but had other distinguishing features. CONCLUSIONS: The postictal breathing pattern can help differentiate generalized tonic-clonic seizures from nonepileptic psychogenic seizures with generalized motor activity and may be helpful to the practitioner obtaining a seizure history in the clinic setting or witnessing a seizure.     

A study of idiopathic generalised epilepsy in an Irish population.

Idiopathic generalised epilepsy (IGE) is subdivided into syndromes based on clinical and EEG features. PURPOSE: The aim of this study was to characterise all cases of IGE with supportive EEG abnormalities in terms of gender differences, seizure types reported, IGE syndromes, family history of epilepsy and EEG findings. We also calculated the limited duration prevalence of IGE in our cohort. METHODS: Data on abnormal EEGs were collected retrospectively from two EEG databases at two tertiary referral centres for neurology. Clinical information was obtained from EEG request forms, standardised EEG questionnaires and medical notes of patients. RESULTS: two hundred twenty-three patients met our inclusion criteria, 89 (39.9%) male and 134 (60.1%) females. Tonic clonic seizures were the most common seizure type reported, 162 (72.65%) having a generalised tonic clonic seizure (GTCS) at some time. IGE with GTCS only (EGTCSA) was the most common syndrome in our cohort being present in 94 patients (34 male, 60 female), with 42 (15 male, 27 female) patients diagnosed with Juvenile myoclonic epilepsy (JME), 23 (9 male, 14 female) with Juvenile absence epilepsy (JAE) and 20 (9 male, 11 female) with childhood absence epilepsy (CAE). EEG studies in all patients showed generalised epileptiform activity. CONCLUSIONS: More women than men were diagnosed with generalised epilepsy. Tonic clonic seizures were the most common seizure type reported. EGTCSA was the most frequent syndrome seen. Gender differences were evident for JAE and JME as previously reported and for EGTCSA, which was not reported to date, and reached statistical significance for EGTCA and JME.     

Favorable seizure outcome in Kabuki make-up syndrome associated with epilepsy

Kabuki make-up syndrome is a mental retardation-malformation syndrome affecting multiple organ systems, with a broad spectrum of neuromuscular dysfunction and mental ability. The incidence of seizures associated with this syndrome ranges from 10 to 40%. However, details of the seizures in this syndrome have not been adequately reported or thoroughly evaluated. In this study, we analyzed seizure characteristics and clinical outcomes in nine patients with Kabuki make-up syndrome. Four patients had generalized seizures and two patients had complex partial seizures, extended to secondary generalized seizures. West's syndrome, complex partial seizure, and atonic seizure were seen in one case each, respectively. Electroencephalograms showed focal spikes in seven cases, diffuse spike and wave burst in one case, and hypsarrhythmia in one case. Seizures were well controlled in eight cases and incompletely controlled in only one case. Together with mental retardation, epilepsy can be a primary feature of Kabuki make-up syndrome. Epilepsy associated with Kabuki make-up syndrome is mainly localization-related epilepsy with a favorable seizure outcome.