Seizures in relation to shunt dysfunction in children with meningomyelocele

To determine whether seizures are a reliable sign of increased intraventricular pressure in children with shunt-dependent hydrocephalus and meningomyelocele, we performed a retrospective chart review of 346 patients with meningomyelocele and shunt-dependent hydrocephalus. Fifty-one patients had seizures. Seizure episodes were investigated to determine whether they were temporally associated with shunt dysfunction. Episodes of actual or presumed shunt dysfunction were analyzed as to presenting symptoms, ventriculogram, computed tomography scan, and shunt film results. These 51 patients had 129 admissions for possible shunt dysfunction. One hundred one admissions were due to increased intraventricular pressure; nine (9%) of these, had a seizure as one of the presenting symptoms. All of the nine episodes had other common presenting symptoms of shunt dysfunction, such as headache, vomiting, lethargy, or respiratory compromise. We conclude that seizures alone are an inadequate predictor of shunt dysfunction in children with meningomyelocele, but can be seen as one of the presenting symptoms.     

利妥昔单抗治疗儿童及青少年成熟B细胞非霍奇金淋巴瘤疗效及安全性的Meta分析

目的 探讨促肾上腺皮质激素（adrenocorticotropic hormone,ACTH）首次治疗婴儿癫痫痉挛综合征（infantile epileptic spasms syndrome,IESS）短期（ACTH治疗28 d时）疗效、复发及预后的影响因素。 方法 收集2008年4月—2018年1月中南大学湘雅医院小儿神经专科首次接受ACTH治疗且随访时间≥2年的IESS患儿的临床资料,采用多因素logistic回归分析探讨ACTH治疗短期疗效、复发和远期预后的影响因素。 结果 ACTH治疗28 d时癫痫控制率为55.5%（111/200）,治疗后12个月持续控制无复发率为67.6%（75/111）。未合并局灶性发作的患儿在ACTH治疗28 d时癫痫控制的可能性是合并局灶性发作患儿的2.463倍（P<0.05）;ACTH治疗14 d时脑电图无高度失律的患儿在ACTH治疗28 d时癫痫控制的可能性是ACTH治疗14 d时脑电图有高度失律患儿的2.415倍（P<0.05）;ACTH治疗前病程每增加1个月,治疗后12个月内复发可能性增加11.8%（P<0.05）。ACTH治疗28 d癫痫未控制患儿中重度发育迟滞或死亡的可能性是癫痫控制患儿的8.314倍（P<0.05）,结构性病因患儿中重度发育迟滞或死亡的可能性是原因不明患儿的14.448倍（P<0.05）。 结论 是否合并局灶性发作、治疗14 d时脑电图高度失律是否消失可作为ACTH治疗短期疗效的预测指标,而治疗前病程可作为ACTH治疗癫痫控制后是否复发的预测指标。IESS患儿的预后与病因相关,而使用ACTH后早期控制癫痫发作,也可改善远期预后。     

Epilepsy and mental retardation following febrile seizures in childhood

In an unselected group of children who were seen following an initial febrile convulsion, the frequency of subsequent afebrile seizures was 3.5% and of mental retardation 1%. The most common afebrile seizure type was generalized major (86%). About 3/4 of the children who developed afebrile seizures did so by three years and all by five years following the initial febrile seizure. The children with afebrile seizures differed from those without afebrile seizures in the frequency of neonatal abnormality, family history of mental retardation, focal initial febrile convulsions, and delay in psychomotor milestones before the initial febrile seizure. Only about 1/3 of the children who developed afebrile seizures ever had a recurrent febrile convulsion and none had complex recurrent febrile seizures. Half the children with mental retardation had histories of delay in psychomotor milestones prior to the initial febrile seizure, and no child with mental retardation had any seizure longer than five minutes. The administration of daily phenobarbital did not reduce the frequency of epilepsy, in spite of a significant reduction in the incidence of recurrent febrile seizures. There remains no evidence that the prevention of recurrent febrile convulsions significantly decreases the frequency of afebrile seizures or mental retardation.     

Epilepsy and Mental Retardation Following Febrile Seizures In Childhood

ABSTRACT. In an unselected group of children who were seen following an initial febrile convulsion, the frequency of subsequent afebrile seizures was 3.5% and of mental retardation 1%. The most common afebrile seizure type was generalized major (86%). About 3/4 of the children who developed afebrile seizures did so by three years and all by five years following the initial febrile seizure. The children with afebrile seizures differed from those without afebrile seizures in the frequency of neonatal abnormality, family history of mental retardation, focal initial febrile convulsions, and delay in psychomotor milestones before the initial febrile seizure. Only about 1/3 of the children who developed afebrile seizures ever had a recurrent febrile convulsion and none had complex recurrent febrile seizures. Half the children with mental retardation had histories of delay in psychomotor milestones prior to the initial febrile seizure, and no child with mental retardation had any seizure longer than five minutes. The administration of daily phenobarbital did not reduce the frequency of epilepsy, in spite of a significant reduction in the incidence of recurrent febrile seizures. There remains no evidence that the prevention of recurrent febrile convulsions significantly decreases the frequency of afebrile seizures or mental retardation.     

Computed tomography findings in partial seizures.

The computed tomography findings in 82 children with partial seizures of unknown aetiology were reviewed. All had seizures with predominantly focal motor phenomena and none had abnormality on neurological examination. Findings on computed tomography were normal in 64 children (78%) and abnormal in 18 children (22%). Fourteen children had changes representing static pathology (mainly cerebral atrophy) which did not influence patient management but four had potentially correctable lesions (two tumours and two arteriovenous malformations). There were no correlations between seizure control, seizure duration, intellectual handicap, postictal weakness, electroencephalographic findings, and abnormality on the computed tomogram. In particular, none of these features were useful in predicting the presence of a tumour or arteriovenous malformation. It is concluded that a computed tomogram is indicated in every child with partial seizures.     

Prolactin and seizure activity.

Prolactin secretion after tonic-clonic seizures (10 patients), complex partial seizures (five) and non-epileptic attacks (three) was studied in a group of children aged between 0.3 and 14 years. Seven patients with other subcategories of seizure disorders were also studied. Eight children with tonic-clonic seizures exhibited post ictal concentrations of prolactin greater than 500 mU/l. One of the children, who responded on one occasion, did not do so on another. Three children with complex partial seizures had post ictal prolactin concentrations greater than 500 mU/l, while in two the increased values were more modest (390 mU/l and 420 mU/l). The timing of the peak post ictal prolactin concentration varied from less than 20 minutes to a prolonged plateau for three hours. Other seizure types--simple partial with motor signs (2), absence seizure (1), myoclonic seizure (1), minor epileptic status (3) (with one exception), and non-epileptic attacks (3) were not associated with post ictal concentrations greater than 500 mU/l.     

癫癎儿童发作间期全夜睡眠状况初步分析

目的：随着睡眠医学的发展,癫癎患者的睡眠质量作为生活质量的一个重要内容已日益受到重视。由于儿童睡眠研究的复杂性,目前关于癫癎儿童睡眠状态的研究较少。该研究旨在初步探讨癫癎对儿童发作间期睡眠的影响。方法：采用多导睡眠生理脑电记录仪对48名癫癎儿童及12名正常健康对照儿童进行全夜多导睡眠脑电记录仪检查,按照癫癎发作类型、发作好发时间将患儿分为全面性癫癎组、局灶性癫癎组以及清醒发作组、睡眠发作组,评价各组患儿与健康对照儿童之间各项睡眠参数的差异。结果：与健康对照儿童比较,癫癎儿童睡眠效率(SE)下降（85.4±8.6）% vs （90.9±5.8）% ,(P<0.05)。局灶性癫癎儿童睡眠总记录时间(TRT)延长、SE下降;全面性癫癎非快速动眼S1期睡眠比例（S1%）增加,快速动眼睡眠比例(REM%)减少;易在睡眠期发作的癫癎S1%延长,REM%、SE较对照组减少,其中全面性癫癎组REM%、SE缩短,局灶性癫癎组TRT、S1%延长,SE下降;易在觉醒期发作的癫癎与对照组各项睡眠参数无统计学差异。结论：癫癎可影响儿童的睡眠结构,不同类型的癫癎对儿童睡眠结构影响不同。全面性癫癎患儿浅睡眠延长、快动眼睡眠缩短,其中易在觉醒期发作的儿童以浅睡眠延长为主,易在睡眠期发作的儿童以快动眼睡眠缩短、睡眠效率降低为主。局灶性癫癎患儿睡眠效率降低,其中易在觉醒期发作的儿童睡眠结构无异常,易在睡眠期发作的儿童以浅睡期延长、睡眠效率降低为主。［中国当代儿科杂志,2007,9（1）：6-10］     

婴儿严重肌阵挛癫痫的临床特征和基因突变分析

目的 探讨婴儿严重肌阵挛癫癎(SMEI)的临床特点和基因诊断.方法分析13例SMEI患儿的临床和脑电图(EEG)特点及钠离子通道SCN1A基因突变筛查结果.结果男10例,女3例.8例有热性惊厥和癫痫家族史.惊厥起病年龄2～9个月,平均5.6个月.首次发作为热性惊厥9例.13例在病程早期均以反复发热诱发的全面性或一侧性阵挛或强直阵挛发作为主,其中8例有热性惊厥持续状态.出现无热惊厥的年龄为2～21个月.病程中均出现多种发作类型.发作均有热敏感的特点,诱发因素包括发热、洗热水澡和疫苗接种.起病后出现智力发育落后11例.共济失调5例,锥体束征阳性2例.EEG在1岁前多数正常,1岁后出现全导或局灶放电.头颅MBI检查异常2例.13例均应用多种抗癫痫药治疗,发作均未完全控制.卡马西平和拉莫三嗪使部分患儿发作加重.10例发现有SCN1A基因突变.结论 SMEI的临床特点是:1岁以内起病,首次发作常为热性惊厥;1岁以后出现多种发作形式和智力发育落后;发作具有热敏感的特点;EEG早期正常,以后出现全导或局灶放电.筛查SCN1A基因突变有助于早期明确诊断,指导选择抗癫癎药物.     

One vs. two years of anti-epileptic therapy in children with single small enhancing CT lesions

The duration of anti-epileptic drug (AED) therapy in children with seizures due to single small enhancing CT lesions (SSECTL) is controversial. We sought to determine whether there is any difference in the rate of seizure recurrence after 1 vs. 2 years of AED therapy and to identify the factors predictive of seizure recurrence. A total of 115 consecutive children with seizures and SSECTL were randomly assigned to two groups. Group A received AED(s) for 1 year and Group B for 2 years seizure-free interval. CT scan and EEG were done prior to AED withdrawal and children were followed-up for seizure recurrence for at least 1 year. Association between seizure recurrence and clinical and CT characteristics was analysed. Groups A and B consisted of 55 and 51 children, respectively (nine were lost to follow-up). There were 61 boys and 45 girls; mean age 9.33 years. Most (93 per cent) had focal seizures: 36 per cent complex partial, 22 per cent simple partial, 35 per cent partial with secondary generalization; 21 per cent had status epilepticus. The two groups were comparable in clinical, EEG and CT characteristics. CT scan and EEG prior to AED withdrawal were abnormal in 44 per cent and 33 per cent respectively. Six children, three from each group had seizure recurrence. Significant association was found between seizure recurrence and abnormal CT (persistence/calcification of lesion) and abnormal EEG prior to AED withdrawal (p < 0.01). The relative risk of seizure recurrence in a child with abnormal CT and EEG prior to AED withdrawal was 26.2 (95 per cent confidence interval 3.3-210.2, p = 0.0003). No association was found between seizure recurrence and any of the other variables. There was no difference in seizure recurrence after 1 vs. 2 years of AED therapy. Combination of persistent/calcified CT lesion and abnormal EEG prior to AED withdrawal was the best predictor of seizure recurrence.     

Antecedents of seizure disorders in early childhood

One or more nonfebrile seizures occurred between the ages of 1 month and 7 years in eight per 1000 white and in nine per 1000 black children enrolled in a large prospective study. We examined hundreds of prenatal and perinatal factors as predictors of childhood seizure disorders. Congenital malformations of the fetus (cerebral and noncerebral), family history of certain neurologic disorders, and neonatal seizures were the major predictors identified. Forty percent of children with postneonatal seizures and 68% of children with minor motor seizures had one or more of these risk factors compared with 21% of the seizure-free population. More than half of the children with minor motor seizures and a third of the infants with neonatal seizures had congenital malformations. Based on the prenatal and perinatal factors examined, prediction of postneonatal seizures carried a high rate of false-positive identification, indicating that our knowledge of the etiology of childhood seizure disorders is still very limited.     

肌阵挛失张力癫(癎)的临床和脑电图特点

目的 总结肌阵挛失张力癫(癎)(MAE)的临床和脑电图特点.方法 分析MAE患儿的临床和视频脑电图及同步肌电图资料,并对治疗效果进行随访.结果 收集MAE患儿共47例,其中25例有热性惊厥史,20例有热性惊厥和(或)癫(癎)家族史,发病前智力运动发育均正常.起病年龄1.4～5.8岁,首次发作为无热的全面强直-阵挛发作(GTCS)41例(87.2%),肌阵挛发作4例,失张力发作2例.病程中均有多种发作类型,包括GTCS46例(97.9%)、肌阵挛失张力发作34例(72.3%)、肌阵挛发作47例(100%)、失张力发作32例(68.1%)、不典型失神36例(76.6%)和强直发作3例(6.4%).出现多种类型的发作时脑电图背景均为弥漫性慢波或顶区为主的θ节律,发作间期呈全导1～4 Hz(以2～3 Hz为主)棘慢波、多棘慢波发放.所有患儿均首选抗癫(癎)药物(AEDs)治疗,41例(87.2%)应用AEDs发作控制,其中37例单用或合用丙戊酸,26例联合应用拉莫三嗪.10例(21.3%)发病后出现智力落后.结论 MAE的临床特点包括:发病前发育正常,多数以GTCS起病,病程中具有多种全面性癫(癎)发作类型,肌阵挛失张力发作是其特征性发作类型;脑电图呈全导棘慢波、多棘慢波发放.早期明确诊断并合理选择AEDs是取得良好预后的关键.

Abstract：

Objective To summarize the electroclinical characteristics of myoclonic atonic epilepsy (MAE) in children. Method The clinical data, video electroencephalogram (EEG) and simultaneous electromyography (EMG) of MAE patients were analyzed. The treatment and its effects were followed up.Result In 47 MAE patients, 25 had a history of febrile seizures ( FS), 20 had a family history of FS or epilepsy. All patients had a normal development before the illness. The age of afebrile seizure onset was between 1.4 years to 5.8 years. The first seizure was generalized tonic-clonic seizure (GTCS) in 41 patients (87.2%). All patients had multiple seizure types, including 47 GTCS (97.9%), 34 myoclonic atonic seizures (72. 3% ), 47 myoclonic seizures ( 100% ), 32 atonic seizures (68. 1% ), 36 atypical absences (76. 6% ) and 3 tonic seizures (6. 4% ). EEG backgrounds were slow or parietal θ rhythm, interictal EEG showed 1-4 Hz (predominant 2-3 Hz) generalized spike and wave or poly spike and wave discharges in all cases. Seizures were controlled by antiepileptic drugs (AEDs) in 41 patients (87.2%). Valproate was used in 37. Lamotrigine was used in 26. Mild mental retardation was observed in 10 children after the onset of the illness. Conclusion The clinical features of MAE included the following: the development was normal before the onset of the illness; the onset of seizure type was often GTCS. All patients had multiple generalized seizure types. Myoclonic atonic seizure was its characteristic seizure type. EEG showed generalized discharges. Early diagnosis and rational choice of AEDs are important for getting a better prognosis.     

反复发作热性惊厥患儿海马体积动态变化的临床研究

目的 分析反复发作热性惊厥患儿海马体积(HCV)的变化及意义.方法 回顾性选择34例热性惊厥反复发作且于2013年1月1日至2019年9月30日期间完善2次头部及海马磁共振平扫患儿的临床资料和检查结果.根据随访时间,分为第1次随访组和第2次随访组;根据预后,分为热性惊厥组、无热惊厥组和癫痫组.分析比较各组患儿HCV变化...     

咪哒唑仑与地西泮治疗小儿急性惊厥的对照研究

目的：比较咪哒唑仑（MDL）与地西泮（DZP）治疗小儿急性惊厥的疗效,探讨小儿急性惊厥安全、高效的治疗方法。方法：将120例急性惊厥患儿随机分为MDL组(60例)和DZP组(60例),分别予MDL（0.3~0.5 mg/kg）和DZP(0.5~1 mg/kg)治疗,对出现惊厥反复或呈惊厥持续状态患儿,前组给予MDL维持（每小时1~8 mg/kg）,后组给予DZP维持（每小时0.5~1 mg/kg）或联合苯巴比妥钠治疗,比较两种方法疗效。结果：全部患儿于10 min内惊厥得到基本控制,MDL组与DZP组平均控制时间差异无统计学意义。MDL组与DZP组分别有5例及13例患儿10 min后出现惊厥反复或呈惊厥持续状态。维持治疗后平均控制时间分别为40±32 min和69±24 min,差异有统计学意义 (P<0．05)。两组中无一例出现与MDL及DZP相关的副反应。结论：MDL用于治疗小儿急性惊厥安全、高效,对于惊厥反复或呈惊厥持续状态患儿,疗效优于DZP。［中国当代儿科杂志,2010,12（7）：530-532］     

儿童良性枕叶癫癎的临床及脑电图特点

目的：分析比较Panayiotopoulos型及Gastaut型儿童良性枕叶癫癎（BOE）的临床、脑电图特点。方法：回顾性分析16例Panayiotopoulos型及7例Gastaut型BOE患儿的临床资料。结果：Panayiotopoulos型BOE平均起病年龄4.5岁,Gastaut型9.1岁,前者临床特征为发作期的呕吐,惊厥形式多为头眼偏转发作,以夜间为多,常泛化为全身发作,持续时间较长,但发作频率较低;Gastaut型枕叶癫癎视觉症状更常见,发作频率高, 均为白天发作,发作较少全身泛化,6例伴有明显的头痛。23例患儿脑电图均显示枕区为主的后头部高幅癎性放电,常为闭眼诱发,睁眼抑制。Panayiotopoulos型6例及Gastaut型5例患儿进行了睁闭眼试验,Panayiotopoulos型和Gastaut型各4例显示睁眼抑制现象。11 例Panayiotopoulos型及所有7例Gastaut型患儿接受抗癫癎单药治疗,11 例Panayiotopoulos型患儿均对药物反应良好,Gastaut型2例无效,2例伴认知功能障碍。结论：Panayiotopoulos型及Gastaut型儿童BOE在起病年龄、发作频率、持续时间、昼夜分布、视觉症状及对治疗的反应等方面均有差别。［中国当代儿科杂志,2010,12（7）：527-529］     

Vigabatrin in pediatric patients with refractory epilepsy

New generation antiepileptic medications have improved seizure outcome in patients with intractable epilepsy. We studied the efficacy and side effect profile of vigabatrin (VGB) in pediatric patients with intractable seizure disorder. We reviewed the database of our short-term video-EEG monitoring laboratory to screen patients with intractable epilepsy who were on VGB either alone or in combination for three months or more. We subsequently reviewed the medical records of these patients to abstract clinical information regarding age, sex, seizure type, epilepsy syndrome, efficacy and side effects of VGB. Of 111 patients, 75 (68%) were male and 36 (32%) female. Seizure onset was during the newborn period in 12 patients (11%), during the first year of life beyond the newborn period in 47 patients (42%), between 1-5 years in 23 patients (21%), and above five years in the remaining 29 patients (26%). Fifty-four patients (48.6%) had partial onset seizures with or without secondary generalization; 49 patients (44.1%) had primary generalized seizures; 8 patients (7.2%) had two or more types of seizure. Fifty-three percent of patients had mental retardation, and 35% had abnormal findings on physical/ neurological examination. Of 98 patients, 70 (71.4%) had abnormal magnetic resonance imaging (MRI) findings. Ninety-seven percent of patients had been on polytherapy before VGB was added to treatment. VGB reduced seizure frequency by at least 50% in 33.3% of patients with partial seizures, and in 30.6% of patients with primary generalized seizures. Six of the responders with partial seizures had complete resolution of their seizures. Most common side effects included visual field defects, increased appetite and obesity. Vigabatrin seems to be more effective in partial seizures in childhood intractable epilepsy. Patients should be closely monitored regarding side effects of VGB.     

The efficacy of the ketogenic diet-1998: a prospective evaluation of intervention in 150 children

OBJECTIVE: The ketogenic diet is a high-fat, low-protein, low-carbohydrate diet developed in the 1920s for the treatment of children with difficult to control seizures. Despite advances in both the pharmacotherapy and the surgery of epilepsy, many children continue to have difficult-to-control seizures. This prospective study sought to determine the ketogenic diet's effectiveness and tolerability in children refractory to today's medications. METHODS: One hundred fifty consecutive children, ages 1 to 16 years, virtually all of whom continued to have more than two seizures per week despite adequate therapy with at least two anticonvulsant medications, were prospectively enrolled in this study, treated with the ketogenic diet, and followed for a minimum of 1 year. Seizure frequency was tabulated from patients' daily seizure calendars and seizure reduction calculated as percentage of baseline frequency. Adverse events and reasons for diet discontinuation were recorded. RESULTS: The children (mean age, 5.3 years), averaged 410 seizures per month before the diet, despite an exposure to a mean of 6.2 antiepileptic medications. Three months after diet initiation, 83% of those starting remained on the diet and 34% had >90% decrease in seizures. At 6 months, 71% still remained on the diet and 32% had a >90% decrease in seizures. At 1 year, 55% remained on the diet and 27% had a >90% decrease in seizure frequency. Most of those discontinuing the diet did so because it was either insufficiently effective or too restrictive. Seven percent stopped because of intercurrent illness. CONCLUSIONS: The ketogenic diet should be considered as alternative therapy for children with difficult-to-control seizures. It is more effective than many of the new anticonvulsant medications and is well tolerated by children and families when it is effective.     

Pyridoxal phosphate is better than pyridoxine for controlling idiopathic intractable epilepsy.

AIM: To study the difference between pyridoxine (PN) and its active form, pyridoxal phosphate, (PLP) in control of idiopathic intractable epilepsy in children. METHODS: Among 574 children with active epilepsy, 94 (aged 8 months to 15 years) were diagnosed with idiopathic intractable epilepsy for more than six months. All received intravenous PLP 10 mg/kg, then 10 mg/kg/day in four divided doses. If seizures recurred within 24 hours, another dose of 40 mg/kg was given, followed by 50 mg/kg/day in four divided doses. For those patients whose seizures were totally controlled, PLP was replaced by the same dose of oral PN. If the seizure recurred, intravenous PLP was infused followed by oral PLP 50 mg/kg/day. RESULTS: Fifty seven patients had generalised seizures (of whom 13 had infantile spasms) and 37 had focal seizure. Eleven had dramatic and sustained responses to PLP; of these, five also responded to PN. Within six months of treatment with PLP or PN, five of the 11 patients were seizure free and had their previous antiepileptic medicine tapered off gradually. Two were controlled with pyridoxine and the other three needed PLP to maintain seizure freedom. The remaining six responders needed PLP exclusively for seizure control. Six of the 11 responders to PLP had infantile spasms (46%); four of them needed PLP exclusively. The other five responders were in the remaining 81 patients with other seizure type. CONCLUSIONS: PLP could replace PN in the treatment of intractable childhood epilepsy, particularly in the treatment of infantile spasms.     

Single photon emission computed tomography in seizure disorders.

Fourteen children with various seizure disorders were studied using a cerebral blood flow tracer, 123I iodoamphetamine (0.05 mCi/kg), and single photon emission computed tomography (SPECT). In the five patients with radiological lesions, SPECT showed congruent or more extensive abnormalities. Five of the nine children with a normal scan on computed tomography had abnormal SPECT studies consisting of focal hypoperfusion, diffuse hemispheric hypoperfusion, multifocal and bilateral hypoperfusion, or focal hyperperfusion. A focal lesion seen on SPECT has been found in children with tonic-clonic seizures suggesting secondarily generalised seizures. Moreover the pattern seen on SPECT seemed to be related to the clinical status. An extensive impairment found on SPECT was associated with a poor evolution in terms of intellectual performance and seizure frequency. Conversely all children with a normal result on SPECT had less than two seizures per year and normal neurological and intellectual development.     

Pyridoxal phosphate is better than pyridoxine for controlling idiopathic intractable epilepsy

Aim: To study the difference between pyridoxine (PN) and its active form, pyridoxal phosphate, (PLP) in control of idiopathic intractable epilepsy in children. Methods: Among 574 children with active epilepsy, 94 (aged 8 months to 15 years) were diagnosed with idiopathic intractable epilepsy for more than six months. All received intravenous PLP 10 mg/kg, then 10 mg/kg/day in four divided doses. If seizures recurred within 24 hours, another dose of 40 mg/kg was given, followed by 50 mg/kg/day in four divided doses. For those patients whose seizures were totally controlled, PLP was replaced by the same dose of oral PN. If the seizure recurred, intravenous PLP was infused followed by oral PLP 50 mg/kg/day. Results: Fifty seven patients had generalised seizures (of whom 13 had infantile spasms) and 37 had focal seizure. Eleven had dramatic and sustained responses to PLP; of these, five also responded to PN. Within six months of treatment with PLP or PN, five of the 11 patients were seizure free and had their previous antiepileptic medicine tapered off gradually. Two were controlled with pyridoxine and the other three needed PLP to maintain seizure freedom. The remaining six responders needed PLP exclusively for seizure control. Six of the 11 responders to PLP had infantile spasms (46%); four of them needed PLP exclusively. The other five responders were in the remaining 81 patients with other seizure type. Conclusions: PLP could replace PN in the treatment of intractable childhood epilepsy, particularly in the treatment of infantile spasms.     

视频脑电图诊断非癫癎性发作的价值

为了解视频脑电图对非癫Jian性发作的诊断价值，对252例拟诊或排除癫Jian的患儿进行视频脑电图检查。结果：共监测到发作52例，非癫Jian性发作35例，其中睡眠期肌阵挛10例，睡眠障碍6例，习惯性抽动5例，歇斯底里2例，非癫Jian性发作性强直4例，行为异常4例，头痛、腹痛发作2例，百日咳发作、婴儿拥抱反射各1例；癫Jian性发作17例，其中强直和阵挛性发作4例，强直性发作4例，阵挛性发作3例，局限运动性发作2例，复杂部分性发作、肌阵挛性发作、婴儿痉挛、大田原综合征各1例。结果表明，视频脑电图是鉴别发作性质及类型的最有效的检查方法，也是鉴别癫Jian与非癫Jian性发作的有效方法。