Experience with the International League Against Epilepsy Classifications of Epileptic Seizures (1981) and Epilepsies and Epileptic Syndrome (1989) in Epileptic Children in a Developing Country

Four hundred eighty-three epileptic children attending the Pediatric Epilepsy Clinic at Bai Jerbai Wadia Hospital for Children, Bombay, India were classified according to the International League Against Epilepsy (ILAE) classification of epileptic seizures (1981) and epilepsies and epileptic syndromes (1989). The predominant seizures were partial (53.6), generalized (40.3%), and unclassifiable (6%). In epilepsies and epileptic syndromes, 55.3% were partial, 27% were generalized, 13.5% were undetermined, and 4.1% were special syndromes. Although our results were similar in many respects to those of other reported series, some differences were observed in the incidence of partial and generalized seizures, and partial and generalized epileptic syndromes and their subgroups, such as idiopathic, symptomatic, and cryptogenic partial syndromes, idiopathic generalized syndromes, and symptomatic specific syndromes. These differences are probably due to different age limits, methods of case ascertainment and inclusion criteria, different genetic and environmental factors, variable interpretation of clinical and EEG features, and lack of facilities for investigation in developing countries. Despite various limitations, we were able to classify most cases; the ILAE classification can be used in developing countries so that comparison can be made with other studies.     

Hemimegalencephaly: clinical implications and surgical treatment

Introduction Hemimegalencephaly (HME) is a quite rare malformation of the cortical development arising from an abnormal proliferation of anomalous neuronal and glial cells that generally leads to the hypertrophy of the whole affected cerebral hemisphere. The pathogenesis of such a complex malformation is still unknown even though several hypotheses are reported in literature.Background HME can occur alone or associated with neurocutaneous disorders, such as neurofibromatosis, epidermal nevus syndrome, Ito’s hypomelanosis, and Klippel–Trenonay–Weber syndrome. The clinical picture is usually dominated by a severe and drug-resistant epilepsy. Other common findings are represented by macrocrania, mean/severe mental retardation, unilateral motor deficit, and hemianopia. The EEG shows different abnormal patterns, mainly characterized by suppression burst and/or hemihypsarrhythmia. Although neuroimaging and histologic investigations often show typical findings (enlarged hemisphere, malformed ventricular system, alteration of the normal gyration), the differential diagnosis with other disorders of the neuronal and glial proliferation may be difficult to obtain. Hemispherectomy/hemispherotomy is the most effective treatment to control seizure, and it also seems to provide good results on the psychomotor development when performed early, as demonstrated by the literature review and by the reported personal series reported here (20 children). The surgical therapy of HME, however, is still burdened by a quite high complication rate and mortality risk.     

Intrahippocampal Injection of Endothelin-1: A New Model of Ischemia-induced Seizures in Immature Rats

Summary:  The goal of this study was to develop a new model of ischemia-induced seizures in immature rats using injection of vasoconstrictor Endothelin-1 (ET-1) into the brain. ET-1 (10, 20, or 40 pmol) was infused into the left dorsal hippocampus of freely moving Wistar rats 12 (P12) and 25 (P25) days old. Animals were then video/EEG-monitored for 100 min and monitoring was repeated 22 h later. Parameters of electrographic seizures (frequency and mean duration) as well as pattern of their behavioral correlates were evaluated. The pattern of behavioral seizures was used to develop model-specific scoring system. Cresyl violet and Fluoro Jade-B-staining were used to evaluate brain damage. Extension of the lesion was correlated with seizure severity. After ET-1-injection, seizures occurred in 83–100% animals of all age-and-dose groups and persisted for 24 h except P12 rats with 10 pmol. There were no differences in average seizure duration (18–40 s) or seizure frequency (3–7 seizures/100 min) among individual dose-groups. Between the 1st and 2nd observation period, total seizure duration decreased in 71% of P12 and 47% of P25 rats. Electrographic seizure activity was most frequently accompanied by clonus, incidence of more severe convulsions (barrel rolling or generalized clonic seizures) increased with dose of ET-1. Morphologic examination did not reveal any dose-related difference in damage severity, hippocampal damage was however more extensive in P12 compared to P25 animals. Seizure severity correlated positively with severity of the damage in both age groups. Our study presents focal injection of ET-1 into the brain as a new and practical model of ischemia-induced seizures in immature rats.     

一例促皮质素静脉外渗至局部皮肤坏死的案例解析与改进

对1例癫痫性痉挛患儿采用促皮质素静脉给药时发生静脉外渗至局部皮肤坏死的案例进行解析。针对事件发生原因,制定并采取措施:静脉穿刺难度事前评估,优化临床监护,加强相关知识培训,以肌肉注射代替静脉滴注等,避免了此类事件的再次发生,提升了临床合理用药水平,确保了患者安全。     

膀胱颈部电切术治疗膀胱颈梗阻

目的：探讨经尿道电切术治疗膀胱颈梗阻的方法和治疗效果。方法:根据14例膀胱颈部挛缩的情况,分别采用膀胱颈口切沟术,膀胱颈口号下半环切除加12点切沟术。结果：12例排尿通畅,肾脏、输尿管、膀胱的功能形成恢复正常;2例合并糖尿病者排尿困难症状改善,残余悄量明显减少。未出现尿失禁症状。结论：经尿道膀胱颈口电切术治疗膀胱梗阻是安全有效的治疗方法,根据颈部狭窄程度的不同,选择不同的电切方法,可以提高手术治疗效果。     

A prospective randomised trial on the effect of monitoring plasma anticonvulsant levels in epilepsy

Summary To evaluate whether knowledge of plasma levels of anti-epileptic drugs has an effect on therapeutic outcome, 127 epileptic outpatients were randomly assigned to two groups (A and B). Plasma levels of group A were reported to the treating physician who attempted to keep the plasma levels within the therapeutic range. The treating physician was not informed of the results of plasma lavel determinations of group B. Data from 105 patients were available for assessment at the end of the study year. Therapeutic results of groups A and B were not significantly different. The reduction in seizure frequency was associated with an increase in plasma concentrations of the anti-epileptic drugs. Thus, under the conditions of the study, knowledge of plasma levels of anti-epileptic drugs did not further improve therapeutic results.

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Zusammenfassung Um festzustellen, ob die Kenntnis des Plasmaspiegels der Antiepileptika das Therapieergebnis verbessern kann, wurden 127 ambulant behandelte Patienten mit Epilepsie in randomisierter Reihenfolge in zwei Gruppen eingeteilt (A und B). Die Plasmaspiegel der Antiepileptika in Gruppe A wurden dem behandelnden Arzt mitgeteilt, der versuchen sollte, die Plasmaspiegel in den Therapeutischen Bereich zu bringen. Die Ergebnisse der Plasmaspiegelbestimmung in Gruppe B (Kontrollgruppe) wurden dem behandelnden Arzt nicht mitgeteilt. Am Ende des Untersuchungsjahres konnten die Daten von 105 Patienten ausgewertet werden. Das Behandlungsergebnis von Gruppe A und von Gruppe B war am Ende des Beobachtungsjahres nicht signifikant verschieden. Die Abnahme der Anfallshäufigkeit ging mit einem Anstieg der Plasmakonzentration der Antiepileptika einher. Somit konnte unter den Bedingungen dieser Studie das Therapieergebnis durch die Kenntnis der Plasmaspiegel der Antiepileptika nicht weiter verbessert werden.

     

Plasma concentrations of valproate during maintenance therapy in epileptic children

Summary A total of 20 children with various types of epilepsy were treated with valproate, 11 with monotherapy and 9 with valproate in combination with phenobarbitone, phenytoin, or carbamazepine. Valproate was given either every 8 or 12 h. At least two different dose levels were tried in each patient. The pharmacokinetics of valproate during the interval between doses was determined using a gas chromatographic technique. The clinical effect of the treatment was assessed by interviewing the parents.The plasma concentrations showed considerable fluctuation during the intervals between doses. The mean increase from pre-administration to peak level was 82% when the dose interval was 12 h, and 62% when it was 8 h. The mean plasma half-life of valproate, using a one-compartment model, was 10.9±1.3 h (mean±SD). The plasma half-life of valproate was decreased when the drug was combined with the other anti-epileptics. The calculated area under the concentration versus time curve was linearly related to dose, both in a single patient on four dose levels and when different patients were compared. The clinical effect of valproate monotherapy was best in patients with absences, usually good in myoclonus and less favourable in other types of epilepsy. For children with absences, the optimal dose range of valproate was between 20 and 40 mg/kg/24 h. In comparison, the myoclonic types of epilepsy needed a slightly higher dose level, between 30 and 60 mg/kg/24 h. In the latter group a therapeutic window seems to exist, since patients below and above the suggested dose levels were not well-controlled. Therapeutic monitoring of valproate does not appear meaningful when the drug is used as monotherapy. However, in combination therapy, determination of the plasma levels of all anti-convulsants used may be helpful. The large fluctuations of valproate during a dose interval must be taken into consideration when the clinical effects are analysed.Supported by the Swedish Medical Research Council (Project No. 522), Stiftelsen Margarethahemmet, and Sällskapet Barnavård     

癫痫儿童认知学习能力的研究

目的:探讨癫痫儿童的认知学习状况。方法:采用学习障碍筛查量表(PRS)测量60例癫痫儿童和60例健康对照,并用丹麦维迪Keypoint诱发电位仪检测其事件相关电位P300。结果:①癫痫儿童PRS量表总分、言语得分及非言语得分均较正常儿童降低,差异有显著性(P<0.01);②癫痫儿童P300潜伏期较正常儿童延长,差异有显著性(P<0.01);③全面性发作组与部分性发作组比较,P300与PRS量表均未见显著性差异(P>0.05)。结论:本研究表明癫痫儿童存在学习障碍,PRS量表与P300可从不同角度反映癫痫儿童的认知学习状况。     

Additional observation of a de novo pathogenic variant in KCNT2 leading to epileptic encephalopathy with clinical features of frontal lobe epilepsy

IntroductionKCNT2 was recently recognized as a gene associated with neurodevelopmental disorder and epilepsy.Case reportWe present an additional observation of a 16-year-old male patient with a novel de novo KCNT2 likely pathogenic variant and review the five previously reported cases of de novo variants in this gene.DiscussionWhole exome sequencing identified the missense variant c.725C > A p.(Thr242Asn), which was confirmed by Sanger sequencing. Our patient has a refractory stereotyped and monomorphic type of hyperkinetic focal motor seizure, similar to what is seen in frontal lobe epilepsy, occurring only during sleep. This type of seizure is not usually seen in epileptic encephalopathies.     

乌拉地尔复合艾司洛尔控制性降压在高血压患者脊柱手术中的应用

目的评估在经后路椎体间植骨、椎弓根钉内固定术中,高血压患者脊柱手术中乌拉地尔复合艾司洛尔控制性降压的可行性。方法 60例经后路椎体间植骨、椎弓根钉内固定术高血压患者随机分为两组,即U组(单纯乌拉地尔组)、U-E组(乌拉地尔复合艾司洛尔组),各3O例。手术开始时即行控制性降压,U组:先静脉注射盐酸乌拉地尔0.4mg/kg,此后持续泵注乌拉地尔,维持初始量20～25μg/(kg·min);U-E组:先静脉注射盐酸乌拉地尔0.4mg/kg、艾司洛尔10mg,此后持续泵注盐酸乌拉地尔20～25μg/(kg·min),同时持续泵注艾司洛尔,速率20～30μg/(kg·min)。使平均动脉压(MAP)降至目标血压(MAP降至基础血压的70%)。此后根据血压情况调整用药速度,至手术主要步骤操作完毕、术野止血完毕后停止降压。记录开始降压前即刻(T0)、降压5min(T1)、降压10min(T2)、降压30min(T3)、降压60min(T4)、停止降压后15min(T5)6个时间点血压(SBP、DBP、MAP)、心率(HR)、中心静脉压(CVP)的变化及晶胶体输入量、尿量、出血量和输血量、手术时间,并进行血气分析。结果两组患者MAP的下降均显著低于降压前(P<0.01);降压期间,U组心率在T1、T2、T3时增快,与T0比较有差异有统计学意义;U-E组的心率显著低于U组(P<0.05)。两组术中出血量、尿量、输血量比价,差异无统计学意义(P>0.05)。结论乌拉地尔复合艾司洛尔控制性降压应用于高血压患者脊柱手术安全、有效,可控性良好。