Craniosynostosis following hemispherectomy in a 2.5-month-old boy with intractable epilepsy

The authors report on the case of a 6-week-old boy who presented with infantile spasms. At 2.5 months of age, the patient underwent a right hemispherectomy. Approximately 3 months postoperatively, the patient presented with left coronal craniosynostosis. Subsequent cranial vault remodeling resulted in satisfactory cosmesis. Four years after surgery, the patient remains seizure free without the need for anticonvulsant medications. The authors believe this to be the first reported case of iatrogenic craniosynostosis due to hemispherectomy, and they describe 2 potential mechanisms for its development. This case suggests that, in the surgical treatment of infants with intractable epilepsy, minimization of brain volume loss through disconnection techniques should be considered, among other factors, when determining the best course of action.     

Hemimegalencephaly and intractable epilepsy: complications of hemispherectomy and their correlations with the surgical technique. A report on 15 cases

Hemispherectomy is required in most cases of hemimegalencephaly in order to control epilepsy refractory to medical treatment. Although there is a general agreement on the effectiveness of the procedure in controlling the seizure disorder, the choice of the surgical technique is still a subject of debate. In particular, anatomical hemispherectomy is blamed to be associated with a higher incidence of surgical complications, namely hydrocephalus and hemosiderosis, than other less ablative operations such as functional hemispherectomies. A series of 15 children with hemimegalencephaly, who had undergone anatomical hemispherectomy (11 cases), functional hemispherectomy (2 cases), and hemidecortication (2 cases) at the Pediatric Neurosurgery Section, Catholic University Medical School, Rome, is reported. Twelve of these patients presented with one or more complications in their postoperative course. Temporary complications, which resolved spontaneously or following medical therapy, included fever, wound breakdown, worsening of preoperative motor deficit, unilateral third cranial nerve deficit, dystonia, and anemia. In 8 patients, postoperative complications led to a second surgical procedure. A CSF shunt was necessary in 5 children, to control a secondary hydrocephalus. Two subjects underwent a toilette of the residual cavity because of persisting chemical abnormalities in CSF parameters. In a child a cranioplasty procedure was necessary as a consequence of an infection of the hemicranial bone flap. There was no apparent correlation between the rate and the type of complications with a specific surgical procedure. On the other hand, the age factor appeared to play an important role in the occurrence of secondary hydrocephalus, as all 5 children with this complication were less than 9 months old at the time of the hemispherectomy. Surgical mortality was nil in this series.     

��ͯ�����������ҩ�����Ʋ���

??Intractable epilepsy often requires a combination of drugs. To reduce the seizures in children?? minimize the side effects of drugs?? and improve the patient’s quality of life?? it is very important to be familiar with the main mechanisms and pharmacokinetics of antiepileptic drugs?? and correctly choose drugs according to the types of seizure and syndrome. Also every time to add a new drug?? it must consider the dosage and speed of titration of the drug to avoid serious adverse effects.     

Clinical experiences with topiramate in children with intractable epilepsy.

At a tertial referral epilepsy centre 39 children were consecutively enrolled in an open add-on study with topiramate (TPM). All children had intractable epilepsy; the mean seizure frequency was 36 per month, and 31 children were treated with polypharmacy. All but five children were mentally retarded. The initial dose of TPM was 0.5-1 mg/kg daily, slowly titrated with 1-3 mg/kg daily every second week with an estimated target dose of 10 mg/kg daily. At latest follow-up 19 children continued on TPM, three (8%) were seizure-free, eight (21%) had a seizure reduction of more than 50% and eight (21%) improved their general condition. Mean follow-up was 13 months (range 9-36 months). Seizure reduction was seen in focal as well as generalized epilepsies. Adverse effects were reported in 21 cases (54%), weight loss and sedation being most frequent. The mean steady state dose in the children continuing on TPM was at latest follow-up: 14 mg/kg daily (< 5 years), 10 mg/kg daily (5-7 years), 5.8 mg/kg daily (8-17 years). The corresponding plasma level varied from 3 to 45 mumol/litre, and a significant correlation between the daily dose in mg/kg and the plasma level was found. Two patients with progressive myoclonus epilepsy are described separately; one had a dramatic general improvement. It is concluded that TPM seems to be a promising new broad-spectrum anti-epileptic drug, which is efficacious even in epilepsy syndromes, intractable to other new anti-epileptic drugs such as vigabatrin and lamotrigine.     

Effective everolimus treatment of inoperable, life-threatening subependymal giant cell astrocytoma and intractable epilepsy in a patient with tuberous sclerosis complex

We present successful everolimus treatment of a huge subependymal giant cell astrocytoma in a 10-year old boy with tuberous sclerosis complex. The patient underwent several partial tumor resections complicated by intraoperative cardiac arrest. The tumor has been regrowing and produced severe clinical symptoms. Everolimus treatment resulted in marked tumor regression, significant improvement in patient’s ambulation and cessation of seizures. Moreover, the therapy was well tolerated. These findings indicate that everolimus treatment should be considered as a therapeutic option alternative to surgery in patients with tuberous sclerosis complex.     

Surgery for intractable epilepsy: issues and outcome

For certain forms of childhood epilepsy that remain uncontrolled despite adequate treatment with standard antiepileptic medication, surgical therapy should be considered as a potential avenue of treatment. The prognosis for seizure control after early surgery is generally favorable and is at least comparable with that of adults. Remissions are often long-standing or permanent. The results are particularly beneficial if a discrete region of abnormal epileptogenic tissue can be excised. The three major procedures--focal resection, corpus callosotomy, and hemispherectomy--have all been performed successfully in children, and their complication rates are low. With the exception of the obvious benefit conferred by alleviating seizures at a younger age, earlier surgery also improves later psychosocial status and adaptive function. By contrast, persistent seizure activity decreases the likelihood of achieving a functionally independent and economically productive adult life. The psychosocial benefits therefore constitute the most important argument in favor of early surgical intervention. Although relatively little is known about the neuropsychologic consequences of early surgery, there are few deleterious long-term cognitive or behavioral deficits if surgery is uncomplicated. However, relatively little is known about neural reorganization after early surgical excision or hemispheric disconnection, and this remains the greatest challenge for the future.     

38例儿童颞叶难治性癫痫病理分析#br#

目的：探讨儿童颞叶难治性癫痫的病理特征。方法回顾性分析2011年10月至2013年10月行手术治疗的38例颞叶癫痫患儿的病理资料。结果38例患儿年龄2.5~14岁,男21例、女17例。病理诊断局灶性皮层发育不良(FCD)21例,其中FCDⅠa1例、FCDⅠb2例、FCDⅡa2例、FCDⅡb3例、FCDⅢa 5例、FCDⅢb 1例、FCDⅢc 1例、FCDⅢd 6例;微皮层发育不良3例;单纯海马硬化3例;神经系统肿瘤7例,其中胚胎发育不良性神经上皮瘤5例,星形胶质瘤2例;脑炎2例;蛛网膜囊肿2例。结论 FCD是引起儿童颞叶难治性癫痫最常见的原因,其中FCDIIId型为最常见的亚型。     

��ͯ���������������Ӧ֤������ʱ��ѡ��

??Epilepsy is a common chronic neurological disorder in children. Among them?? about 20% are drug resistant. Epilepsy surgery is often the most effective treatment option for medically intractable epilepsy. Although there are many common features between pediatric and adult epilepsy surgery??there are also many differences unique to children in etiology?? neuro-imaging?? electrical-clinical features and operative techniques. These differences are critical during the many stages of the pediatric epilepsy surgery?? especially for choosing correct surgical indications and timing. On the other hand?? the presurgical discussion and decision should be best performed at a special pediatric epilepsy center with the input of a team of pediatric neurologists?? surgeons and some other specialists.     

儿童癫痫的手术治疗

目的 总结分析儿童期顽固性癫痫手术治疗效果和经验。方法 回顾性分析2001年7月～2002年11月在我科接受手术治疗的25例14岁以下的顽固性癫痫病例，统计患儿的发病、检查及手术治疗情况，手术方式的选择应依据患儿术前脑电图与术中脑电图的定位情况而进行。结果 本组25例患儿，年龄3～14岁，病程平均5．3年，影像学异常9例，占36．0％(9／25)，智商明确缺陷12例，占48．0％(12／25)，以强直一痉挛性发作为主16例，占64．0％(16／25)。病灶位于额叶10例，占40．0％(10／25)，颞叶6例，占24．0％(6／25)，额颞叶5例，占20．0％(5／25)，顶叶和枕叶各2例，占8.0％(2／25)。手术行病灶切除及前颢叶切除11例，占44．0％(11／25)，选择性海马一杏仁核切除4例，占16．0％(4／25)，胼胝体切开15例，占60.0％(15／25)，单纯多处软膜下横切2例，占8，0％(2／25)。术后出现一过性对侧轻瘫4例，一过性语言不利2例。术后疗效满意23例占92.0％(23／25)，显著改善1例占4.0％(1／25)，良好1例占4．0％(1／25)。结论 合理的选择手术方式，对儿童期顽固性癫痫手术治疗，手术效果良好，非常安全。     

小儿难治性癫痫62例药物治疗分析

目的评价抗癫痫药单药大剂量及联合用药治疗小儿难治性癫痫的疗效.方法在血药浓度监测下对54例患儿投予大剂量卡马西平(CBZ)或丙戊酸钠(VPA),无效者及呈混合类型发作者采取联合用药方式.结果单用CBZ者11%发作获完全控制,单用VPA者15%发作停止.这些患儿的血药浓度均已达到或超过有效浓度的上限.全组单药或联合用药共17例(27%)患儿获控制,16例(26%)发作减少50%以上.结论难治性癫痫患儿可以采用单药大剂量治疗及联合用药的方法,但应注意监测血药浓度,以避免发生中毒反应.     

儿童难治性癫癎致癎灶的病理改变

目的：研究儿童难治性癫癎致癎灶的病理改变及其临床意义。方法：儿童难治性癫癎患者 30 例,手术切除致癎灶,在光镜和电镜下观察其病理学改变。结果：致癎灶病理组织学检查发现皮质发育不良 14 例（47%）,海马硬化 11 例（37%）,胚胎发育不良性神经上皮瘤1例（3%）,节细胞胶质瘤 1 例（3%）,脑软化 3 例（10%）。电镜下可见海马及海马外皮层突触数量及结构改变,神经元减少及核固缩,星形胶质细胞肿胀、退变,线粒体结构改变。 结论：海马及海马外皮层病理结构改变,特别是突触重塑,可能是难治性癫癎反复自发性发作的形态学基础,该病理变化与癎样活动的产生及神经系统的兴奋抑制平衡失调有关。