Sturge-Weber综合征所致难治性癫痫的手术治疗

目的探讨Sturge-Weber综合征所致难治性癫痫的手术方法以及其疗效。方法回顾性分析2009年4月至2016年4月手术治疗30例Sturge-Weber综合征所致难治性癫痫的临床资料。结果术后随访3个月至6.1年,按Engel分级评估疗效,EngelⅠ级24例,Ⅱ级4例,Ⅳ级2例。术后未见永久性功能障碍。10例术后出现对侧肢体肌力下降1~3级,大部分在3个月后恢复至术前水平;1例出现感觉性失语,术后3个月恢复正常。结论手术是治疗Sturge-Weber综合征所致难治性癫痫的安全有效的措施。根据病人年龄、神经功能,病变部位及范围不同,采取不同的手术方式,可在控制癫痫发作的同时最大限度保留神经功能。     

儿童Sturge-Weber综合征继发癫痫的临床脑电图特征及手术治疗

目的 总结经手术治疗儿童Sturge-Weber综合征(Sturge-Weber syndrome,SWS)继发癫痫的癫痫发作特点、脑电图特征、手术方式及术后疗效,提高对此类疾病的认识.方法 回顾性分析我院儿童癫痫中心自2015年5月-2020年5月经手术治疗7例SWS的患儿资料.收集7例患儿人口学特征、癫痫发作形式、...     

儿童颅内海绵状血管瘤并发癫痫的手术治疗

目的明确儿童颅内海绵状血管瘤并发癫痫的手术治疗效果。方法回顾我院从2010年2月至2014年5月经手术治疗27例儿童颅内海绵状血管瘤并发癫痫患儿的病例资料。分析儿童海绵状血管瘤的临床特点,随访患者手术后癫痫缓解情况。手术后癫痫治疗情况用Engle’s分级进行评估,并对手术效果及预后进行分析。结果所有患者均行手术切除。术后1例基底节区病变切除后出现对侧肢体偏瘫,经治疗后到随访时神经功能已恢复至术前状态。1例术后出现颅内感染,经治疗后好转出院。手术后癫痫缓解良好,Engle’s分级1级25例,2级1例,3级1例。患者中无死亡及严重并发症患者。结论儿童颅内海绵状血管瘤并发癫痫手术治疗可有效治疗癫痫,并可避免海绵状血管瘤再次出血而改善患者预后。对多发病变及长期癫痫患者术前需充分评估。     

颞浅动脉颅内移植术治疗脑血栓一例

患者 男,42岁。因脑血栓经长期药物治疗无效住院。检查:神清,口角歪斜,流诞,左侧鼻唇沟变浅,左侧肢体肌力“0”级,左侧腹壁反射、提睾反射消失,左侧上下肢病理反射( ),右侧颈动脉造影示颈内动脉虹吸弯处完全闭塞。1785年9月5日在局麻下行右颞浅动脉颅内移植脑贴敷术。术后第4天,患者述左侧肢体麻胀感,肌力恢复至Ⅱ-级,第12天左上肢肌力Ⅱ级,左下肢肌力Ⅲ级,13     

大脑镰旁脑膜瘤术后丙戊酸钠中毒1例

正颅脑手术,特别是幕上开颅手术,为预防术后癫痫常预防性应用抗癫痫药物,而丙戊酸钠是临床上最常用的预防和控制癫痫的药物之一。本文报道大脑镰旁脑膜瘤术后丙戊酸钠严重中毒1例。1病例资料55岁女性,因头晕、左侧肢体乏力1个月入院。入院体格检查:左侧肢体肌力约4级,未发现其他神经系统阳性体征。入院后头部MRI检查示右侧大脑镰旁占位,大小约3cm×4 cm,位于右侧中央前回前方,且中央前回向后外侧受压明显,考虑脑膜瘤。常规预防癫痫治疗(口服丙戊酸钠缓释片,0.5 g,2次/d)。     

皮瓣塌陷综合征患者颅骨修补术后继发蛛网膜下腔出血1例

<正>皮瓣塌陷综合征(sinking skin flap syndrome,SSFS)是去骨瓣减压术后的并发症之一,颅骨修补术是防治SSFS的有效方法。本文报道1例SSFS患者行三维钛网颅骨修补术后出现蛛网膜下腔出血的患者。1病例资料患者,女性,64岁,因突发意识障碍、呕吐、左侧肢体偏瘫2 d入院。头部CT扫描显示右侧大脑半球梗死(图1A)。急诊行右额颞顶去骨瓣减压术,术后2 d患者神志清楚。经过综合治疗,神经功能逐步恢复,左侧肢体肌力恢复到3级,随     

大脑半球切除术治疗2例4月龄症状性 West综合征报告及文献复习

目的：对2例4月龄症状性West综合征实施大脑半球切除术后随访,并结合文献分析大脑半球切除术后癫痫控制效果及神经功能恢复状况。方法2例4月龄患儿均为一侧半球广泛病变,具有典型West综合征临床特点,进行相关检查及详细术前评估后,在导航系统引导下行功能性大脑半球切除术。结果手术过程顺利,术后2例患者发作消失。术后分别随访31个月、28个月,无癫痫发作,2例肢体运动改善,智力及认知功能明显改善,复查脑电图提示背景明显改善。均未见高幅失律。结论结合文献复习,提示对于半球病变导致的West综合征早期行手术治疗可获得满意的效果。术后不仅癫痫发作可以完全消失,而且患儿的运动、智力和认知功能在不同程度上都有所提高,生活质量明显改善。     

选择性杏仁核-海马切除术对内侧颞叶癫痫病人神经心理学的影响

目的 探讨选择性杏仁核-海马切除术（SAH）对内侧颞叶癫痫病人认知功能的影响。方法 回顾性分析2009年1月~2017年5月接受SAH治疗的67例内侧颞叶癫痫的临床资料,术前、术后3个月和术后1年均行详细的神经心理学评估,包括智商、记忆商和语言功能。结果 36例行左侧SAH,31例行右侧SAH。术后1年,癫痫控制效果达到Engel分级Ⅰ级50例,Ⅱ级7例,Ⅲ级8例,Ⅳ级2例。术后3个月,左侧手术病人言语功能、记忆商较术前明显降低（P<0.05）,右侧手术病人言语理解指数、语义流畅性测验明显改善（P<0.05）;术后1年,无论是左侧手术病人,还是右侧手术病人,智商、记忆商、言语功能较术后3个月略改善（P>0.05）;手术前后视觉记忆均无明显变化（P>0.05）。结论 海马硬化性内侧颞叶癫痫行SAH后,左侧手术病人会出现比较明显的言语和记忆功能减低。     

Rasmussen脑炎(附1例报告及文献复习)

目的 探讨Rasmussen脑炎的临床特点及诊断。方法 回顾分析1例Rasmussen脑炎患儿的临床资料,并复习相关文献进行分析。结果 女性,7岁。起病时表现为间断右侧眼睑抽动伴右侧肢体抖动,起病后有认知损害,予抗癫■药及免疫抑制剂等治疗病情好转。病程约6个月时加重,出现累及右侧面部及右侧肢体的局灶性癫■持续状态和语言功能障碍。患儿有定向障碍,记忆力减退,右侧肢体肌力4级,右侧巴氏征阳性。头颅MRI病初时仅有左侧额叶局部异常信号,一过性累及左侧颞叶,逐渐发展为左侧大脑半球萎缩。脑电图可见左侧半球弥漫性慢波,夹杂尖波、棘波发放。行左侧额、顶、岛叶癫■灶切除术及大脑半球离断术后,患儿再无癫■发作。能独走,右侧肢体肌力4级,精细动作差,能说简单句。结论 临床表现为局灶性癫■持续状态、进行性神经功能和认知损害,头颅影像学表现为大脑皮质进行性局部萎缩,脑电图表现为一侧大脑半球慢波可伴尖波、棘波,应考虑Rasmussen脑炎。     

硬膜下脓肿误诊为慢性硬膜下血肿1例

正1病例资料男性,67岁。因发现言语不清、右侧肢体无力3 d入院。入院体格检查:神志清楚,精神差,言语不清,思维力、理解力、定向力、记忆力明显减退;颈抵抗,左侧肢体肌力5级,右侧肢体肌力3级,四肢肌张力正常,右侧巴氏征阳性。头颅CT示左侧额颞顶部硬膜下慢性血肿。初步诊断为左侧额颞顶部慢性硬膜下血肿,给予脱水、营养神经等治疗。入院2 h突发四肢抽搐,持续3～4 min,考虑癫痫发作,给予地西泮注     

Hemisphärotomie bei einer 17-jährigen Patientin mit Sturge-Weber-Syndrom

Hemispherotomy is predominantly carried out in childhood. It can generally be assumed that preoperative neurological deficits (homonymous hemianopsia, hemiparesis) are present. We report on a 17-year-old patient with refractory epilepsy with Sturge-Weber syndrome who was treated with a modified hemispherotomy despite absence of hemiparesis and additional idiopathic generalized epilepsy. Postoperatively (11 months) the patient was seizure-free and, despite postoperative spastic paresis of the left foot, reported clearly improved everyday functioning and quality of life.     

Late complications of hemispherectomy: report of a case relieved by surgery

A case of Sturge-Weber disease treated with left hemispherectomy presented, 11 years later, with complications related to delayed intracranial haemorrhage. A loculation syndrome of the right lateral ventricle was detected and it was corrected by a ventriculoatrial shunt operation. The side of the hemispherectomy was evacuated of all the chronic products of haemorrhage, including the subdural membrane. The patient was relieved of her symptoms. It is considered that complications related to delayed haemorrhage after hemispherectomy are remediable.     

手术治疗不伴面部血管瘤的Sturge-Weber综合征1例

目的 总结不伴面部血管瘤的致(癎)性Sturge-Weber综合征的诊治经验.方法 报告1例女性病人,9岁,表现为药物难治性癫(癎)1年.发作形式为微笑-意识丧失-倒地抽搐.不伴面部和全身血管瘤.MRI显示:左侧顶枕交界区皮质病变,T1W呈等信号,增强后病变沿脑回强化;PET显示:病变及周围葡萄糖代谢降低,病变呈"电车轨道样"钙化.在神经导航和术中皮质脑电图(ECoG)监测下行左侧顶枕叶致(癎)灶切除术.结果 病理报告为软脑膜血管瘤.随访11个月,病人无癫(癎)发作,无严重手术并发症发生.结论 应加强对不伴面部血管痣性Sturge-Weber综合征的认识.手术切除致(癎)灶是治疗致(癎)性Sturge-Weber综合征的有效方法.     

Outcome of infants with unilateral Sturge-Weber syndrome and early onset seizures

Patients with Sturge-Weber syndrome often present with seizures during the first year of life. Currently, only patients with clinically significant seizures who do not respond to medical treatment are candidates for early epileptic surgery. However, a delay of surgical treatment may result in cognitive deterioration. We studied the correlation between parameters and outcome of seizures to re-examine the criteria for early epilepsy surgery. We performed a retrospective chart review combined with telephone interviews of parents of all Israeli infants with unilateral Sturge-Weber syndrome and early onset seizures, and we examined whether age of seizure onset and seizure intensity were correlated with cognitive level and the degree of hemiparesis at follow-up. We recruited a total of 15 patients with unilateral Sturge-Weber syndrome and early onset seizures, five of whom underwent epilepsy surgery. The mean follow-up period of all the patients was 15 years: six patients had normal intelligence, four had borderline cognitive level, three had mild mental retardation and two had moderate mental retardation. Eight of the ten non-operated patients still experience seizures at follow-up. Cognitive delay was significantly correlated with seizure intensity in the early period, but not with the age of seizures onset, the degree of hemiparesis, or the presence of ongoing seizures. We conclude that high seizure intensity in young patients with Sturge-Weber syndrome is a prognostic marker for mental deterioration.     

Catastrophic Epilepsy in Childhood

Summary: Although for most children epilepsy is a relatively benign disorder, for some, epilepsy can be designated as "catastrophic" because the seizures are so difficult to control and because they are strongly associated with mental retardation. The catastrophic childhood epilepsies include uncommon disorders such as early infantile epileptic encephalopathy with suppression burst, severe myoclonic epilepsy of infancy, and epilepsy with myoclonic-astatic seizures. There are other syndromes that are relatively common such as infantile spasms, Lennox-Gastaut syndrome, and Sturge-Weber syndrome. Many children with catastrophic epilepsy have the seizures as a result of underlying brain abnormalities that will inevitably lead to mental retardation whether or not they have seizures. In some patients, however, the mental retardation appears to be caused by the seizures. Developmental plasticity provides children with an opportunity to recover from significant brain injuries. However, the plasticity may also be the cause of the mental retardation. In such patients, control of the seizures may lead to more normal intellectual development. Thus, every effort should be made to control seizures in children with catastrophic epilepsy.     

Traumatic Korsakoff syndrome

A case of Korsakoff's syndrome occurring after a fall is reported. The patient was comatose on admission. Cerebral contusions were seen in the frontal lobes bilaterally, left temporal lobe, right caudate nucleus and cerebellar vermis. Cerebral blood flow (CBF) study showed diffuse hyperaemia. Although his conscious state improved gradually, he showed disorientation, antegrate and retrograde amnesia and confabulation 2.5 months later and was diagnosed as having Korsakoff's syndrome. Magnetic resonance imaging (MRI) and CBF studies during this period showed damage in the midbrain and general hypoperfusion. He recovered gradually, confabulation disappeared but a mild memory disturbance remained; hypoperfusion on CBF studies also improved up to the normal range. Among the limbic diencephalic structures, the frontal lobes were thought to be involved most prominently in this case of Korsakoff syndrome.     

Sturge-Weber syndrome: altered blood vessel fibronectin expression and morphology

Sturge-Weber syndrome presents with vascular malformations of the brain, skin, and eye. Fibronectin has potent effects on angiogenesis, vessel remodeling, and vessel innervation density. To determine fibronectin expression in the blood vessels of Sturge-Weber syndrome brain and skin tissue and to quantify the density and circumference of Sturge-Weber syndrome blood vessels by type compared with controls, we performed in situ hybridization for fibronectin messenger ribonucleic acid (RNA) expression on six Sturge-Weber syndrome cortical brain samples, six epilepsy brain samples, skin from two port-wine stain skin lesions, and two normal skin samples from two subjects with Sturge-Weber syndrome. Fibronectin messenger RNA was expressed in blood vessels and endothelial cells in the parenchyma of both Sturge-Weber syndrome and control brain tissues and in skin samples. Fibronectin expression was significantly reduced by 23% in the Sturge-Weber syndrome meningeal vessels compared with the epilepsy controls (P < .01). Fibronectin expression was significantly increased by 19% in the Sturge-Weber syndrome parenchymal vessels compared with the epilepsy controls (P < .05). No difference was found in the expression of fibronectin in port-wine stain skin blood vessels. The density of leptomeningeal blood vessels in the Sturge-Weber syndrome brain tissue samples was 45% greater than in the epilepsy samples (P < .05). Blood vessel circumference was significantly decreased in the Sturge-Weber syndrome meningeal vessels compared with the controls (27%; P < .05). When blood vessels from different brain regions were compared, fibronectin expression was decreased in Sturge-Weber syndrome meningeal vessels and was increased in the parenchymal vessels. Altered blood vessel fibronectin expression in Sturge-Weber syndrome could contribute to abnormal vascular structure and function in this disorder.     

Myoclonic-astatic epilepsy in a child with Sturge-Weber syndrome

A child with Sturge-Weber syndrome and a left occipital leptomeningeal angioma developed focal seizures at 6 years of age that responded initially to oxcarbazepine. After 7 months of seizure freedom, the patient developed typical myoclonic-astatic seizures associated with generalized electrographic discharges, which worsened as oxcarbazepine was increased. The seizures and electroencephalogram improved dramatically in 3 weeks as the oxcarbazepine was withdrawn and valproic acid was initiated. This case demonstrates the importance of recognizing that children with epilepsy due to focal lesions can develop secondary bilateral synchrony that can be aggravated by medications that are effective for partial seizures. In such cases, treatment with a broad-spectrum antiepileptic may be advantageous.     

Sturge-Weber disease: operative indications and surgical results

Patients with Sturge-Weber disease with epilepsy refractory to medical therapy have been reported to develop slowly progressive neurological deficits and ultimately become moderately or severely disabled. We studied six patients with Sturge-Weber syndrome including its incomplete form. Three out of six patients with Sturge-Weber syndrome revealed evolution of calcified angioma on computed tomography. All of the three cases developed medically intractable seizures. Total and/or subtotal hemispherectomy was performed for these three cases. The surgery was effective for controlling seizures in all three cases except one with infantile spasm with hypsarrythmia on electroencephalogram who is still on anticonvulsant. Although the unremitting deterioration in mental retardation and hemiparesis was not effectively prevented by the surgery possibly because the timing of surgery was delayed in one case, the surgery not only stopped the frequent medically-intractable seizures, but also dramatically prevented the psychomotor deterioration in the other case. Although the role of surgical treatment for the patients with Sturge-Weber syndrome remains poorly defined, one can expect excellent results if the indications for surgery are carefully analyzed and hemispherectomy is performed on an individual basis.     

A volumetric magnetic resonance imaging study of brain structures in children with Down syndrome

BACKGROUND AND PURPOSE : Down syndrome (DS) is the most common genetic cause of mental retardation with deficits in language and memory. Mental retardation of varying degrees is the most consistent feature of DS. The objective of this study was to use high-resolution magnetic resonance imaging (MRI) techniques to investigate the volumes of the hippocampus, amygdala, and temporal and frontal lobes in children with DS compared with healthy children. MATERIAL AND METHODS : MRI of 49 patients was reviewed prospectively. The study included 23 children with DS (9 girls and 14 boys, mean age 6.7 ± 3.7 years) and 26 healthy children (11 girls and 15 boys, mean age 8.3 ± 2.4 years). Volumes of the right and left hippocampus, the right and left amygdala, temporal and frontal lobes and the total brain volume were measured by a radiologist who was unaware of the diagnosis. RESULTS : Total brain volume in children with DS was significantly lower compared with controls. It was associated with significantly lower volume of the frontal and temporal lobes. Children with DS had a significantly smaller right and left hippocampus volume and a significantly smaller right and left amygdala volume than did the control group. We also found a negative correlation between mental retardation and volume of the right hippocampus. CONCLUSIONS : The presence of these abnormalities from an early age contributes to the specific cognitive and developmental deficits seen in children with DS.