3种不同方式治疗小儿难治性癫痫的临床疗效及安全性分析

目的探讨3种不同方式治疗小儿难治性癫痫的临床疗效及其安全性。方法选取难治性癫痫患儿120例为研究对象,应用随机数字表法分为3组,对照组患儿给予抗癫痫药物治疗,生酮饮食组患儿给予生酮饮食治疗,神经生长因子组给予鼠神经生长因子联合抗癫痫药物治疗。对比3组患儿的临床疗效、治疗前后发作次数、生活质量的变化及不良反应发生情况。结果与对照组及生酮饮食组对比,神经生长因子组患儿的总有效率及生活质量显著升高,癫痫发作次数显著降低;生酮饮食组患儿的总有效率及生活质量显著高于对照组(P0.05);3组患儿的不良反应发生率差异无统计学意义(P0.05)。结论鼠神经生长因子联合抗癫痫药物治疗小儿难治性癫痫的临床疗效优于应用抗癫痫药物及生酮饮食治疗,且安全性高,值得临床推广应用。     

伴发癫痫的结节性硬化23例临床分析

目的:分析总结伴癫痫的结节性硬化患者的临床特点。方法:回顾性分析23例伴癫痫的结节性硬化患者的临床资料。结果:本组23例伴癫痫的结节性硬化患者中,<2岁有14例,为部分性发作。5例患者从部分发作转化为全身强直-阵挛发作,其中22例(95.7%)抗癫痫治疗有效,1例通过手术治疗控制癫痫病情。患儿最常见的皮肤表现是各种形态的色素减退斑和面部皮脂腺瘤。23例患儿中智力障碍者有8例(34.78%)。结论:伴癫痫的结节性硬化患者发病早,主要为部分性癫痫发作,发生形式多变,抗癫痫治疗有效。     

Sturge—Weber综合征

Sturge-Weber综合征（Sturge-Weber syndrome,SWS）又称脑面血管瘤病或脑三叉神经血管瘤病,最早见于1860年Schrimer报道的-36岁男性患者,双侧面部葡萄酒色血管痣和单侧眼积水。后Sturge又报道一个六岁半的女孩患右侧面部血管痣、右侧高眼压和癫痫发作口’;其他作者也有类似报道。     

脑面部血管瘤1例分析

对脑面部血管瘤1例分析如下. 1 病历摘要 男,19岁.因发作性左侧肢体抽搐伴无力17 a、再发3 d于2010-11-08入院.该患者于17 a前开始反复出现肢体抽搐,以左侧肢体明显,抽搐时意识不清,无大小便失禁,抽搐后出现肢体无力.严重时有全身强直痉挛发作,初未处理,去年开始在我院诊断为症状性癫痫,予抗癫痫药治疗后好转,但后自行停药.     

抗癫痫药物对癫痫患儿骨密度的影响及与骨代谢和体格发育的相关性

目的 探讨抗癫痫药物治疗对癫痫患儿骨密度的影响及与骨代谢和体格发育的相关性。方法 对150例癫痫患儿给予抗癫痫药物治疗(脱落6例),治疗1 a后检测骨密度,统计骨密度异常发生率,根据是否发生骨密度异常分为骨密度异常组58例和骨密度正常组86例,比较两组相关临床资料,分析抗癫痫药物治疗癫痫患儿发生骨密度异常的影响因素。治疗1 a后比较两组骨密度、血钙、血磷、血清骨碱性磷酸酶、血清骨钙素、身高、体质量,分析骨密度与骨代谢指标、体格发育指标的相关性。结果 癫痫患儿抗癫痫药物治疗1 a后骨密度异常发生率为40.3%。两组抗癫痫药物数量、抗癫痫药物种类、户外活动时间、定期补充维生素D情况比较差异有统计学意义(P<0.01)。多药治疗、采用传统抗癫痫药物治疗、户外活动时间少、未定期补充维生素D均为骨密度异常的危险因素。癫痫患儿抗癫痫药物治疗1 a后骨密度与血钙、血磷、血清骨钙素、身高、体质量呈显著正相关(P<0.05),与血清骨碱性磷酸酶呈显著负相关(P<0.05)。结论 癫痫患儿抗癫痫药物治疗期间骨密度异常发生率较高,多药治疗、传统抗癫痫药物治疗、户外活动时间少、未定期补充维生...     

癫痫患儿再次痫性发作的诱发因素

目的 探讨癫痫患儿再次痫性发作的诱发因素,减少或尽量消除诱发因素,减少癫痫再发,提高抗癫痫治疗的效果.方法 对2008年10月～2009年12月在苏州大学附属儿童医院神经内科已经确诊为癫痫、正规抗痫药（AED）治疗中、因痫性发作再次来院的70例患儿进行调查,分析其诱发因素.结果 〈6岁患儿中感染是最主要的诱发因素,≥6岁患儿中诱发因素依次为感染、学习压力大、睡眠不足、劳累、电视电脑等.结论 癫痫患儿再次痫性发作的诱发因素在不同年龄组儿童表现不同,诱发因素的存在直接影响癫痫的治疗效果,控制或减少诱发因素,可以减少癫痫发作,以进一步提高抗癫痫治疗效果.     

35例癫痫患儿用苯巴孚治疗前后血清T3,T4,TSH变化

目的：探讨抗癫痫药苯巴比妥对癫痫患儿甲状腺功能的影响。方法：对３５例癫痫患儿用苯巴妥治疗,治疗前后血清Ｔ３、Ｔ４、ＴＳＨ进行测定,并与正常对照组比较。结果：用药前Ｔ３、Ｔ４与正常对照组比较无明显变化;用药后Ｔ３、Ｔ４显著降低（Ｐ〉０．０５,Ｐ〈０．０１）;用药前后ＴＳＨ无明显改变。结论：癫痫患儿用苯巴比妥治疗后血清Ｔ３、Ｔ４的改变主要是抗癫痫药苯巴比妥引起的。     

144例癫痫患儿家长的健康教育

小儿癫痫是一种脑功能障碍性疾病,患儿家长常因得不到及时、有效的相关知识,心理紧张、焦虑、矛盾导致治疗信心不足,影响患儿预后.针对这一状况,我科1997年3月至1998年11月对144例癫痫患儿家长在入院时,治疗前,治疗中分别进行癫痫发作时注意事项、发病机理、用药指导及预后与治疗的关系的宣教,收到了较好效果,96%的患儿顺利完成了住院期间抗癫痫的规范治疗.此外,通过出院指导,家长较好的掌握患儿疾病与休息、学习、娱乐、睡眠的关系.使规范的抗癫痫治疗得以延续,为患儿获得良好预后提供保证.     

抗癫痫药和脑电图放电指数对癫痫患儿认知功能的影响

目的观察新老抗癫痫药和脑电图放电指数对癫痫患儿认知功能的影响。方法前瞻性纳入2015年8月至2018年1月沧州市人民医院门诊收治的50例未服用过抗癫痫药物的新诊断的继发性癫痫患儿。将入选癫痫组患儿随机分为丙戊酸钠(VPA)组和托吡酯(TPM)组,每组25例,两组分别给予口服VPA和TPM。VPA组和TPM组患儿用药前及用药后6月采用蒙特利尔认知量表(MoCA)对所有研究对象分别进行两次认知功能检测。观察TPM和VPA治疗6个月后癫痫患儿认知功能的变化,并探讨脑电图放电指数大于或等于0. 5和脑电图放电指数小于0. 5患儿认知功能的情况。结果两组患儿治疗前视空间功能、命名能力、计算力、语言能力、抽象能力、定向力及延迟回忆能力评分无显著差异(P 0. 05);治疗后,VPA组患儿的视空间功能、命名能力、计算力、语言能力、抽象能力、定向力及延迟回忆能力评分均无明显改变(P 0. 05),而TPM组患儿以上评分均显著降低(P 0. 05)。脑电图放电指数小于0. 5患儿的视空间功能、命名能力、计算力、语言能力、抽象能力、定向力及延迟回忆能力评分均高于脑放电指数大于或等于0. 5的患儿。结论丙戊酸治疗癫痫患儿对认知功能无明显不良反应,而托吡酯对患儿认知功能有一定的损害,丙戊酸的安全性更高。癫痫患儿的认知功能与脑电图放电指数密切相关,临床上治疗癫痫时需定期监测脑电图。     

先天代谢缺陷病合并癫痫临床特点分析

目的:分析先天代谢缺陷病(IEM)中继发性癫痫的临床特点,为临床诊治提供依据.方法:对2003年1月至2007年12月收治的先天代谢缺陷病患儿进行回顾性分析,包括一般情况、病史、临床症状、脑电图检测结果等.结果:共61例11种代谢病患儿合并癫痫.惊厥首发年龄<1岁34例(55.7%),占首位.甲基丙二酸尿症与线粒体病是合并癫痫的主要病种,这两种疾病惠儿分为癫痫组34例、无癫痫组31例,癫痫组18例(52.9%)患儿惊厥表现为多种形式发作;无癫痫组5例(16.1%)患儿脑电图异常,表现为慢波背景及节律失调.结论:IEM引起的继发性癫痫惊厥出现早、抽搐形式多样.治疗时要选用对代谢无影响的抗癫痫药.脑电图应作为确诊IEM惠儿的常规检查.     

Sturge-Weber综合征的影像诊断(附6例报告)

目的探讨Sturge-Weber综合征影像学表现及其诊断价值。方法回顾性分析6例Sturge-Weber综合征临床特征及其CT、MRI表现。结果6例均有单侧面部血管瘤及癫痫,1例血管瘤达头顶皮肤,3例CT可见患侧脑萎缩及钙化,2例同侧侧脑室扩大,1倒双侧侧脑室扩大,1例患侧颅骨增厚,1例无异常发现;MRI所见脑萎缩及侧脑室扩大同CT所见,3例增强扫描显示病变范围明显大于CT和MRI平扫所见。结论通过CT和MRI影像学检查,特别以增强MRI扫描,对Sturge-Weber综合征的诊断有确诊价值。     

Sturge-Weber综合征的CT与MRI比较影像学研究(附9例报告)

目的 探讨CT与MRI在诊断Sturge-Weber综合征中的价值。方法 收集Sturge-Weber综合征的病例共9例,所有患儿均行常规CT扫描和MRI检查,其中2例同时行MRI增强扫描。结果 （1）CT表现：6例一侧脑叶受累;2例双侧额顶叶均受累;1例CT未见异常。其中5例脑叶呈中重度萎缩。8例皮层内出现脑回状钙化;3例一侧受累脑叶灰白质分界不清;2例合并一侧脉络丛扩大;1例合并有深静脉血管团出现。（2）MRI表现：9例MRI中5例一侧脑叶受累;4例双侧额顶枕叶均受累,所有患儿均有不同程度的脑萎缩,其中轻度者4例,中度者3例,重度者2例,仅有2例在萎缩的皮层下出现钙化;8例受累脑叶灰白质分界不清;5例一侧脑室内脉络丛呈囊状扩大;3例一侧颅板板障增厚;4例于侧脑室旁、室管膜下区出现粗大的静脉团。2例增强扫描后,受累侧皮层出现脑回状强化。结果 MRI与CT的综合分析。对于本病的诊断和病情的判定有一定的价值。     

Sturge-Weber综合征CT诊断(附12例分析)

目的:探讨Sturge-Weber综合征的CT表现和诊断价值。方法:回顾性分析12例Sturge-Weber综合征的CT表现。结果:12例均有脑皮层小片状到“脑回状”钙化。其中右侧6例,左侧5例,1例为两侧额、顶、颞、枕叶广泛钙化;脑萎缩10例;颅骨增厚4例;1例增强扫描示钙化灶周围皮层表面“脑回状”强化。结论:Sturge-Weber 综合征颅内皮层钙化具有一定CT特征;典型的临床及CT表现可以确诊本病;有颅面部血管瘤而CT平扫阴性时应复查。     

Diagnosis and neurosurgical treatment of glossopharyngeal neuralgia: clinical findings and 3-D visualization of neurovascular compression in 19 consecutive patients

Glossopharyngeal neuralgia is a rare condition with neuralgic sharp pain in the pharyngeal and auricular region. Classical glossopharyngeal neuralgia is caused by neurovascular compression at the root entry zone of the nerve. Regarding the rare occurrence of glossopharyngeal neuralgia, we report clinical data and magnetic resonance imaging (MRI) findings in a case series of 19 patients, of whom 18 underwent surgery. Two patients additionally suffered from trigeminal neuralgia and three from additional symptomatic vagal nerve compression. In all patients, ipsilateral neurovascular compression syndrome of the IX cranial nerve could be shown by high-resolution MRI and image processing, which was confirmed intraoperatively. Additional neurovascular compression of the V cranial nerve was shown in patients suffering from trigeminal neuralgia. Vagal nerve neurovascular compression could be seen in all patients during surgery. Sixteen patients were completely pain free after surgery without need of anticonvulsant treatment. As a consequence of the operation, two patients suffered from transient cerebrospinal fluid hypersecretion as a reaction to Teflon implants. One patient suffered postoperatively from deep vein thrombosis and pulmonary embolism. Six patients showed transient cranial nerve dysfunctions (difficulties in swallowing, vocal cord paresis), but all recovered within 1 week. One patient complained of a gnawing and burning pain in the cervical area. Microvascular decompression is a second-line treatment after failure of standard medical treatment with high success in glossopharyngeal neuralgia. High-resolution MRI and 3D visualization of the brainstem and accompanying vessels as well as the cranial nerves is helpful in identifying neurovascular compression before microvascular decompression procedure.     

中枢神经系统受累的儿童噬血细胞性淋巴组织增生症临床分析

目的分析儿童噬血细胞性淋巴组织增生症(hemophagocytic lymphohistiocytosis, HLH)患儿中枢神经系统受累的临床特征和预后。方法回顾性分析13例出现中枢神经系统受累的HLH患儿的临床及影像学资料,并应用Kaplan-Meier法分析5年生存率。结果 13例HLH患儿中癫痫发作7例,意识障碍3例,面神经麻痹1例,烦躁1例,吞咽困难和构音障碍1例;1例有恶性血液病家族史;3例MUNC13-4基因突变,1例编码perforin-1基因突变;4例感染EB病毒;13例患儿均行脑CT和MRI检查,7例影像学表现正常,6例异常;7例癫痫发作患者中2例服用抗癫痫药物治疗,5例未服用抗癫痫药物情况下癫痫停止发作;13例患儿死亡4例,5年生存率为69%。结论中枢神经系统受累的HLH患者死亡风险较高,脑CT/MRI影像检查可表现异常,对提高HLH诊断有一定价值。     

Primary yawning headache

To describe three patients with recurrent severe paroxysmal headache precipitated by yawning. Pain elicited by yawning is a well-recognized clinical phenomenon in patients with cranial neuralgia, temporomandibular joint dysfunction syndrome and Eagle syndrome. Clinical history, neurological and oral examinations, brain magnetic resonance imaging (MRI), cranial nerve electrophysiological testing and skull X-rays are reported. In all the patients pain was induced by yawning; in the third patient pain was also triggered by eructation. None had history of migraine. Facial gestures and forceful opening of the mouth did not reproduce the pain. The first patient had retroauricular pain, simvastatin-induced myopathy and subclinical axonal peripheral neuropathy; the second patient had a post-viral benign sensory neuropathy; and the third had retroauricular and facial pain and no underlying neurological illness. Cranial nerve testing and MRI of the brain were normal except for a coincidentally found pituitary adenoma on the first patient. Headache or cranial pain with yawning may occur in patients with no apparent cause (primary yawning headache). It is a chronic, benign condition that requires no specific treatment but needs to be distinguished from secondary yawning headache, of greater clinical relevance.     

PHACES综合征颅内影像学表现

目的 分析PHACES综合征颅内影像学表现。方法 回顾性分析9例符合PHACES综合征诊断标准患儿的影像学资料，包括4例颅脑MRI、3例增强CT和2例增强CT及MRI，观察有无颅内大血管、脑结构异常及其他伴随异常。结果 6例存在颅内大血管异常，包括2例单侧颈内动脉发育不良，1例单侧颈内动脉发育不良伴同侧大脑中动脉狭窄，1例单侧颈内动脉缺如伴大脑后动脉起始部囊性动脉瘤，1例寰前节间动脉，1例原始眼动脉。8例存在颅后窝脑结构异常，其中6例单侧小脑发育不良，2例Dandy-Walker畸形；6例合并颅内血管瘤，位于桥小脑角区，与皮下血管瘤不相连。6例伴发眼眶内血管瘤，其中5例同侧存在颅内血管瘤，但不与之相连。3例存在幕上脑积水。结论 单侧小脑发育不良和颈内动脉异常是PHACES综合征最常见颅内表现；颅内血管瘤在PHACES综合征患儿中并不少见。     

Mastoid bone fracture presenting as unusual delayed onset of facial nerve palsy

Delayed-onset facial nerve paralysis is a rather uncommon complication of a mastoid bone fracture for children younger than 10 years. We routinely arrange a cranial computed tomography (CT) for patients encountering initial loss of consciousness, severe headache, intractable vomiting, and/or any neurologic deficit arising from trauma to the head. However, minor symptomatic cranial nerve damage may be missed and the presenting symptom diagnosed as being a peripheral nerve problem. Herein, we report a case of a young boy who presented at our emergency department (ED) 3 days subsequent to his accident, complaining of hearing loss in the right ear and paralysis of the ipsilateral face. Unpredictably, we observed his cranial CT scan revealing a linear fracture of the skull over the right temporal bone involving the right mastoid air cells. The patient was treated conservatively and recovered well without any adverse neurologic consequences. We emphasize that ED physicians should arrange a cranial CT scan for a head-injured child with symptomatic facial nerve palsy, even if there are no symptoms such as severe headache, vomiting, Battle sign, and/or initial loss of consciousness.     

Traumatic intracranial hemorrhages in facial fracture patients: review of 2,195 patients

OBJECTIVE: Patients sustaining facial fractures are at risk for accompanying traumatic intracranial hematomas, which are a major cause of morbidity and mortality. Prompt recognition is crucial in improving patient survival and recovery. This study examined which simple clinical signs identify facial fracture patients at risk for intracranial hemorrhage before the performance of computed tomography. DESIGN AND METHODS: Retrospective study of 2,195 patients with facial fractures during a period of 7 years. By means of univariate and multivariate analysis clinical features potentially predictive for (a) intracranial hemorrhage and (b) surgery for intracranial hemorrhage were identified. SETTING: Critical care units of anesthesiology and neurology, general traumatology, and oral and maxillofacial surgery in a level I trauma university hospital. RESULTS: Seizures (OR 22.1) and vomiting/nausea (OR 20.2) were the strongest independent predictors of intracranial bleeding in facial fracture patients. For intracranial hemorrhages requiring surgical intervention closed head injuries (OR 9.75) and cranial vault fractures (OR 5.0) were the most significant risk factors. However, among those patients without vomiting/nausea and without seizures and without closed head injury ( n=1,628), 20 patients (1.2%) suffered intracranial hemorrhage, and six (0.37%) of them required surgical intervention. CONCLUSIONS: Simple clinical symptoms, such as seizures, vomiting/nausea, history of a closed head injury or cranial vault fractures are strong predictors for intracranial hemorrhage in facial fracture patients. The early consideration of such important indicators allows us to detect patients at elevated risk of an intracranial hematoma requiring surgical intervention.     

Headache in Sturge-Weber Syndrome

SYNOPSIS
Based on reports of headache in arteriovenous malformations and epilepsy, one would predict that the prevalence of migraine headache in the Sturge-Weber syndrome would be higher than in the general population.
A questionnaire designed to determine the prevalence and type of headache by IHS criteria was sent to the 500 members of the Sturge-Weber Foundation.
The average age of the patients in this sample (71 patients) was 15 years. The sex ratio was approximately one to one. Headache meeting IHS criteria for migraine occurred in 28% (equally among males and females) compared to 17% in females and 5% in males in the general population, Neurologic deficits occurred in 58% during migraine, The prevalence of migraine in children under the age of 10 (31%) was higher than that reported in the general population (5%) and the age of onset was younger. The mechanism of migraine headache in Sturge-Weber syndrome may be similar to that found in epilepsy and vascular malformations.