轻度胃肠炎伴良性婴幼儿惊厥24例临床分析

目的 分析轻度胃肠炎伴良性婴幼儿惊厥(benign infantile convulsion with mild gastroenteritis,BICG)的临床特点.方法 对24例BICG住院患儿进行临床观察和出院后随访.结果 24例患儿中,男8例,女16例.平均年龄16.8个月,发病年龄8～22个月.冬季多见.患儿既往精神运动发育正常,无癫(癎)家族史.临床腹泻症状不严重,惊厥表现形式均为全身强直-阵挛发作,多发生在病程的3d内.10例(41.7％)患儿有2次或多次惊厥发作,平均1.7次.实验室检查:血生化检查均正常,16例行脑脊液检查均正常.发作间期EEG均正常.20例接受头颅CT或MRI检查,除1例有透明隔囊肿,其余均无异常.急性期后均未给予长期抗癫(癎)治疗,随访8～46个月,只有1例患儿(4.2％)再患轻度胃肠炎时再发惊厥.所有患儿精神运动发育正常.结论 BICG预后良好,不需要长期抗癫(癎)药物治疗.     

轻度胃肠炎伴婴幼儿良性惊厥248例临床及预后分析

目的：探讨轻度胃肠炎伴婴幼儿良性惊厥的临床特点及预后。方法对248例符合轻度胃肠炎伴婴幼儿良性惊厥诊断的患儿进行临床分析,并随访12个月以上。结果发病年龄3月龄~3岁;1次病程中惊厥发作2次以上者124例,惊厥发作5 min以上者64例;头颅CT/MRI检查、脑电图检查,均未见异常;血糖、血电解质均正常,脑脊液常规、生物化学检查正常,脑脊液细菌涂片和培养均为阴性;随访中有14例(5．6%)出现复发,12例(4．8%)出现热性惊厥,8例(3．2%)转化为癫疒间。结论轻度胃肠炎伴婴幼儿良性惊厥多数病例预后良好,少数病例有向热性惊厥及癫疒间转化的可能,在轻度胃肠炎伴婴幼儿良性惊厥后出现热性惊厥的患儿转化为癫疒间的可能性较大,且轻度胃肠炎伴婴幼儿良性惊厥在惊厥频繁发作时对中枢神经元会造成损伤,若再次有惊厥发作应及时就诊,以免延误诊治。     

轻度胃肠炎伴婴幼儿良性惊厥26例

目的探讨轻度胃肠炎伴婴幼儿良性惊厥(BICE)的临床特征。方法回顾性分析26例BICE临床资料,观察病情,行血液常规、生化、大便常规及脑影像学(CT/MRI)和视频脑电图等相关检查,按照BICE的诊断标准,除外热性惊厥及癫。予抗惊厥药物对症治疗,随访2 a。结果 26例均有BICE典型的临床特征,高发时间为当年11月份-次年3月份。胃肠炎以轮状病毒多见,主要症状为轻度腹泻或(和)呕吐伴无热惊厥。发作形式为全身强直-阵挛发作,或局限发作泛化为全面性发作。脑CT/MRI检查正常或轻度异常,随访0.5 a有3例复发,复发次数≤3次,经治疗惊厥症状全部消失。23例2 a内无复发,发作前后精神运动发育正常。结论对于年龄6个月～3岁患儿,有轻度腹泻、呕吐伴无热惊厥,无酸碱失衡、电解质紊乱及血糖异常,无脑电图异常及脑脊液改变者可考虑BICE,尽早确诊,不必要长期复杂的检查及抗癫治疗。     

轻度胃肠炎并婴幼儿良性惊厥40例临床分析

目的：研究轻度胃肠炎并婴幼儿良性惊厥的临床特征、病因及治疗措施。方法：收集本院住院的轻度胃肠炎并婴幼儿良性惊厥的患儿40例。观察记录患儿住院期间的胃肠炎表现及惊厥特点,并进行总结。用金标法及ELISA对患儿粪便及脑脊液进行轮状病毒及杯状病毒进行检测。随访半年以上。结果：患儿胃肠症状多较轻微,无或轻度脱水;惊厥多为成簇发作;血清电解质及血糖正常或基本正常;脑脊液及头颅影像学检查正常;发作间期脑电图：8例于中央中线或中央区或额区少量癫癎样放电, 1例中央中线及顶区癫癎样波发放明显,其他31例未见异常或睡眠背景欠佳;粪便轮状病毒抗原检测阳性11例,杯状病毒抗原阳性4例;脑脊液轮状病毒及杯状病毒抗体均为阴性;28例苯巴比妥钠预防性肌注（每次5～10 mg/kg）后惊厥再发22例;随访半年以上,1例进展为癫癎,39例无惊厥发作及其他神经系统后遗症。结论：轻度胃肠炎并婴幼儿良性惊厥消化系统表现轻,惊厥往往成簇发作;致惊机制尚不清楚,病毒引起脑炎的可能性不大;常规剂量的苯巴比妥钠肌注不能预防惊厥的反复发作;绝大多数患儿预后良好。［中国当代儿科杂志,2010,12（7）：533-535］     

轻度胃肠炎伴婴幼儿良性惊厥24例

目的：了解伴轻度胃肠炎婴幼儿良性惊厥（BICE）患儿临床经过。方法：对本院2002年12月-2004年2月收治的BICE患儿24例进行2年～3年4个月随访，临床发作有成簇惊厥发作倾向，排除电解质异常、热性惊厥、癫痫及癫痫综合征、症状性癫痫及神经运动发育异常，观察患儿惊厥的复发率及预后。结果：脑电图中央区小尖波4例；CT示脑外部积水1例；经2年随访观察，因胃肠道症状再发惊厥2例，因高热再发惊厥2次1例；随访患儿均未应用抗癫痫药，智能发育正常。结论：BICE患儿惊厥发作次数较少。部分患儿惊厥呈成簇发作，患儿临床预后均良好。     

轻度胃肠炎伴发良性婴幼儿惊厥20例临床分析

目的 分析轻度胃肠炎伴发良性婴幼儿惊厥的特点.方法 2003年7月～2006年5月对我院儿科20例4～30个月的轻度胃肠炎伴发良性婴幼儿惊厥患儿进行临床观察和出院后12个月以上的随访.结果 20例发病年龄最小4个月,最大30个月,1～2岁共14例.冬季发病10例,秋季发病8例.3 d内发生惊厥17例,均为全身发作,持续时间短,不超过5 min,一次病程中惊厥平均发作1.8次.临床急性胃肠炎症状不严重,伴无热惊厥呈全身强直阵挛发作,间期EEG正常,血清电解质、血糖、肝功能检查均正常,10例做脑脊液检查正常,12例做头颅CT检查结果均正常.全部病例无癫(癎)和热性惊厥家族史,出院随访17例有2例惊厥复发,17例患儿生长发育如正常儿.结论 轻度胃肠炎伴发良性婴幼儿惊厥有以下特点:(1)1～2岁高发,冬秋季多发,无家族史;(2)无热惊厥于病程的1～5 d发生,全身性发作为主,无脱水酸中毒;(3)血清电解质、血糖、肝功能、脑脊液检查、发作间期EEG均正常;(4)部分病例可复发,次数≤2次,病程少于1年,预后好.     

幼儿间断抽搐1年半伴发育落后

该文报道1例吡哆醇依赖性癫癎患儿。患儿女,2岁时因发育落后,间断抽搐1年半就诊。患儿新生儿期曾有“缺氧”病史,婴儿期早期即出现难以控制的惊厥发作,部分性发作为主,多次癫癎持续状态,多种抗癫癎药物均不能控制发作。发热感冒时抽搐发作频繁。治疗前后多次视频脑电图及头颅MRI检查均正常。曾考虑诊断为Dravet综合征,后通过ALDH7A1基因检测确诊为吡哆醇依赖性癫癎。确诊后逐渐减停抗癫癎药物,仅单纯口服吡哆醇,发作基本控制。     

口服抗癫癎药物治疗儿童热性惊厥的临床与脑电图分析

目的：了解有癫癎高危因素的热性惊厥患儿应用丙戊酸钠与托吡酯口服治疗4年的临床与脑电图改变情况。方法：对2004~2005年的132例有应用抗癫癎药物指征的热性惊厥患儿给予丙戊酸钠与托吡酯口服治疗,每半年随访一次病情变化、血常规和肝肾功能;每一年随访一次睡眠脑电图,共随访4年。结果：随访时间1~10年,丙戊酸钠口服液单药治疗110例,长期控制率为98.2%,95例已停药,10例正处在减药过程中。托吡酯单药治疗13例,均发作控制,顺利停药。无一例出现血常规和肝肾功能异常表现。治疗后睡眠脑电图恢复正常者102例,出现局灶性改变者8 例,两侧同步性棘慢波4例,3 Hz棘慢波及多棘慢波2例。结论：对于癫癎高危因素的热性惊厥患儿,早期使用抗癫癎药物丙戊酸钠或托吡酯,疗效确切,无明显不良反应。抗癫癎药物治疗后大部分患儿睡眠脑电图恢复正常。     

轻度胃肠炎伴良性婴幼儿惊厥8例临床分析

目的　分析轻度胃肠炎伴良性婴幼儿惊厥 (BICE)的临床特点。方法　1999年 7月至 2003年 3月,对北京中日友好医院儿科 8例年龄 3~36个月的轻度胃肠炎伴惊厥患儿进行临床观察和出院后 18个月以上的随访。结果　8例发病年龄(14 0±5 9)个月,冬季发病 4例, 3d内发生惊厥 6例,全身性发作 7例,有早期频繁发作 5例,一次病程中惊厥平均发作 2 4次。发作间期EEG背景正常,脑影像学、血生化及脑脊液检查正常。惊厥停止后未行抗疒间治疗, 3例复发,复发≤2次,病程少于 1年。全部病例无癫疒间和热性惊厥家族史,精神运动发育正常。结论　本组BICE具有以下特点: (1) 1 ~2岁高发,冬季多发,无家族史; (2)无热惊厥于病程的 1 ~5d发生,全身性发作为主,早期频繁发作多见,无脱水及酸中毒; 930血电解质、脑脊液、脑影像学和发作间期脑电图正常; (4)部分病例可复发,次数≤2次,病程少于 1年,预后好。     

特发性儿童枕叶癫癎21例临床分析

目的总结特发性儿童枕叶癫癎的临床表现及脑电图特点,以提高临床诊断水平。方法回顾性分析2000—2007年广西医科大学第一附属医院收治的21例确诊为特发性儿童枕叶癫癎患儿的临床表现及脑电图特点。结果起病年龄5个月至14岁;发作性呕吐11例,眼球偏转11例,头偏转8例,发作后头痛8例,口咽部自动症5例,眼睑扑动6例,黑蒙5例,视物模糊3例,视幻觉2例;继发半侧肢体抽搐5例,继发全身性发作7例,21例头颅影像学检查均正常。所有病例发作间期脑电图均有癎样放电,闭眼出现,睁眼消失。随访1～5年,其中早发型儿童良性枕叶癫癎(EBOS)12例,晚发型儿童良性枕叶癫癎(LOS)9例,EBOS对多种抗癫癎药物(AEDS)反应较好,12例EBOS患儿中11例单药治疗完全控制发作,1例患儿两种AEDS联合应用可以完全控制发作,IOS对AEDS治疗反应稍差,9例LOS患儿中有2例未完全控制。结论特发性儿童枕叶癫癎是一组较具特征表现的癫癎综合征,掌握临床表现及脑电图特点,常可做出正确诊断。     

Comparison of patients with Kawasaki disease with retropharyngeal edema and patients with retropharyngeal abscess

Kawasaki disease with retropharyngeal edema (KD with RPE) is a rare complication, and it is diagnosed by neck CT. Most reported cases had a delayed diagnosis because those patients' conditions were misdiagnosed as retropharyngeal abscess (RPA). The purpose of this study was to differentiate KD with RPE from RPA. We performed a retrospective case–control study comparing children with KD with RPE to those with RPA hospitalized at the tertiary pediatric hospital in Tokyo between 2005 and 2011. The 39 patients revealing RPE on neck CT were divided into two groups: group A was classified as KD (n?=?21) and group B was classified as non-KD (n?=?18). Patients in group B were finally evaluated as having RPA clinically and were treated with antibiotic therapy. A significantly higher proportion of patients in group B complained of dysphagia (11 patients vs. 5 patients; p?=?0.0170) and neck pain (17 patients vs. 12 patients; p?=?0.0106). Neck CT revealed a ring enhancement (16 patients vs. no patients; p?<?0.0001) and mass effect in a greater proportion of patients in group B (11 patients vs. 1 patient; p?<?0.0003). Conclusion: Careful attention to manifestations and close analyses of CT imaging may allow clinicians to differentiate KD with RPE from RPA.     

Patients with moyamoya disease presenting with movement disorder

Moyamoya disease is a rare cerebrovascular disease characterized by idiopathic bilateral stenosis or occlusion of bilateral internal carotid arteries and the development of characteristic leptomeningeal collateral vessels at the base of the brain. Typical presentations include transient ischemic attacks or stroke, and hemorrhage. Presentation with movement disorders is extremely rare, especially in the pediatric population. The authors describe the cases of 4 children with moyamoya disease who presented with movement disorders. Among 446 patients (118 pediatric) with moyamoya disease surgically treated by the senior author, 4 pediatric patients had presented with movement disorders. The clinical records, imaging studies, surgical details, and postoperative clinical and imaging data were retrospectively reviewed. The initial presenting symptom was movement disorder in all 4 patients: chorea in 2, hemiballismus in 1, and involuntary limb shaking in 1. All the patients had watershed infarcts involving the frontal subcortical region on MR imaging. Additionally, 1 patient had a ganglionic infarct. Single-photon emission computed tomography studies showed frontoparietal cortical and subcortical hypoperfusion in all patients. Three patients had bilateral disease, whereas 1 had unilateral disease. All the patients underwent superficial temporal artery-middle cerebral artery bypass. Postoperatively, all 4 patients had complete improvement in their symptoms. The SPECT scans revealed normal perfusion in 3 patients and a small residual perfusion deficit in 1. Movement disorders are a rare presenting feature of moyamoya disease. Hypoperfusion of the frontal cortical and subcortical region was seen in all patients, and the symptomatology was attributed to ischemic dysfunction and imbalance in the cortical-subcortical-ganglionic-thalamic-cortical circuitry. Combined revascularization with superficial temporal artery-middle cerebral artery bypass and encephaloduroarteriosynangiosis leads to excellent results.     

Treatment with probucol of children with familial hypercholesterolaemia

Nine children with familial hypercholesterolaemia, age range 2 to 12 years, were treated with a low cholesterol diet and probucol (10 mg/kg/day). The year before, the children received, as only treatment, a low fat-cholesterol diet. During this period their mean plasma total cholesterol level fell from 8.2 +/- 1.45 mmol/l to 7.17 +/- 0.84 mmol/l (12.6%). This level was further reduced to 5.92 +/- 0.63 mmol/l (17.1%) after the addition of probucol. Plasma high density lipoprotein cholesterol levels were lowered in absolute terms but not in relation to total cholesterol. No apparent side effects were observed. However, the use of probucol should be restricted for the moment to severe cases of hypercholesterolaemia as the long-term excretion of the drug in children is not yet known.