晚发型癫痫性痉挛临床与脑电图特征分析

探讨晚发型癫痫性痉挛患儿的临床和脑电图（EEG）特征、治疗反应及预后。方法　对北京大学第一医院儿科2000年6月至2007年8月59例晚发型癫痫性痉挛患儿的临床和EEG资料进行回顾性研究,其中37例有随访,随访时间13～90个月,末次随访年龄为2岁6个月至12岁2个月（中位数60个月）。结果　59例患儿癫痫起病年龄为12～98个月,中位年龄22个月。以痉挛发作起病36例（61.0%）,以其他发作类型起病23例（39.0%）。17例（28.8%）在病程中有部分性发作,22例（37.3%）合并其他全面性发作形式,20例（33.9%）病程中仅有痉挛发作。EEG 14例（23.7%）为典型高度失律;2例（3.4%）为一侧性高度失律;43例（72.9%）无典型的高度失律。总计22例（37.3％）表现为局灶性或一侧性临床、影像学和（或）EEG异常。44例（74.6%）为症状性癫痫,围生期脑损伤、中枢神经系统感染、脑结构异常为主要病因。14例（23.7%）符合晚发型West综合征,7例（11.9%）符合Lennox-Gastaut综合征。促肾上腺皮质激素（ACTH）治疗后近期无发作率32.0%,有效率58.0%,复发率为61.5%。随访的37例患儿中,在抗癫痫药物治疗下至末次随访时46%维持临床无发作。80%以上患儿在治疗随访过程中精神运动发育落后或倒退,16%为重度落后。统计学分析显示,癫痫性痉挛病因、癫痫发作类型、痉挛发作形式、ACTH疗效与长期发作控制率及精神运动发育转归均无显著相关性。结论　晚发型癫痫性痉挛并非仅见于West综合征,可由诸多严重的脑部疾病引起,常合并其他部分性或全面性发作,EEG多数无典型高度失律,治疗困难,预后不良,ACTH仍为本病首选治疗方法。     

婴儿痉挛临床诊治与预后127例分析

目的通过对127例婴儿痉挛患儿的临床诊治和随访结果资料分析，了解婴儿痉挛的诊断治疗与预后之间的关系，为婴儿痉挛的临床诊治和预后判断提供依据。 方法采用回顾性资料分析和前瞻性队列研究，对1998年1月至2002年12月在复旦大学儿科医院收治的婴儿痉挛患儿进行分析和随访，对影响其预后的相关因素进行分析。 结果共有127例婴儿痉挛患儿进入研究。起病年龄为(6.1±3.1)个月,患儿随访时间(38±8)个月；72.4%为症状性,宫内发育迟缓、先天脑发育畸形、出生时窒息为主要病因,脑发育不全、智能发育迟缓多见；27.6%为隐原性或原发性。脑电图表现为高峰节律紊乱、弥漫或局灶性棘慢波发放。促肾上腺皮质激素(ACTH)、丙戊酸钠、氯硝基安定、硝基安定、苯巴比妥、托吡酯、拉莫三嗪为常用的治疗药物。在症状性婴儿痉挛中，76.1%预后不良,原发性或隐原性婴儿痉挛中，有60.8%的病例发作得到控制；有明确病因、起病年龄<3个月、起病前发育落后、EEG背景活动变慢或有局灶性棘慢波发放，可能提示婴儿痉挛预后不良。 结论婴儿痉挛多为症状性，有明确病因、起病年龄<3个月、起病前发育异常、EEG背景活动变慢或有局灶性棘慢波发放，与婴儿痉挛预后不良有关。     

肌阵挛失张力癫痫48例治疗及预后随访

目的　总结肌阵挛失张力癫痫（MAE）的治疗、脑电图（EEG）演变及预后,为选择治疗方案及预后评估提供参考。方法　对2005年11月至2010年12月北京大学第一医院儿科收集的MAE患儿48例,随访其治疗用药、疗效、EEG演变及预后。结果　48例中,男41例,女7例。随访时间8个月至5年5个月。应用抗癫痫药物（AEDs）发作控制42例（87.5%）,其中单用或联合应用丙戊酸38例,联合应用拉莫三嗪25例、左乙拉西坦10例、氯硝西泮9例、托吡酯8例。应用促肾上腺皮质激素（ACTH）发作控制2例。在应用AEDs和ACTH发作控制的44例中,36例EEG全导棘慢波、多棘慢波于病程7～49个月时消失,30例EEG于病程7～44个月时恢复正常,6例遗留背景θ节律。随访发现,有认知损伤11例（22.9%）。病程中出现癫痫持续状态、强直发作、EEG持续痫样放电及发作未控制与出现认知损伤相关（P < 0.05或0.01）。结论　丙戊酸和拉莫三嗪对MAE疗效好,ACTH对少数难治性MAE有效;MAE发作控制后,EEG全导棘慢波、多棘慢波发放首先消失,背景θ节律恢复较晚;合理选择AEDs治疗,多数MAE患儿预后良好,仅少数出现认知损伤。     

生酮饮食对不同病因及发作形式儿童难治性癫痫疗效差异的临床观察

目的分析生酮饮食对不同病因及发作形式儿童难治性癫痫疗效差异。方法选择2017年11月至2018年11月在本院接受生酮饮食治疗的难治性癫痫患儿50例作为观察对象,依据不同病因及发作形式进行分组。依据病因分为不明原因癫痫组和症状性癫痫组各25例。依据发作形式分为混合发作癫痫组20例和单一发作癫痫组30例;依据不同发作类型分为癫痫性痉挛发作组10例和非癫痫性痉挛发作组40例。患儿血酮2 mmol/L时开始生酮饮食。同时给予枸缘酸钾药物,每日0.5 g,复合维生素矿物质制剂每日6 g。生酮饮食脂肪与蛋白以及碳水化合物比例为4:1或3:1。治疗时间超过3个月,观察治疗效果。结果症状性癫痫组患儿治疗总有效率为80.0%(20/25),显著高于不明原因癫痫组48.0%(12/25),差异有统计学意义(P0.05)。单一发作癫痫组和混合发作癫痫组总有效率比较差异无统计学意义(P0.05)。癫痫性痉挛发作组总有效率为80.0%(8/10),显著高于非癫痫性痉挛发作组45.0%(18/40),差异有统计学意义(P0.05)。结论生酮饮食在难治性癫痫患儿治疗中具有较高应用价值,而本次研究进一步证实了,生酮饮食在不同发作形式癫痫患儿中并不存在明显差异,在原因上,症状性癫痫患儿的治疗效果更为理想,在发作类型上,癫痫性痉挛发作患儿的治疗效果更理想。     

从胎儿期延续至新生儿期的心房扑动三例报告(附文献复习)

目的 探讨新生儿心房扑动(简称房扑)的起病、病因、治疗及预后. 方法回顾分析2005年至2007年我院新生儿病房收治的3例房扑患儿的起病、治疗和预后,并对相关文献进行复习和总结. 结果 3例患儿药物治疗有效,随访结果预后较好,分别随访至8个月和2岁,房扑未复发. 结论宫内胎儿房扑应积极给孕母治疗,新生儿房扑可选择药物治疗、食道心房超速起搏及直流电复律等,预后较好.     

轻度胃肠炎伴良性婴幼儿惊厥35例临床特点分析

目的 总结轻度胃肠炎伴良性婴幼儿惊厥（BICE）的临床特点、脑电图及预后.方法 分析35例轻度胃肠炎伴良性婴幼儿惊厥的临床症状、实验室检查、脑电图及随访情况.结果 35例患儿主要表现为轻度腹泻,阵发性无热惊厥,发作形式为全身强直阵挛发作,发作时间多在病程2~5 d,头颅CT/MRI、脑电图、电解质、血糖、脑脊液正常,惊厥发作时给予临时止惊、补液等对症处理,未用抗癫痫药治疗.随访2年,3例患胃肠炎后出现惊厥,随访后再无惊厥发作,余患儿无复发,发病前后精神运动发育正常.结论 轻度胃肠炎伴婴幼儿良性惊厥是一种与胃肠炎有关的无热性惊厥,预后良好,不需要长期抗惊厥治疗.     

丙戊酸钠治疗儿童失神癫疒间的疗效

目的观察丙戊酸钠单剂治疗儿童失神癫疒间的疗效。 方法选择1990-04—2004-05在首都儿科研究所儿童医院诊断失神癫疒间并首选口服丙戊酸患儿123例，发作未能完全控制者加用氯硝西泮，观察其疗效并进行追踪。 结果123例患儿中，丙戊酸单药治疗后发作完全控制者105例，发作完全控制率844%，其余18例单用丙戊酸发作未能完全控制者，加用氯硝西泮后，发作均得到完全控制，全部患儿发作完全控制率为100%。追踪34例停药后的患儿9个月至10年，其中2例复发，占59%。 结论丙戊酸单药治疗失神癫疒间能使844%的患儿发作完全控制，发作不能完全控制者应加用氯硝西泮,二者联合用药可使患儿的临床发作得到完全控制,且起效快。     

电化学疗法治疗婴幼儿体表血管瘤464例

目的:探讨电化学疗法治疗婴幼儿体表血管瘤的疗效。方法:采用ZAY-6B及BK-2000型电化学治疗仪对464例患儿实施电化学治疗。结果:446例随访6个月—10年,共11例复发,有效率100%,首次治疗Ⅰ级(完全缓解率)99.1%(442/446)。结论:利用电化学疗法治疗婴幼儿表血管瘤具有创伤小、出血少、操作简便、安全可靠、对全身干扰小、治疗较彻底等优点。特别适用于面积较大,血供丰富,手术无法切除者,或特殊部位及经其他治疗后复发者,是一种有效的治疗手段,而且便于推广应用。     

急性播散性脑脊髓炎68例临床特征分析及预后研究

目的　探讨儿童急性播散性脑脊髓炎（ADEM）的临床特征与预后。方法　分析2001年2月至2007年3月68例复旦大学附属儿科医院ADEM患儿住院病史资料及 近远期疗效随访。结果　发病年龄1岁至15岁4个月,中位数6.5岁;住院患儿逐年增加。有前驱感染史者占57.4%;预防接种史者占4.4%;38.2%无明确病因。临床 分型：脑型42例（61.8%）,脑脊髓型18例（26.4%）,脊髓型8例（11.8%）;主要症状表现发热、瘫痪、惊厥、精神异常、锥体外系症状、意识障碍、认知障碍 、括约肌障碍、共济失调等,部分患儿出现语言功能障碍、颅神经麻痹。脑脊液免疫指标检查57例,44例（77.2%）阳性[IgG指数增加和（或）寡克隆带阳性]。 皮质激素近期疗效：起效时间55.9%≤3 d、26.5%≤7 d、17.6%＞1周;好转时间48.5%≤1周;8例自然缓解,死亡1例;远期疗效：病程≥1 48例;病程 < 1年 20 例。随访结果：痊愈38例;后遗症9例（病程1年以上尚未恢复者）;7例复发;随访中8例;失访5例。结论　儿童ADEM多发生于3～12岁,临床特征复杂多样,病 情轻重和病程长短不一;临床有多灶脑病的表现时应重视颅脑MRI检查以早期发现本病;多数远期预后良好,部分严重者完全恢复时间超过6个月,少数患儿留有 神经系统后遗症。     

宫腔镜联合孕激素治疗年轻患者早期子宫内膜癌的疗效及预后分析

目的:探讨宫腔镜联合孕激素治疗年轻早期子宫内膜癌患者的疗效及预后。方法:回顾性分析四川大学华西第二医院2008年1月至2014年12月自愿接受宫腔镜联合孕激素治疗的年轻早期子宫内膜癌16例患者的临床资料,且随访资料完整。所有患者行宫腔镜下刮宫或宫腔镜下病灶切除+刮宫,其中7例联合口服醋酸甲羟孕酮(250 mg/d,MPA组),9例联合醋酸甲地孕酮(160 mg/d,MA组)治疗。比较两组患者缓解、复发及生育等情况。结果:随访中位时间为54个月(17~90个月),其中12例(75.0%)完全缓解,3例(18.8%)部分缓解,1例(6.2%)无反应,MPA组和MA组的完全缓解率分别为71.4%(5/7)和77.8%(7/9),差异无统计学意义(P0.05)。16例患者中有3例在完全缓解后复发(MPA组1例,MA组2例),复发平均时间为42个月。16例患者中有8例(50.0%)完成了妊娠(其中1例2次妊娠),有6次(66.6%)妊娠顺利生产,3次(33.3%)流产。结论:宫腔镜联合孕激素治疗对于年轻早期子宫内膜癌患者保守治疗是有效的,其中MPA和MA的治疗效果无明显差异,但均存在着一定的复发风险,所以应对其进行严格的筛选和严密的随访。     

复发子宫内膜异位症二次手术的临床特点

目的:探讨子宫内膜异位症(EMs)非根治术后复发并行二次手术患者的临床特点。方法:回顾分析2010年1月至2015年8月于浙江大学医学院附属妇产科医院行二次手术治疗的290例EMs复发患者的临床资料。结果:290例患者二次手术平均间隔为(5.96±3.38)年,初次手术年龄大、初次手术r-AFS分期越高的患者的两次手术间隔时间短(P0.05),初次手术为半根治手术或单侧卵巢切除、术后有生育史的患者的两次手术间隔时间长(P0.05)。有腺肌病史、初次手术r-AFS分期高的患者的复发分期明显高于无腺肌病史、初次手术r-AFS分期低者(P0.05)。4.0%接受术后短期辅助药物治疗(≤6月)的患者1年内接受二次手术,12.2%未接受药物治疗的患者在1年内接受二次手术。结论:EMs初次手术分期高、有腺肌病史是复发的危险因素,初次保守手术单侧卵巢切除或者子宫切除及术后生育是延长二次手术间隔的保护因素。术后短期辅助药物治疗(≤6月)对降低术后1年内二次手术率有意义,但对延长远期二次手术间隔无明显意义。     

甲基丙二酸血症合并癫痫27例临床特点及预后分析

总结甲基丙二酸血症（MMA）合并癫痫的临床特点及预后。方法　对1997年1月至2009年12月北京大学第一医院儿科住院确诊的27例MMA合并癫痫发作患儿的临床资料、辅助检查结果、随访资料进行分析。结果　27例患儿除癫痫发作外,合并其他神经系统受损表现包括：智力运动发育落后或倒退22例、发作性嗜睡10例、肌张力增高8例、肌张力减低8例、反复呕吐4例、震颤和共济失调各2例、姿势异常1例。癫痫首次发作年龄为生后8d至11岁,发作类型包括部分性发作21例、全面强直阵挛发作5例、强直发作3例、肌阵挛发作3例,痉挛发作2例;5例患儿同时有2～3种发作类型。脑电图（EEG）检查有22例异常,包括：背景节律慢17例,局灶或多灶性痫样放电16例、广泛性痫样放电4例、高度失律2例和爆发抑制1例。头颅MRI检查24例显示脑萎缩14例,脑白质异常信号12例,胼胝体发育不良2例,双侧基底节病变2例和小脑萎缩1例。维生素B12有效型较维生素B12无效型患儿预后好。结论　癫痫是MMA的常见症状,部分性发作较其他发作类型更常见。对不明原因的癫痫患儿若伴其他神经系统症状,应进行尿有机酸分析,早期明确病因诊断,改善预后。     

The correlation of seizures in newborn infants with significant acidosis at birth with umbilical artery cord gas values

OBJECTIVE: To correlate umbilical blood gas variables with neonatal seizures in neonates with significant acidosis at birth (pH < or = 7.1). METHODS: We reviewed the maternal and neonatal charts of 238 patients at a gestational age of 32 weeks or more with cord gases done at delivery and an umbilical artery pH of 7.1 or less. All infants transferred to the neonatal intensive care unit were studied, and those with neonatal seizures secondary to hypoxic ischemic encephalopathy were identified. We used the perinatal outcome of early neonatal seizures secondary to hypoxic ischemic encephalopathy to divide the patients into two groups. The relationship between the umbilical artery parameters of pH, base deficit, partial oxygen pressure (pO(2)), partial carbon dioxide pressure (pCO(2)), and the neonatal outcome of seizures were determined with Student t tests and multiple logistic regression analysis. RESULTS: Umbilical artery base deficit, pO(2), and pCO(2) were significantly elevated in newborns who had seizures, whereas cord pH was decreased. Using multiple regression analysis the variable neonatal seizure was predicted only by low umbilical artery pH. A pH of less than 7 was more sensitive (73.8%) than a base excess of -16 (52.5%) in predicting the development of neonatal seizures. CONCLUSION: Our data suggest that severe fetal acidemia identified by a pH less than 7.0 was the most important umbilical blood gas variable for predicting early onset of neonatal seizures.     

Intellectual outcomes of patients with congenital hypothyroidism not detected by neonatal screening.

Mental retardation is a major sequela of delayed treatment of congenital hypothyroidism. In this study, we investigated the intellectual outcomes of patients with congenital hypothyroidism diagnosed before a nationwide screening project started. Eighty-two patients had intelligence tests done while in the euthyroid state. Their mean intelligence quotient (IQ) was 78 +/- 21 (+/- SD) (n = 79). The mean verbal IQ was significantly higher than the mean full-scale IQ. The mean IQs of both ectopic thyroid (84 +/- 21, n = 28) and dyshormonogenic patients (85 +/- 14, n = 18) were significantly higher than that of patients with athyrosis (64 +/- 18, n = 19) (p < 0.05). Patients who were treated before 3 months of age had significantly higher mean IQs (90 +/- 16, n = 16) than those treated after 3 months (75 +/- 21, n = 63) (p < 0.01). Our results confirm that both patients with ectopic thyroids and those with dyshormonogenesis have better intellectual outcomes than athyrotic patients do, and that early treatment improves the intellectual outcome of patients with congenital hypothyroidism.     

Ultrasound-Guided Aspiration and Ethanol Sclerotherapy (EST) for Treatment of Cyst Recurrence in Patients after Previous Endometriosis Surgery: Analysis of Influencing Factors Using a Decision Tree

Study ObjectiveTo evaluate the efficiency of transvaginal aspiration accompanied by ethanol sclerotherapy for treating cyst recurrence in patients who have previously undergone surgery to treat endometriosis and to analyze various factors that influence success rates using a data mining system.DesignRetrospective cohort study (Canadian Task Force classification II-3).SettingTeaching hospital affiliated with Chang Gung University, Taoyuan, Taiwan.PatientsOne hundred ninety-six patients with endometrioma recurrence.InterventionA total of 274 transvaginal aspirations followed by sclerotherapy with 95% ethanol. Treatment times varied from immediate removal (0–10 minutes) to in situ retention. Patients were followed up at 3, 6, and 12 months to detect complications, determine the size and persistence of cysts, obtain the pelvic pain score, and assess for pregnancy or the need for repeat surgical intervention. A decision tree was used to determine factors from the collected data that most influenced the success of treatment.Measurements and Main ResultsCyst size was consistently reduced until 6 months after ethanol sclerotherapy. The mean (SD) cyst reduction rate was 37.2% (42.2%), and the pain score reduction rate was 20.5% (71.5%). The antral follicle count was simultaneously increased by 36.4%. Sixty-three patients (23%) required repeated surgery during the observation period and were treated with either repeat aspiration (13.5%) or major laparoscopic or open laparotomic interventions (8.4%). Eighteen of 101 infertile patients (17.8%) achieved pregnancy. The total recovery rate (pregnancy or no persistence of symptoms or cyst) was significantly higher in patients in the groups that received longer treatment (7–10 minutes and retention) than in the groups with shorter treatment (0–6 minute) (47.0% vs 28.7%; p < .005). The highest recovery rate was observed in patients with longer treatment time, smaller cysts (≤5.05 cm), lower CA 125 level (≤62.03 IU/mL), and fewer cysts (≤3 cm) (35 of 49 [71.4%]). In patients with larger cysts and cysts with clear contents, better success can be achieved with longer treatment. The use of postoperative ovarian suppression, traditional Chinese medicine, or no therapy for 6 months before the study was not significant among groups.ConclusionUltrasound-guided sclerotherapy with 95% ethanol retention is an effective alternative therapy for recurrent ovarian endometrioma, in particular in selected patient groups.     

Intellectual outcome of patients with congenital hypothyroidism detected by neonatal screening.

BACKGROUND AND PURPOSE: Mental retardation is a major sequela of delayed treatment for congenital hypothyroidism; congenital hypothyroidism can be treated early if detected with neonatal screening. We evaluated the intellectual outcomes of 62 patients with congenital hypothyroidism detected by neonatal screening at a major teaching hospital in northern Taiwan. The effects of thyroid pathology, age at the initiation of treatment, socioeconomic status, and severity of hypothyroidism on intellectual outcome were also analyzed. METHODS: All patients had euthyroid status at the time of intelligence testing. The Chinese Fourth Revision of the Binet-Simon Scales was used to evaluate the patients' intelligence between the ages of 3 and 6 years. RESULTS: The mean intelligence quotient (IQ) score was 102 +/- 18. Only four of the 62 patients were mentally retarded. Patients with lower initial serum thyroxine concentrations (T4; < 2 micrograms/dL) at the time of diagnosis of congenital hypothyroidism had significantly lower IQs (95 +/- 19, n = 26) than those with higher initial T4 concentrations (106 +/- 16, n = 36; p < 0.05). Patients with fewer than three ossification centers had lower IQs (91 +/- 20, n = 12) than those with three or more (104 +/- 17, n = 36; p < 0.05). Significantly lower IQs were also found in patients with a smaller femoral epiphysis area (< 0.1 cm2) (92 +/- 20, n = 15) than in those with larger epiphyses (106 +/- 15, n = 21; p < 0.05). The type of pathology (ectopia, athyrosis, dyshormonogenesis), age at the start of treatment (before or after 30 days of age), and socioeconomic status did not significantly affect the intellectual outcome. CONCLUSIONS: Our results indicate that intellectual outcome in Taiwanese patients with congenital hypothyroidism has been improved by neonatal screening and that the severity of hypothyroidism at diagnosis is the most important prognostic factor affecting intellectual outcome in these patients.     

Clinical signs predict 30-month neurodevelopmental outcome after neonatal encephalopathy

OBJECTIVE: This study was undertaken to determine the value of a neonatal encephalopathy score (ES) and the presence of seizures for predicting 30-month neurodevelopmental outcome. STUDY DESIGN: In a cohort study, 68 term newborn infants with encephalopathy were evaluated with an ES based on alertness, feeding, tone, respiratory status, reflexes, and seizure activity (range: 0-6). Seizures were noted as present or absent clinically. Significant cognitive deficits (Mental Development Index <70), motor disability (spastic triplegia/quadriplegia), or death were abnormal outcomes. RESULTS: Twenty-two newborn infants (32%) had abnormal outcomes. With the use of maximum ES and presence of seizures from days 1 to 3 of life, 87% of newborn infants were correctly classified (area under receiver operating curve 0.93). By using ES and presence of seizures on day 1 only, 87% of newborn infants were correctly classified (area under receiver operating curve 0.89). CONCLUSION: The severity of neonatal encephalopathy and the presence of seizures are valuable predictors of 30-month neurodevelopmental outcome, as early as the first day of life.