小儿癫(癎)持续状态36例临床分析

目的总结不同类型小儿癫持续状态(SE)的临床、脑电图特点及治疗反应,提高诊断及治疗水平。方法对36例临床发作及脑电图监测符合SE的患儿按发作类型进行分类,分析不同类型发作特点、病因、脑电图变化及治疗反应。结果症状性癫19例,隐源性癫14例,特发性癫3例。既往SE史14例,4例为首次发作。10例发病前智力、运动发育落后,5例发病后倒退。12例诊断为癫综合征。不同发作类型脑电图改变不同。非惊厥性癫持续状态(NCSE)患儿脑电图均表现为背景活动差,慢波多,其中4例伴有睡眠中癫性电持续状态。12例给予氯硝西泮,11例有效控制。结论不同类型的SE发作形式及脑电图表现不同,NCSE更为复杂,早期诊断和处理可提高生存率,减少神经后遗症的发生。     

儿童非癫性发作32例误诊分析

非癫性发作non-epilepticseizures,NES)又称为非癫性发作事件,是指多种原因引起的阵发性临床发作,不伴有发作期脑电图样放电,发作时间持续数秒至数分钟,甚至数小时[1]。据文献报告NES5%~20%被误诊为癫[2]。我院神经科2005年收治32例外院误诊为癫的NES患儿,现报告如下。1临床资料1.1一般资料本组32例,男18例,女14例;年龄2个月~15岁;均于外院诊断为癫,其中19例于求诊本院前已应用抗癫药物,用药时间1个月~2年。病程5天~3年。发作性症状:头痛、头晕8例,多发性抽动6例,癔症样发作4例,婴儿强直样NES4例,睡眠肌阵挛3例,屏气发作…     

癫持续状态27例诊治分析

目的探讨惊厥性癫持续状态的诊断及治疗方法,改善患者预后。方法回顾性分析27例惊厥性癫持续状态患者的临床资料。结果惊厥性癫持续状态其病因以脑血管病、特发性癫自行停药或减药、脑炎及脑外伤等为主;多数患者地西泮、丙戊酸、苯巴比妥钠等抗癫药物治疗有效,症状控制不良者给予丙泊酚可控制疾病发作;本组治愈23例,死亡3例,自动出院1例。结论癫持续状态多因脑血管病和特发性癫患者不规律服药所引起,脑炎及脑外伤也是常见病因;发作早期地西泮、苯巴比妥钠有良好治疗效果,丙泊酚可作为难治性癫持续状态的首选药物。     

癫持续状态27例诊治分析

目的探讨惊厥性癫持续状态的诊断及治疗方法,改善患者预后。方法回顾性分析27例惊厥性癫持续状态患者的临床资料。结果惊厥性癫持续状态其病因以脑血管病、特发性癫自行停药或减药、脑炎及脑外伤等为主;多数患者地西泮、丙戊酸、苯巴比妥钠等抗癫药物治疗有效,症状控制不良者给予丙泊酚可控制疾病发作;本组治愈23例,死亡3例,自动出院1例。结论癫持续状态多因脑血管病和特发性癫患者不规律服药所引起,脑炎及脑外伤也是常见病因;发作早期地西泮、苯巴比妥钠有良好治疗效果,丙泊酚可作为难治性癫持续状态的首选药物。     

儿童癫认知障碍研究进展

癫是儿童时期的常见病,多发病,约30%的癫患儿同时伴有认知功能障碍,主要表现为注意力、记忆力、推理判断及学习能力的下降。其原因是多方面的,包括致疒间灶部位、不同发作类型、发病年龄、病程、发作频率、抗癫药物及家庭社会因素均影响患儿认知。应用神经心理学评定量表、事件相关电位及功能影像学检查可以测量认知功能损伤程度。通过综合治疗可提高癫患儿的认知功能。     

维拉帕米对难治性癫大鼠的疗效及海马P-糖蛋白表达的影响

目的:观察耐药蛋白抑制剂维拉帕米对难治性癫大鼠的疗效及海马P-糖蛋白表达的影响。方法:Wistar大鼠50只,随机分为生理盐水组(NS组)、匹罗卡品组(PILO组)、卡马西平组(CBZ组)、低剂量维拉帕米组(LDV组)和高剂量维拉帕米组(HDV组),氯化锂-匹罗卡品腹腔注射制作大鼠自发性癫模型,选择耐药癫大鼠,分别给予常规或添加维拉帕米抗癫治疗,观察大鼠性发作频率及海马P-糖蛋白表达的变化。结果:与NS组相比,其它4组大鼠性发作频率增高,海马P-糖蛋白表达增强(P0.05);LDV组和HDV组大鼠的性发作频率及海马P-糖蛋白表达较PILO组和CBZ组降低(P0.05);PILO组和CBZ组大鼠性发作频率及海马P-糖蛋白表达无明显差异,LDV组和HDV组大鼠性发作频率及海马P-糖蛋白表达无明显差异。结论:添加维拉帕米治疗可明显减少难治性癫大鼠性发作频率,降低难治性癫大鼠海马P-糖蛋白的表达。     

AEEG监测106例婴幼儿癫性或非癫性发作的临床意义

发作性疾病或症状在婴幼儿较为常见,临床鉴别癫或非癫性发作较为困难。目前24小时动态脑电图AEEG)的应用,能长程监测病人的临床发作与脑电图的关系,为发作性疾病的诊断和鉴别诊断提供了可靠的依据。我院2002年4月~2005年7月共行AEEG监测婴幼儿256例,监测到发作性疾病106例,占41.4%,现分析报告如下。1临床资料1.1一般资料本组106例监测到发作的婴幼儿,均来自我院神经科门诊或病房,男60例,女46例,男女比例1.3∶1;年龄40天~3岁;病程1天~1年;临床表现分别为发作性意识改变、抽搐、凝视、口唇发青、四肢不自主运动、睡眠相关障碍等,临床…     

额叶癫(癎)的临床及脑电图特点

目的 分析和总结额叶癫(癎)的临床及脑电图特点.方法对80例确诊为额叶癫(癎)患者的临床和脑电图资料进行分析.结果①额叶癫(癎)发作形式:单纯部分性发作、复杂部分性发作及继发全身泛化.发作特点:发作时间短、运动突出,为强直性或姿势性,发作后意识障碍轻或无,发作频、夜间增多;②发作间期脑电图:额部导联的棘波或尖波,一侧或双侧多时性尖波或慢波;发作期脑电图:额部爆发性节律,很快同步化.结论额叶癫(癎)是一组较为特征性的癫(癎)综合征.     

左乙拉西坦单药治疗成人癫60例临床研究

目的:研究左乙拉西坦(LEV)单药治疗成人癫的临床疗效及副作用。方法:对60例成人癫患者进行LEV单药治疗的开放性自身对照研究,根据发作类型分为部分性发作组35例和全面性发作组25例,观察其疗效及副作用。结果:LEV单药治疗后癫发作完全控制率21.7%,有效率50%;部分性发作组完全控制率17.1%,有效率45.7%,全面性发作组完全控制率28%,有效率56%,2组间疗效比较差异无统计学意义。11例出现不良反应,包括嗜睡、头晕、食欲下降和兴奋激动,均可耐受。结论:LEV单药治疗成人各类型癫均有疗效,不良反应轻。     

额叶癫癎发作的临床表现及脑电图特征

目的:探讨额叶癫癎发作的临床表现与脑电图特征。方法:对36例额叶癫癎发作患者临床表现及脑电图特征进行同步分析。结果:额叶癫癎发作频繁而短暂,以睡眠中发作为主。常见发作形式为复杂部分性发作,主要表现为过度运动、偏转性强直、姿势性强直、发声及重复语言、额叶失神等。常规脑电图检查阳性率30.6%,长时间视频监测阳性率91.7%。结论:额叶癫癎发作是一组以运动症状为主的癫癎综合征,临床表现复杂多样,长程视频监测为无创性诊断额叶癫癎发作最佳方法。     

Hungarian migraine epidemiology

OBJECTIVE: A population-based epidemiological survey of migraine without aura and migraine with aura completed by mailed questionnaire in Hungary. The diagnosis and classification of migraine was according to the criteria of the International Headache Society. METHODS: The validity of the self-administered headache questionnaire was evaluated by a neurologist using clinical interviews. The study population was a random sample of 2000 men and women aged between 15 and 80 years. The questionnaire was completed by 813 of 1910 people (392 men and 421 women), providing a 42.6% participation rate. RESULTS: Sixty-seven percent of these people experienced some kind of headache during their lives. The 1-year prevalence of migraine without aura was 7.6% (the female/male ratio was 3:1), while the 1-year prevalence of migraine with aura was 2% (female/male ratio was 2:1). Most migraineurs experienced headache attacks 1 to 4 times monthly, which lasted 24 hours. The most characteristic accompanying signs were nausea and phonophobia. Stress, sleep deprivation, hot weather, and fasting were the most common precipitating factors of a migraine attack. In the migraine with aura group, the most common aura was a visual disturbance. Only 43% of migraineurs had ever consulted a physician for headache, and only 15% of patients had missed work or school because of a migraine attack in the previous year. Most of the patients suffering from migraine without aura were between 20 and 40 years old, while migraineurs with aura were over 40 years old. According to this investigation, the prevalence of migraine was not lower than in Western countries.     

发作性运动性舞蹈徐动症诊断与鉴别

目的　分析易误诊的发作性运动性舞蹈徐动症的临床特点。方法　分析发作性运动性舞蹈徐动症 2 1例 ,部分患者随诊。结果　在发作前均有明显的诱发因素 ,大多为突然运动、紧张、过度换气或惊吓。临床表现为发作性肢体的偏转、扭曲或者姿势的静止 ,多为一侧性 ,持续时间 1s～ 5 min,发作频率为每天 10余次或数月 1次不等 ,发作时意识清楚 ,发作间歇期完全正常。 6例有家族史 ,发病年龄 7～ 13岁。其电生理和神经影像学检查无明确异常。卡马西平类药物治疗有满意的疗效。结论　发作性运动性舞蹈徐动症是一种由运动诱发的、短暂的、发作性局部或全身不随意运动 ,属于离子通路疾病 ,用抗癫痫药疗效好     

Acute intermittent porphyria: biochemical and clinical analysis in the Argentinean population.

Acute intermittent porphyria (AIP) is the most common type of hepatic acute porphyria. In this work, we have analyzed the biochemical data of all Argentinean AIP families studied in the Porphyrins and Porphyrias Research Centre (CIPYP). We have shown that: (i) the prevalence for this population is about 1:125,000; (ii) the disease is more frequent in women than in men (7:3); (iii) about 60% are latent carriers; (iv) 15% of patients with symptomatic AIP died during an acute attack; (v) the most important precipitating factors of acute attacks in our population were the ingestion of therapeutic drugs (25%), anesthetics in surgical interventions (25%) and infections (20%); (vi) the initial symptom in Argentinean AIP individuals is severe abdominal pain (100%), and it is often accompanied by constipation (37%), anorexia (37%) and tachycardia (30%); and (vii) the percentage of recurrence of the acute attacks is high (81%).     

Migraine attacks and sleep in children

Falling asleep as a means of ending migraine attack was studied in 133 4-16-year-old children in out-patient settings. Children registered 999 migraine attacks in headache diaries using a visual analogue scale (VAS) in 409 attacks and a five-face scale in 590 attacks. The distribution of maximal pain intensity was similar on both scales; on VAS 88% assigned grades between 63 and 100, and on the face scale 93% assigned grades of 4 or 5. Children fell asleep during 33% of the attacks (n = 329), in 64% of these within the first hour (n = 209). Of the children, 68% (n = 91) had fallen asleep at least once during an attack. Falling asleep was more common in children under 8 years of age than in older children. In those under 8 years, 62% (95% confidence interval (CI) 49-75%) of attacks were resolved by sleep, in those aged 8-12 years 34% (26-41%), and in children >12 years 24% (15-33%) (ANOVA, P<0.0001). Pain was relieved without sleep in 43% (n = 431) of attacks, in 38% of these (n = 383) within the first 4 h. The data on migraine resolution were missing for 24% (n = 239) of the attacks, most often because the attack exceeded the 5-h observation period. This study confirms that migraine attacks in children are extremely painful and often resolve during an interval of sleep in children under 8 years of age.     

青春期后哮喘患者病情预后相关因素分析

目的 了解青春期后哮喘患者病情缓解的基本状况及相关因素.方法 对392例12～26岁的哮喘患者进行有关“青春期前后病情缓解的基本状况”的问卷调查.对与青春期后哮喘缓解有关的因素进行计数资料x2检验,将单因素分析筛选出的变量行多因素Logistic回归分析.结果 392例哮喘患者青春期后病情缓解率为66.6％(261/392),男性患者、女性患者缓解率比较差异无统计学意义.在青春期后病情未缓解的哮喘患者哮喘首次发作年龄及末次发作年龄均较缓解组大(均P ＜0.01).单因素x2检验分析显示:8岁后哮喘发作仍频繁者(年最多发作6次以上)其病情在青春期后缓解率较低(P＜0.01).伴发的变应性鼻炎症状越严重(中-重度)的哮喘患者青春期后的缓解率较低,与轻度或无明显鼻炎患者比较差异有统计学意义(P＜0.01).个人哮喘急性发作的高发季节在春夏秋季者其在青春期后哮喘病情缓解率较低,与冬季发病者比较差异有统计学意义(P＜0.05).血总IgE越高的哮喘患者,青春期后哮喘病情缓解率较低(P＜0.05).规范化的吸入激素联合特异性免疫治疗的哮喘患者在青春期后缓解率较高,与吸入激素组及未规范治疗组比较差异有统计学意义(P＜0.01).经常运动健身的哮喘患者在青春期后缓解率更高,与不经常运动健身的哮喘患者比较,差异有统计学意义(P＜0.01).而多因素logistic回归分析显示:8岁后哮喘发作越少、伴发的变应性鼻炎程度越轻、经过吸入激素联合特异性免疫治疗、经常运动健身、8岁前年患呼吸道感染3次以上及无明显变应原的哮喘患者在青春期后哮喘病情更易缓解(P＜0.05).结论 青春期后哮喘患者病情缓解率较高,减轻自身变态反应性炎症程度及给予积极的干预治疗措施如特异性免疫治疗及运动健身等,能使青春期后哮喘病情的缓解率提高.     

Efficacy of a fixed combination of indomethacin,prochlorperazine, and caffeine versus sumatriptan in acute treatment of multiple migraine attacks: a multicenter,randomized, crossover trial

OBJECTIVE: To compare the efficacy of a fixed combination of indomethacin, prochlorperazine, and caffeine suppositories with sumatriptan suppositories in the treatment of 2 consecutive migraine attacks of moderate or severe intensity in a multicenter, randomized, crossover study. BACKGROUND: A fixed combination of indomethacin, prochlorperazine, and caffeine is the most commonly used drug for the acute treatment of migraine in Italy. No studies have been published comparing the efficacy of this combination with sumatriptan, the most widely prescribed of the triptans. METHODS: One hundred twelve patients with migraine with or without aura according to the diagnostic criteria of the International Headache Society were randomized to treat 2 migraine attacks with a fixed combination of indomethacin, prochlorperazine, and caffeine and 2 migraine attacks with sumatriptan. Both drugs were rectally administered in a single dose for each attack. Patients were asked to take study medication as soon as possible at the onset of a headache. RESULTS: Of the 112 patients, 88 were compliant to the protocol. More attacks became pain-free at 2 hours postdose (primary end point) on the combination than on sumatriptan (49% versus 34%; P<.01), while there was no difference in the relief of headache at 2 hours postdose (71% versus 65%). The combination was statistically superior to sumatriptan in the time to a pain-free response (a higher percentage of attacks became pain-free from 0.5 hours postdose to 5 hours postdose), in alleviation of nausea, and in a sustained pain-free response (pain-free at 2 hours postdose with no use of rescue medication or relapses within 48 hours). Moreover, a significant consistent response was achieved for the combination compared with sumatriptan across (higher percentage of patients pain-free at 2 hours postdose in the first, second, third, and fourth treated attack) and within patients (pain-free in 2 of 2 treated attacks in 35% of patients taking the combination and 20% of patients on sumatriptan). Both drugs were well-tolerated. CONCLUSIONS: This study, analyzed according to the more recent guidelines for controlled trials in migraine, showed that a fixed combination of indomethacin, prochlorperazine, and caffeine is significantly more effective than sumatriptan in the acute treatment of migraine attacks. It is notable that the combination is less expensive than sumatriptan per unit dose.     

Ophthalmoplegia With Migraine in Adults: Is It Ophthalmoplegic Migraine?

Objective.— Ophthalmoplegic migraine (OM) is a rare disorder characterized by recurrent oculomotor nerve palsy in children, following migraine headaches. We report 62 adults, seen consecutively, who developed acute ophthalmoplegia with severe attacks of migraine over a 10-year (1996-2005) period. An overwhelming majority of these patients had an antecedent worsening in severity of migraine headaches, before the ophthalmoplegic attack.
Methods.— Sixty-two patients, aged 15-68 years, with an acute attack of OM underwent detailed clinical, biochemical, and neuroradiological evaluation.
Results.— There were 62 patients with 86 attacks of OM. Whereas 48 patients had a single attack, 14 had 2 or more attacks, fulfilling the International Headache Society criteria for probable and definite OM, respectively. At presentation, isolated abducens, oculomotor, and trochlear nerve involvements were seen in 35 (56.5%), 21 (33.9%), and 5 (8.1%) patients, respectively. One patient had simultaneous involvement of 3rd and 6th nerves. Fifty-one (82.3%) patients exhibited an antecedent worsening in severity of migraine, before developing ophthalmoplegia during (59/95.2%) or within 24 hours (3/4.8%) of a severe migraine attack, respectively. Detailed biochemistry and cranial neuroimaging were normal. No case had any nerve enhancement. Use of steroids hastened recovery ( P  < .05).
Conclusion.— We conclude: (1) OM in adults is characterized by single attacks of ophthalmoplegia in a great majority of patients; and (2) 6th nerve involvement occurs commonly. Our results indicate that moving OM to the chapter on cranial neuralgias in the second edition of the International Headache Classification may be premature, since nerve palsy occurred during a severe migraine attack in all patients.     

Migraine in children – an evaluation of the IHS criteria

The purpose of the present study was to compare the new criteria by the International Headache Society (IHS) with criteria for the definition of childhood migraine previously used. In particular, we considered the problem of the minimally acceptable headache duration in children. In a postal questionnaire, 36.5% of 3580 children 8–9 years old reported headache attacks. Ninety-five children (2.7%) fulfilled the IHS criteria. Three percent fulfilled the previously used migraine definition of Vahlquist. Some of the children in the latter group had considerably shorter headache attacks than those of the IHS patients, but otherwise their symptoms were very similar. When the IHS criterion of headache attack duration was lowered from a minimum of 2 h to 1 h, or omitted, the number of migraine patients increased from 95 to 114 and 127, respectively.