三例儿童交替性偏瘫的回顾性分析

目的探讨儿童交替性偏瘫病因、临床特点、治疗效果。方法回顾性分析3例儿童交替性偏瘫患者的病因、临床表现及疗效。结果本组3例患者起病年龄均小于18个月,反复发作的交替性偏瘫；进行性的智能障碍,其中1例伴有短暂眼球震颤及眼球活动障碍,1例伴有张力障碍性姿势异常；睡眠可缓解无力及锥体外系症状,应用氟桂嗪治疗后,2例患者发作频率及持续时间降低,1例无效。结论本病病因不明,多为散发,临床表现为18月内起病的发作性交替性偏瘫,辅助检查无特征性改变,氟桂利嗪治疗部分有效。     

儿童交替性偏瘫(附3例报告)

目的探讨儿童交替性偏瘫（AHC）的临床特点和治疗方法。方法对3例AHC患儿的临床资料进行分析。结果3例患儿的临床特征为生后18个月内起病．反复发作、时间不等的交替性偏瘫，伴有眼位异常，肌张力异常，舞蹈样徐动动作，植物神经机能紊乱和智力障碍；睡眠可缓解。结论本病的主要特征为生后18个月内起病的发作性交替性偏瘫，伴有锥体外系症状及智能障碍，Flunarizine治疗有效。     

儿童交替性偏瘫一例

目的了解儿童交替性偏瘫(AHC)的诊断及治疗方法的合理选择。方法介绍我院收治的1例难治的儿童交替性偏瘫患儿的临床特点,检索国内外10年内的相关文献。结果本病的临床特点为反复发作性的偏瘫,睡眠后缓解,可有其他发作性症状,如发作性肌张力障碍、眼球运动异常和认知障碍等。氟桂利嗪是最常用的药物,托吡酯片、阿立哌唑也可用于治疗本病。结论本病是一种罕见的神经系统发作性疾病,临床表现多样,容易误诊,目前没有标准的治疗方法。     

托吡酯治疗儿童交替性偏瘫的疗效观察

目的 观察托吡酯(TPM)治疗儿童交替性偏瘫(AHC)的临床疗效.方法 对氟桂利嗪等药物治疗无效的6例AHC患儿应用TPM治疗,观察TPM治疗后偏瘫及伴随的癫(癎)和偏头痛的发作频率、持续时间及严重程度的变化.结果 TPM治疗6个月后患儿偏瘫发作频率降低76.4%、持续时间减少80.2%,严重程度亦降低.癫(癎)发作频率和持续时间分别降低84.5%和75.5%,偏头痛发作频率和持续时间分别降低77.5%和76.4%.结论 TPM对氟桂利嗪等治疗无效AHC患儿具有较好的疗效.     

儿童交替性偏瘫的临床特征和脑血流灌注改变

目的探讨儿童交替性偏瘫的临床特点及脑血流改变。方法对近23年收治的11例患儿的临床资料进行分析,并对6例患儿应用单光子发射计算机断层扫描进行脑血流灌注观察,其中4例在发作间期,1例在发作期,1例在发作间期和发作期均进行测定,以兴趣区法作半定量分析。结果11例患儿的临床特征一般为18个月内起病,表现为频繁发作的交替性偏瘫、短暂的眼球震颤、肌张力异常、舞蹈徐动样动作,常伴自主神经功能紊乱和认知功能减退;睡眠可缓解上述症状。2次发作期单光子发射计算机断层扫描均示偏瘫对侧脑血流减少,5次发作间期均正常。结论本病的主要临床特征为一般18个月内起病的反复发作的交替性偏瘫,伴锥体外系症状及智能障碍;发作期偏瘫对侧脑血流减少。     

儿童交替性偏瘫

儿童交替性偏瘫(AHC)是一种罕见的综合征。其特征是频繁发作一过性偏瘫,可累及身体任何一侧或两侧。症状首发于18个月内。常伴有发作性眼球运动障碍、植物神经症状、肌张力障碍姿式(dystonic posturing)及舞蹈指痉病(choreathetosis,其典型的发作过程是以痛性尖叫和躁动为先兆,继无出现眼球运动异常及肌张力障碍,之后出现一侧肢体软瘫。一述发作常因睡眠而戏剧性缓解。为了解AHC的临床表现、病程、预后及对氟桂嗪治疗的反应,作者对10例AHC患者进行观察和治疗。 10例(男3例,女7例),平均年龄11岁(3～     

氟桂嗪致锥体外系反应11例报告

氟桂嗪致锥体外系反应１１例报告刘希涛李美清蔡佰元赵军绩近几年来我们遇到的１１例由氟桂嗪引起的锥体外系反应的病例，现将其临床资料及其处理方法报告如下。一般资料：１１例病人中，男性８例，女性３例：年龄为３１～７２岁，平均年龄为５０±１０．６岁。椎基底动脉...     

氟桂嗪治疗Tourette综合征的临床疗效观察

目的探讨氟桂嗪是否有治疗Tourette综合征(多发性抽动秽语综合征)的作用.方法163例患儿分为3组:泰必利组和泰必利加氟桂嗪治疗组以及单用氟桂嗪治疗组,用药和观察时间至少为6个月,用"不自主运动量表(AIMS)"评分法来评定治疗后抽动症状的改善程度.结果66例泰必利组中有24例抽动症状消失,痊愈率为36.36%;66例泰必利加氟桂嗪治疗组中有36例抽动症状消失,痊愈率为54.55%,两者痊愈率相比差异有显著性意义(P＜0.05).31例单用氟桂嗪治疗组中"轻度"患儿的痊愈率达100%,即病情偏轻者效果较佳,病情严重者效果较差.结论氟桂嗪对Tourette综合征确有治疗作用,其药理机制可能与氟桂嗪的抗多巴胺能活性作用有关.     

氯丙咪嗪与丙咪嗪治疗200例抑郁症对比分析

氯丙咪嗪与丙咪嗪治疗２００例抑郁症对比分析王幼坤陈薇作者对１９８６年３月～１９９２年４月期间住院，符合ＣＣＭＤ—２抑郁症诊断标准患者随机均分为两组，分别以氯丙咪嗪与丙咪嗪进行治疗。所有病例ＨＡＭＤ１７项评分均≥１８分，且无严重躯体疾患。氯丙咪嗪组男６...     

盐酸氟桂利嗪胶囊治疗月经期偏头痛的临床疗效观察

目的探讨盐酸氟桂利嗪胶囊治疗月经期偏头痛的临床疗效及最佳治疗方案。方法将68例月经期偏头痛患者随机分为两组,A组为常规治疗组(每周服用盐酸氟桂利嗪胶囊5天,停用2天),B组为按生物节律周期治疗组(在月经来临前十天服用盐酸氟桂利嗪胶囊,连续服用20天)。所有患者均于睡前服用5mg盐酸氟桂利嗪胶囊,并记录头痛日记和药物不良反应;根据头痛程度、发作频率、持续时间和伴随症状的变化情况,经量化记分,于用药1个月、3个月、6个月进行疗效判定,同时比较药物不良反应的发生率。结果 1个月和3个月两组间盐酸氟桂利嗪胶囊疗效无明显差异(P>0.05),但6个月时,B组患者头痛程度、发作频率、持续时间、伴随症状与A组相比,明显减轻/减少(P<0.001);两组间药物不良发应发生率无差异(P>0.05)。结论盐酸氟桂利嗪胶囊能够有效的防治月经期偏头痛;根据生物节律周期即女性雌激素分泌水平变化给予盐酸氟桂利嗪胶囊防治月经期偏头痛疗效更佳。     

Topiramate: a new agent for patients with alternating hemiplegia of childhood

Alternating hemiplegia of childhood is a rare syndrome characterized by the onset, before 18 months of age, of frequent attacks of alternating paralysis. Here we report the efficacy of topiramate in four patients with alternating hemiplegia of childhood (AHC) that did not respond to flunarizine, as well as in two newly diagnosed patients. Following treatment with topiramate, the frequency and duration of hemiplegic attacks significantly improved in all patients. Additional symptoms such as seizures, migraine, involuntary movements, autonomic symptoms, and impaired mental development also improved. Topiramate is worth trying when treating patients with AHC as a first trial, or a substitute for flunarizine once the latter agent loses effect.     

Treatment of alternating hemiplegia of childhood with aripiprazole

We report the pharmacological treatment of a case of alternating hemiplegia of childhood (AHC) in a 14-year-old female with an established diagnosis. Although the patient's symptoms are consistent with those of the condition, she did not respond to treatment with haloperidol, flunarizine, or propranolol. Treatment with aripiprazole resulted in a reduction in the frequency, duration, and severity of episodes of alternating hemiplegia, along with other therapeutic benefits. After treatment with aripiprazole was started, the patient was inadvertently given an inactive drug, resulting in a worsening of her hemiplegic episodes, which improved again on rechallenge. A comparison of the pharmacological actions of successful and unsuccessful treatments for AHC is made. Modulation of both dopamine and histamine systems together appears to be important in the treatment of AHC and further investigation of such pharmacotherapies is suggested.     

Long-term effect of flunarizine on patients with alternating hemiplegia of childhood in Japan

To determine the effect of flunarizine therapy on patients with alternating hemiplegia of childhood (AHC), we sent a questionnaire by mail to council members of the Japanese Society of Child Neurology. We collected 28 AHC patients, and studied their clinical courses and the effects of drug therapy. All of the patients had received flunarizine. In 18 of the 28 patients, flunarizine reduced the severity, duration, or frequency of the hemiplegic attacks. No other drug was more effective than flunarizine. Some flunarizine non-effective patients were severely deteriorated, for example, they had dementia or were ventilator-assisted. Flunarizine had not only a short-term effect, i.e. it reduced the hemiplegic attacks, but also a long-term effect on the motor and intellectual development in some patients with AHC. Flunarizine is still an essential drug for treating AHC.     

Successful trial of amantadine hydrochloride for two patients with alternating hemiplegia of childhood

We report here the efficacy of amantadine hydrochloride for two patients with alternating hemiplegia of childhood (AHC) that did not respond to flunarizine. Amantadine was administered to one patient at age one year and seven months and to the other at age 25 years. The frequencies and duration of the hemiplegic attacks significantly improved in both patients. However, the attacks gradually returned to the previous level after a significant reduction in seizures for three years in the younger patient with ongoing AHC. Our therapeutic results further support the hypothesis that glutamate and NMDA receptors are involved in inducing alternating hemiplegic attacks, because amantadine as well as its derivative, memantine, are clinically available non-competitive N-methyl-D-aspartate (NMDA) receptor antagonists, with neuroprotective effects. Amantadine is worth trying when treating patients with AHC as a first trial or a substitute for flunarizine once the latter agent looses effect.     

Alternating hemiplegia of childhood: New diagnostic options

A syndrome of alternating hemiplegia of childhood (AHC) is a rare disorder first presented in 1971. AHC is characterized by transient episodes of hemiplegia affecting either one or both sides of the body. Age of onset is before 18 months and the common earliest manifestations are dystonic or tonic attacks and nystagmus. Hemiplegic episodes last minutes to days and the frequency and duration tend to decrease with time. Motor and intellectual development is affected, deficits may also develop later. Epileptic seizures occur in some patients. Neuroimaging of the brain usually reveals no abnormalities. The variability of individual clinical presentations and evolution of symptoms have made diagnosis difficult. Therefore the problems of misdiagnosis could account for the low prevalence of this syndrome. This paper hopes to present actual data on AHC, especially of the results of genetic research and new diagnostic tools.     

Absence of small-vessel abnormalities in alternating hemiplegia of childhood

Objective: To investigate whether Japanese patients with alternating hemiplegia of childhood (AHC) have the similar small-vessel abnormalities in skin reported in European patients with AHC. Methods: Electron microscopic observation of biopsied skin specimens were carried out in six Japanese patients with AHC. All patients (aged 5-17, all boys) had been diagnosed with AHC through their typical clinical courses and symptoms. Results: No abnormal findings in both endothelial cells and smooth muscle cells in skin small-vessels were obtained in the present study, either in the five flunarizine responders or in the one non-responder. Conclusions: From our observations, we hypothesized that there may be some subtypes of AHC. The diverse clinical courses in patients with AHC and the differing efficacy of flunarizine treatment could be explained because of the heterogeneity of AHC subtypes.     

A boy with nystagmus, refractory dystonia and apneic attack due to alternating hemiplegia of childhood

We herein report the findings of a 2-year-6-month-old boy, who had been experiencing monocular pendular nystagmus, strabismus, and episodic eye deviation nystagmus, intractable dystonia and apneic attack which all began when he was 2 days of age. He underwent a complete blood count test, blood chemistry test, analysis of amino acids in the blood and urine, analysis of pyruvate/lactate in blood and cerebrospinal fluid, head computed tomography and magnetic resonance imaging and no abnormal results were identified. His attacks were resistant to multiple antiepileptic and dopaminergic drugs. He showed transient left and/or right hemiplegia after nystagmus, dystonia and/or apneic attacks at 8-months of age with retardation in intelligence. We diagnosed him to have alternating hemiplegia of childhood (AHC). We were unsure how to deal with his attacks after he was discharged from the hospital, however, resuscitation with the ambu bag by his mother at home and the intravenous infusion of diazepam or thiamylal at the hospital together was proven to be an effective method for treating his severe apneic attacks. The effect of diazepam and amantadine on these attacks was transient, however, the administration of flunarizine with amantadine resulted in an improvement in his attacks. We therefore consider the administration of flunarizine to be essential for the effective treatment of AHC in this case.     

An 11-year follow-up study of neonatal-onset, bath-induced alternating hemiplegia of childhood in twins

The authors previously reported on the initial manifestations in a set of female twins, who presented soon after birth with bath-induced paroxysmal events each time they were immersed in a warm water bath. These episodes progressively ceased by the age of 36 months, replaced by paroxysmal episodes of alternating hemiplegia unrelated to water immersion. By age 4 years, the twins developed the classic features of alternating hemiplegia of childhood. Clinical outcomes at the age of 11 years are now reported. Standard and video-electroencephalograms showed a large, slow background activity followed by lower amplitude waves without focal abnormalities or other abnormal findings. This represents the first report on (a) alternating hemiplegia of childhood started with bath-induced paroxysmal episodes; (b) this condition in monozygotic twins; and (c) an 11-year follow-up study in which the twins continue to experience episodes of alternating hemiplegia in the setting of baseline cognitive impairment without epileptic episodes.