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。     

Focal brain dysfunction in a 41-year old man with familial alternating hemiplegia

The acute pathophysiologic changes during hemiplegic spells and the long-term outcome of alternating hemiplegia remain obscure. In a 41-year-old male with familial alternating hemiplegia we found an increase in right frontal cerebral blood flow 3 h into a 5-h left hemiplegic episode. A repeat high-resolution brain SPECT study performed 26 h after the resolution of the left hemiplegia revealed normalization of the frontal blood flow accompanied by hyperperfusion in the right parietal lobe. An interictal SPECT scan several weeks later showed no asymmetries. Head CT and MRI scans were negative. Neuropsychologic assessment and neurologic examination revealed evidence of a diffuse disorder which predominantly involved the right hemisphere. To our knowledge, there are no previous correlative studies of serial highresolution brain SPECT with MRI, or of detailed neuropsychologic assessment, in adult patients with such an advanced course of alternating hemiplegia of childhood.     

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。     

儿童交替性偏瘫六例分析

目的探讨儿童交替性偏瘫（ＡＨＣ）的临床特点及治疗方法。方法对６例ＡＨＣ患儿的临床资料进行分析。结果６例患儿的临床特征为出生后１８个月起内病，频繁发作，持续数分钟至数小时；短暂的眼球震颤，肌张力异常，舞蹈徐动样动作，植物神经机能紊乱和认知机能减退；睡眠可缓解无力及锥体外系症状。应用氟桂嗪治疗后，１例患儿发作完全停止，其余５例患儿均显示发作频率和持续时间降低。结论本病的主要特征为１８个月内起病的发作性交替性偏瘫，伴锥体外系症状及智力障碍，氟桂嗪治疗本病有效     

Benign nocturnal alternating hemiplegia of childhood: A new case with unusual findings

It has been described a neuro developmental disorder labelled “Benign nocturnal alternating hemiplegia of childhood” (BNAHC) characterized by recurrent attacks of nocturnal hemiplegia without progression to neurological or intellectual impairment. We report a female patient who at 11 months revealed a motionless left arm, unusual crying without impairment of consciousness and obvious precipitating factors. The attacks occur during sleep in the early morning with lack of ictal and interictal electroencephalographic abnormalities, progressive neurological deficit, and cognitive impairment. Unlike previous reports of BNAHC our patient come from a family with a history of both migraine, hemiplegic migraine, and sleep disorders. Our study remarks on the typical features described in previous studies and stresses the uncommon aspects that could help to identify the disorder which is likely to have been underestimated. Despite some clinical similarities between BNAHC and familiar hemiplegic migraine and alternating hemiplegia of childhood, the genetic analyses of our patient did not reveal genetic mutations found in both disorders.     

Alternating hemiplegia of childhood: case report

We report the case of a 31/2 year old girl who had had attacks of alternating hemiplegia from the age of 6 months. Peculiar features from the etiopathogenetic angle seem to be the presence of a ring of mild stenosis of the vertebral artery on the right side, the influence of the upright posture on the onset of the attacks and perhaps also of variations in atmospheric pressure. The only drug that had some effect was flunarizine. Phenobartbital, haloperidol and nadolol had no appreciable effect.

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Sommario Viene descritto il caso di una bambina di 3 anni e 6 mesi che ha presentato dall'età di 6 mesi attacchi di emiplegia alternante.Peculiari da un punto di vista etiopatogenetico sembrano essere la presenza di un lieve anello stenosante dell'arteria vertebrale di destra, l'influenza dell'ortostatismo nell'insorgere delle crisi e forse anche le variazioni della pressione atmosferica.Nel controllo delle crisi l'unico farmaco parzialmente efficace è risultato essere la Flunarizina, mentre nessun risultato apprezzabile hanno dato Fenobarbital, Aloperidolo, Nadololo.

     

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.     

体外循环心内直视手术时脑血流速度改变的研究

目的研究体外循环心内直视手术期间脑血流速度的变化和影响因素。方法在１７例患者心内直视手术时应用双通道经颅多普勒于术中动态监测大脑中动脉血流速度的变化。结果在体外循环转流期间，脑血流速度降低并随平均动脉压的改变有一定波动，血流频谱呈锯齿状。结论ＴＣＤ动态监测脑血流速度在体外循环心脏手术过程中对了解脑灌注的改变有一定价值     

TCD对颈内动脉中度及重度狭窄患者脑血流动力学的评估价值

目的应用经颅多普勒超声(transcranial doppler,TCD)评价颈内动脉中度及重度狭窄患者的脑血流动力学变化。方法经数字减影血管造影(digital subtraction angiography,DSA)确诊单侧颈内动脉中度及重度狭窄的患者69例,采用TCD检测其大脑中动脉血流参数,评价其侧支循环开放情况和脑血流储备能力(CRV)。结果 TCD显示侧支循环开放者患侧收缩期峰时血流速度(Vs)、搏动指数(PI)及CVR明显高于无侧支循环开放患者(P0.05),颈内动脉中度狭窄组患侧收缩期峰时血流速度(Vs)、PI值及CVR明显高于重度组(P0.05)。结论颈内动脉狭窄时侧支开放可以改善远端血流动力学指标,但改善程度有限。TCD可检测颈内动脉狭窄患者颅内血流动力学变化,评价颅内侧支循环的建立情况,为临床治疗和评估提供可靠依据。     

Abnormal cerebral glucose metabolism in alternating hemiplegia of childhood

Alternating hemiplegia of childhood (AHC) is a rare and intractable disorder of unknown cause. To determine cerebral neuronal function in five patients with AHC (two adults and three children), we analyzed brain glucose metabolism by positron emission tomography (PET) using 2-deoxy-2 [(18)F] fluoro-d-glucose (FDG), performed between hemiplegic attacks. Interictal FDG-PET revealed abnormal cerebral glucose metabolism; all patients showed low glucose metabolism in the frontal lobes with some laterality, and three had low glucose metabolism in the ipsilateral putamen. The adult patients also showed low glucose metabolism and mild atrophy in the cerebellum. Glucose metabolism in the brainstem was virtually normal for all patients. The areas of low glucose metabolism indicated local or regional neuronal damage, possible reflecting progressive neurological symptoms. AHC might therefore result from focal abnormal glucose metabolism in the brain occurring progressively or permanently, particularly in the frontal lobes and the cerebellum.     

Benign nocturnal alternating hemiplegia of childhood: Two cases with positive evolution

Benign nocturnal alternating hemiplegia (BNAH) of childhood is distinct from the classic form of malignant alternating hemiplegia of childhood [1]. It is characterized by hemiplegic attacks occurring exclusively during sleep [2]. It can be misdiagnosed as migraine, nocturnal frontal lobe epilepsy, benign rolandic epilepsy, Panayiotopoulos syndrome, or sleep-related movement disorder [1], [2], [3] and [4]. Only nine patients have been described to date, with typically, a normal development [1], [5], [6] and [7]. In order to insist about the benignity of the affection, we report two cases: a new three-year-old boy suffering from BNAH and a patient already published to show positive evolution at fourteen years of age. BNAH is a rare disorder but may be underdiagnosed. Making an early diagnosis can help to describe to the parents the good prognosis without treatment.     

Correlation between the arterial pulse wave of the cerebral microcirculation and CBF during breath holding and hyperventilation in human

Objective

To evaluate if relative changes in the amplitude of the arterial pulse wave of the cerebral microcirculation (APWCM) measured by near-infrared spectroscopy (NIRS) may provide information about relative changes of cerebral blood flow (CBF) in cerebral cortex.

Methods

In 10 healthy human volunteers, through simultaneous recording of the APWCM amplitude by means of NIRS and the mean blood flow velocity (MBFV) of middle cerebral artery by means of transcranial Doppler (TCD) at rest and during breath holding and hyperventilation, we evaluate a possible correlation between relative changes of the mean APWCM amplitude and relative changes of MBFV.

Results

We found a significant linear correlation: breath holding: R² 0.84, p < 0.001, hyperventilation: R² 0.81, p < 0.001.

Conclusion

The relative changes of the mean APWCM amplitude seem able to provide information about relative changes of CBF of cerebral cortex in healthy adult humans during breath holding and hyperventilation.

Significance

APWCM detected by NIRS, a safe, repeatable, inexpensive technology and at the bedside may improve the study of cerebral cortex microcirculation in neurological diseases.     

平衡训练对脑卒中偏瘫患者步行能力的影响

目的观察平衡训练对脑卒中偏瘫患者步行能力的影响。方法 92例脑卒中患者随机分为治疗组和对照组各46例,对照组给予常规康复治疗,治疗组采用常规康复训练加平衡功能训练,应用Fugl-Meyer评定中的平衡功能评定法和Holden功能步行分类在2组治疗前后对患者的平衡功能和步行能力进行评定。结果治疗前2组患者Fugl-Meyer评分和Holden评分差异无统计学意义（P〉0.05）;治疗后2组评分均较治疗前提高,但治疗组提高程度大于对照组（P〈0.05）。结论平衡训练可促进脑卒中偏瘫患者步行能力的恢复。     

Regional cerebral hemodynamics in childhood moyamoya disease

Regional cerebral blood flow (rCBF) and its reactivity to acetazolamide were analyzed in a series of 15 cases of childhood moyamoya disease, using the xenon-133 inhalation method and single photon emission CT (SPECT). Most of the patients had normal mean hemispheric cerebral blood flow (mCBF), comparable to that of age-matched healthy children. However, they had abnormal rCBF distribution and disturbed reactivity to acetazolamide in the territory of the internal carotid artery, especially in the frontal lobe. Surgical revascularization for the anterior circulation, which consisted of STA-MCA anastomosis and indirect synangiosis, dramatically improved theses cerebral hemodynamics in the anterior circulation, including the frontal lobe, and reactivity to acetazolamide in the whole brain. These results suggest that surgical revascularization for the anterior circulation should be carried out in order to improve cerebral hemodynamics as widely as possible, especially in the frontal lobe, and may improve cerebral perfusion reserve in the posterior circulation.     

Long-term follow up of an adult with alternating hemiplegia of childhood and a p.Gly755Ser mutation in the ATP1A3 gene

Alternating hemiplegia of childhood (AHC) is a rare neurological disease mainly caused by mutations in the ATP1A3 gene and showing varied clinical severity according to genotype. Patients with a p.Gly755Ser (p.G755S) mutation, one of minor genotypes for AHC, were recently described as having a mild phenotype, although their long-term outcomes are still unclear due to the lack of long-term follow up. Here, we demonstrate the full clinical course of a 43-year-old female AHC patient with p.G755S mutation. Although her motor dysfunction had been relatively mild into her 30?s, she showed a subsequent severe aggravation of symptoms that left her bedridden, concomitant with a recent recurrence of seizure status. The seizures were refractory to anti-epileptic drugs, but administration of flunarizine improved seizures and the paralysis.Our case suggests that the phenotype of AHC with p.G755S mutation is not necessarily mild, despite such a presentation during the patient’s younger years.     

The cerebral hemodynamics of repetitive transcranial magnetic stimulation

Repetitive transcranial magnetic stimulation (rTMS) has been shown to be effective in the treatment of affective disorders. However, only little is known about hemodynamic physiological and safety aspects of this method. We studied the cerebral hemodynamics as measured by transcranial Doppler sonography in 20 healthy subjects during different rTMS procedures. Mean cerebral blood flow velocity (CBFBV), pulsatility index (PI), and oxygen consumption were recorded continuously and averaged directly after the rTMS procedure. RTMS did not influence blood pressure, pulse rate, or blood oxygenation. There was a maximal increase of CBFV in the middle cerebal artery (MCA) of 3.6% and 5.6% during 10 Hz and 20 Hz stimulation, respectively. This increase was only seen on the stimulated left hemisphere. The PI remained unchanged during the whole procedure. It is likely that the increase of CBFV is due to dilatation of the small resistance vessels rather than due to vasoconstriction of the MCA. In terms of cerebral hemodynamics, rTMS is a safe and well-tolerated technique with a lower increase of CBFV than that seen in electroconvulsive therapy. Received: 22 March 2000 / Accepted: 4 July 2000     

脑血流自动调节下限的无创测定方法研究

目的探讨脑血流自动调节下限(LLCA)的无创测定方法。方法选择青年健康志愿者32人,用经颅超声多普勒仪、无创血压监测仪监测和记录大脑中动脉的血流流速、桡动脉血压,用常规法和傅立叶变换法分别测定两组临界关闭压(CCP)和LLCA值。结果用常规法测定CCP有4例为负值,这4例经傅立叶变换法测定均为正值。常规法测定的LLCA值为(66.76±9.14)mmHg,傅立叶变换法测定的LLCA值为(60.79±10.12)mmHg,两者比较有显著性差异。结论临床无创测定LLCA宜用傅立叶变换法。     

临界关闭压在脑血流动力学评价中的应用

目的探讨检测脑血流动力学的快捷、实用的新方法,为临床检测奠定基础。方法利用经颅多普勒(TCD)检测大鼠大脑中动脉(MCA)的血流速度,同步记录有创血压,按照临界关闭压(CCP)的理论计算出脑血流自动调节的下限和微动脉阻力,与改变血压测定的结果进行比较。结果CCP法检测到的脑血流自动调节下限为70.88±24.05mmHg,与常用血压改变测定的结果数值接近,可以相互替代。肾血管性高血压大鼠(RHR)的脑血流自动调节下限和微动脉阻力的升高,与动脉血压的升高,特别是脉压差的增大密切相关。结论按照CCP理论测定脑血流自动调节下限和微动脉的阻力,可以准确、快捷地反映脑血流动力学的生理状态和病理改变。     

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。