眼睑痉挛研究及治疗进展

眼睑痉挛（BSP）是临床较为常见的、以不自主闭眼为特征的局灶型肌张力障碍。原发性、特发性和自发性眼睑痉挛统称为良性特发性眼睑痉挛（BEB）,无明确病因。多于成年期发病,女性多于男性,早期表现为瞬目增多,晚期可出现持续性闭眼甚至功能性失明。由于该病通常呈隐匿性,神经科和眼科检查常无异常发现,易误诊。在本文中,笔者就良性特发性眼睑痉挛的临床特征,以及流行病学、遗传学、发病机制、治疗措施及其相关研究进展进行介绍,拟为今后的临床与基础研究提供参考。     

眼睑痉挛的研究进展

<正>眼睑痉挛(BSP)是一种局限性肌张力障碍疾病,其特征为过度的不自主闭眼。原发性、特发性和自发性眼睑痉挛统称为良性特发性眼睑痉挛(BEB)。对BEB患者来说,随之而来的焦虑和抑郁症比疾病本身带来的困扰要严重[1]。由于该病通常隐袭起病,检查常无异常发现,给诊断带来了困难。1流行病学各国报道的肌张力障碍患病率各不相同,BEB发病率也略有差异。意大利南部肌张力障碍和BEB标化患病率分别     

7例眼睑痉挛型Meige综合征临床误诊分析

目的 探讨眼睑痉挛型Meige综合征的临床特点、诊断、误诊原因及治疗.方法 对上海市第一人民医院神经内科收治的7例误诊的眼睑痉挛型Meige综合征患者的临床特点、误诊原因及治疗进行回顾性分析.结果 眼睑痉挛型Meige综合征典型表现为不自主闭眼,临床上易被误诊为重症肌无力、神经官能症、眼睑炎和干眼症等.误诊的原因主要是对本病认识不足.本组7例患者经治疗后症状均有明显改善.结论 眼睑痉挛型Meige综合征发病早期症状不典型,易误诊误治,要注意和其他疾病鉴别.     

原发性眼睑痉挛的心理疏导与耳穴治疗

眼睑痉挛是眼科常见病,以不自主眼轮匝肌收缩为特征,分为原发性、特发性与继发性眼睑痉挛.以原发性眼睑痉挛最多见,一般认为与交感神经兴奋有关[1],药物治疗效果不佳,应用心理疏导与耳穴治疗可收到良好的效果.     

儿童良性癫痫伴中央颞区棘波的研究进展

儿童良性癫痫伴中央颞区棘波(Benign childhood epilepsy with centrotemporal spikes,BECTS)是一种儿童时期发病的癫痫综合征,属于特发性局灶性癫痫,在所有儿童癫痫中约占8％～23％.其临床特征为局灶性发作,发作间期脑电图显示特征性的中央颞区棘波.目前关于儿童良性癫痫伴...     

Meige综合征的临床及治疗

报道２０例Ｍｅｉｇｅ综合征的临床特征及药物治疗。其中５０岁以上发病者１０例，占５０％。就其临床分型来看，特发性眼睑痉挛９例，眼睑痉挛－口下颌肌张力障碍４例，此二型最为常见。目前，对Ｍｅｉｇｅ综合征的治疗均属探索性治疗，至今尚未发现一类药物对所有病例均有疗效，本组病人中，１０例服用氟哌啶醇有效。８例服用安坦有效，服用溴隐亭和卡马西平有效各１例，而左旋多巴对本病治疗无效。     

特发性眼睑痉挛—口下颌肌张力异常综合征9例报告

特发性眼睑痉挛一口下颌肌张力异常综合征又称Meige氏病,是成人多动症的一种,1910年由Henry Meige首先描述。其临床特征为眼睑痉挛及/或口面部肌肉对称性不规则多动收缩,部分病例伴颈部、躯干或中线部位肌肉痉挛性肌张力异常,多动不可控制,睡眠时消失。多误诊为神经症。我院自1981年至1983年诊治9例,报告如下。     

Meige综合征7例临床分析

Meige综合征又称特发性眼睑痉挛-口下颌肌张力异常(BS-OMD)综合征,1910年由Henry Meige首先描述,临床较少见,易被误诊为神经官能症等,且治疗比较困难.现将我科自1991～1998年诊治的7例报告如下.     

家族性特发性震颤二家系

<正> 特发性震颤(Essential tremor,ET)是原因不明的,以姿势性震颤为主要临床特征的常染色体显性遗传病,常有家族史,故又称为家族性唇颤或遗传性震颤。此病随年龄增长而患病率增加,临床症状呈缓慢进展,良性过程,故又称良性特发性震颤。自1993年以来,我们见到的37例ET患者中,有二家系中存在多人发病情况,现报告如下。 临床资料 家系1系谱如图1,先证者_1(Ⅲ_8),女60岁,因双手不自主震颤十八年就诊。患者42     

抗精神病药致Mejge综合征

1910年Meige描述一种特发性眼睑痉挛和口下颌张力障碍综合征。女性较男性为多,常发生于30～70岁,其特征是双眼睑痉挛和口腔周围、额、颈肌肉不自主运动,极少影响其它肌群,典型特征为累及眼轮匝肌。开始为眨眼次数增多,继而出现不可控制的强直性眼脸痉挛,影响视力,可持续几秒到几分。在仰视或紧张时可诱发,入睡和稍主动控制即能消失,似乎不可能是周围神经损害所致,本文报导3例Meige综合征,1例为特发性的,另2例则由抗精神病药引起。例1,女性,45岁,已婚。病前体健,无任何精神障碍。数年前在全麻下作不大的腹部手术后很快自觉眼睑痉挛,术后一个月觉眨眼次数增多,未加注意,逐渐出现眼肌     

Denervation study of synapses of organ of corti of old world monkeys

Fine structural characteristics of synapses in the spiral organ of Corti were examined, with reference to differences between inner and outer haircell systems, and to location of neurons of origin of efferent axons. Surgical interruption of crossed olivocochlear bundle, of vestibular nerve, of facial nerve, and excision of superior cervical ganglia were used to determine the pathways of efferent axons. Interruption of the vestibular nerve near the brainstem results in degeneration of all efferent terminals on outer hair cells. Mid-line lesions at, and caudal to, the facial colliculus result in degeneration of about half of these efferent terminals. Efferent synaptic bulbs to the inner hair-cell system are small, of the order of one micron, and form type 2 junctions with afferent dendrites. They tend to have more large dense-core vesicles (about 80 nm) than the large efferent terminals of the outer hair-cell system, and appear to be the terminals of axons in the habenula perforata, which exhibit varicosities laden with large dense core vesicles. The varicosities are unaffected by excision of the superior cervical ganglia. So far as our material can reveal, it appears that the varicosities in the habenula perforata do not survive vestibular root interruption, nor do the efferent processes in the internal spiral bundle or at the base of inner hair cells. Most interestingly, the afferent processes of the inner hair-cell system, as identified for example by their relation to pre-synaptic bodies in the inner hair cells, are subject to a trans-synaptic reaction after severance of the vestibular root. They undergo a dramatic cytological transformation, characterized by increase of volume, engorgement with microtubules, microfilaments, microvesicles of various sizes, and clusters of lysosomes. Thus, both the efferent and afferent terminals of the inner hair-cell system show marked cytological differences from the corresponding terminals of the outer hair cell system.     

Mechanism of action of tubocurarine on nicotinic cholinoreceptors of neurons of the sympathetic ganglia of rats

Tubocurarine (Tc) effect on membrane currents elicited by acetylcholine (ACh) was studied in isolated superior cervical ganglion neurons of rat using patch-clamp method in the whole-cell recording mode. The "use-dependent" block of ACh current by Tc was revealed in the experiments with ACh applications, indicating that Tc blocked the channels opened by ACh. Mean lifetime of Tc-open channel complex, tau, was found to be 9.8 +/- 0.5 s (n = 7) at -50 mV and 20-24 degrees C. tau exponentially increased with membrane hyperpolarization (e-fold change in tau corresponded to the membrane potential shift by 61 mV). Inhibition of the ACh-induced current by Tc (3-30 microM/1) was completely abolished by membrane depolarization to the level of 80-100 mV. Inhibition of ACh-induced current was augmented at increased ACh doses. It is concluded that the open channel block produced by Tc is likely to be the only mechanism for Tc action on nicotinic acetylcholine receptors in superior cervical ganglion neurons of rat.     

The effect of age of onset of PD on risk of dementia

Background Dementia occurs in the majority of patients with Parkinson’s disease (PD). Late onset of PD has been reported to be associated with a higher risk for dementia. However, age at onset (AAO) and age at baseline assessment are often correlated. The aim of this study was to explore whether AAO of PD symptoms is a risk factor for dementia independent of the general effect of age. Methods Two community-based studies of PD in New York (n = 281) and Rogaland county, Norway (n = 227) and two population-based groups of healthy elderly from New York (n = 180) and Odense, Denmark (n = 2414) were followed prospectively for 3–4 years and assessed for dementia according to DSM-IIIR. All PD and control cases underwent neurological examination and were followed with neurological and neuropsychological assessments. We used Cox proportional hazards regression based on three different time scales to explore the effect of AAO of PD on risk of dementia, adjusting for age at baseline and other demographic and clinical variables. Findings In both PD groups and in the pooled analyses, there was a significant effect of age at baseline assessment on the time to develop dementia, but there was no effect of AAO independent of age itself. Consistent with these results, there was no increased relative effect of age on the time to develop dementia in PD cases compared with controls. Interpretation This study shows that it is the general effect of age, rather than AAO that is associated with incident dementia in subjects with PD. Received in revised form: 22 December 2005     

Limits of deinstitutionalization--perspectives of relatives of psychiatric patients

After a hopeful beginning, the social process of the reintegration of those with severe mental illness has come to a standstill. I am led to wonder whether "the community" really wants to live together with people suffering from severe mental illness, and if so, how closely? As long as the medical treatment of mental illness provided by the general practitioners is fundamentally deficient, as they are not able to prescribe the necessary interventions--such as out-patient psychiatric nursing, and service providers in the out-patient sector are content with offering increasingly intensive forms of care for the less seriously ill at the cost of the Social Welfare System--the reintegration of those with serious mental illness remains an illusion--which is mainly to the benefit of providers of residential care in homes and hostels.