Meige综合征临床误诊九例分析

目的 探讨Meige综合征的临床特点、诊断、误诊原因及治疗. 方法 对广州市第一人民医院脑系外科收治的9例误诊的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 Meige综合征典型表现为阵发性双侧眼睑痉挛,伴或不伴有双侧口颌部及其他部位肌肉不随意、对称性、不规则阵发性收缩,临床上易被误诊为重症肌无力、面肌痉挛、神经官能症和迟发性运动障碍等,误诊的原因主要是对本病认识不足.本组9例患者经治疗后症状均有明显改善.结论 虽然Meige综合征临床特点较明显,但发病早期易误诊,要注意和其他疾病鉴别.     

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.     

Meige综合征临床误诊二例

Meige综合征属锥体外系的一种少见疾病,在1910年首先由法国神经病学家Meige描述,典型特征是眼睑痉挛及口-下颌肌肉不自主运动等.临床上常被误诊为面肌痉挛、神经官能症、重症肌无力等.为引起同道的高度重视,现将2例误诊的病历报道如下.     

Meige综合征14例临床分析和文献复习

目的观察Meige综合征的临床特点、诊疗经过,复习相关文献以提高对本病的认识。方法分析14例Meige综合征的临床表现、诊疗和预后。结果14例眼睑痉挛型9例,眼睑痉挛合并口下颌肌张力障碍型4例,口下颌肌张力障碍型1例。结论Meige综合征应和干眼症、面肌痉挛、手足徐动症、口舌运动障碍、重症肌无力等相鉴别;氟哌啶醇、泰必利联合应用治疗Meige综合征有效。     

A型肉毒毒素治疗面肌、眼睑痉挛156例临床观察

目的 探讨A型肉毒毒素治疗面肌，眼睑痉挛及Meige综合征的疗效。方法 采用A型肉毒毒素局部注射治疗偏侧面肌痉挛102例。眼睑痉挛41例及Meige综合征13例。并使用Cohen和Albert量表进行评估。结果 症状完全缓解占51.3%。明显改善占37.8%。部分改善占10.9%。疗效平均持续约3-6个月。复发者重复注射仍有效，出现眼睑闭合不全，面肌无力，眼睑下垂共58例，均恢复。结论 局部注射A型肉毒毒素确为一种安全有效。简便易行的治疗面肌。眼睑痉挛及Meige综合征的方法。     

A型肉毒毒素加用抗痉挛药治疗Meige综合征的疗效观察

目的 观察A型肉毒毒素及抗痉挛药物联合治疗Meige综合征的疗效。方法 对30例Meige综合征患者应用A型肉毒毒素对痉挛肌肉多点注射，每点0．1－0.2ml(2.5-5U)，同时服用抗乙酰胆碱和苯二氮Zhuo类药物。结果 A型肉毒毒素对眼睑痉挛的治疗，4－5天症状缓解，1个星期明显缓解，2个星期疗效达最高峰。有效率100％。有效作用时间为10－24周，平均11．7周。而抗乙酰胆碱和苯二氮Zhuo类药物对口下颌肌张力障碍的治疗效果比较明显，可以有效的缓解患者的磨牙咂嘴和舌不自主伸缩等症状。结论 A型肉毒毒素和抗乙酰胆碱及苯二氮Zhuo类药物联合应用是治疗Meige综合征的一种安全、有效、简便的方法。     

A型肉毒毒素治疗局限性肌肉痉挛的临床研究

目的　观察A型肉毒毒素治疗偏侧面肌痉挛、眼睑痉挛及Meige综合征的疗效。方法　对 40例偏侧面肌痉挛、9例眼睑痉挛、1例Meige综合征进行面部肌肉局部多点注射A型肉毒毒素 ,评价其治疗效果。结果　 40例偏侧面肌痉挛者 ,完全缓解 1 5例 (38% ) ,明显缓解2 4例 (60 % ) ,1例无效 ;9例眼睑痉挛者 ,4例完全缓解 ,4例明显缓解 ,1例无效 ;1例Meige综合征部分缓解 ,总有效率达 96 %。起效时间平均 3天 ,缓解时间平均 3 5个月。局部副反应轻微、短暂 ,无全身反应及过敏反应。结论　A型肉毒毒素局部肌肉注射是治疗局限性肌肉痉挛的一种安全、有效的治疗方法。     

吉兰-巴雷综合征30例临床分析

目的分析吉兰-巴雷综合征的临床特点。方法回顾性分析我院收治的30例吉兰-巴雷综合征住院患者的临床资料、实验室检验结果、治疗及预后。结果吉兰-巴雷综合征患者男性多发,且随年龄增长发病率升高;起病前有呼吸道或消化道感染史9例,脑脊液实验室检查正常7例,症状不典型误诊3例,肌电图检查阳性率100%;丙种球蛋白治疗有效率80%(20/25);肢体无力严重、合并呼吸肌麻痹以及治疗延后的患者疗效不佳占50%(7/14)。结论不典型症状起病的吉兰-巴雷患者易误诊,丙球治疗疗效好但需及时,肌无力重、合并呼吸肌麻痹患者预后差。     

5例成人MELAS型线粒体脑肌病的误诊原因分析

目的 分析成人MELAS的误诊原因.方法 回顾分析5例成人MELAS的临床特点.结果 成人MELAS的临床症状、体征及影像学表现多样,早期症状不典型,易误诊为其他神经系统疾病.结论 对于临床症状无法解释的中青年卒中、癫痫患者首先应考虑该病.     

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。     

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.     

Sense of coherence as a predictor of subjective state of health: results of 4 years of follow-up of adults

A number of cross-sectional population studies have shown that a strong sense of coherence (SOC) is associated with various aspects of good perceived health. The association does not seem to be entirely attributable to underlying associations of SOC with other variables, such as age or level of education. OBJECTIVE: The aim of the study reported here was to determine whether SOC predicted subjective state of health. METHODS: The study was carried out as a two-way panel mail survey of 1976 individuals with 4 years interval for two collections of data. The statistical method used was multivariate cumulative logistic modeling. Age, initial subjective state of health, initial occupational training level, and initial degree of social integration were included as potential explanatory variables. RESULTS: A strong SOC predicted good health in women and men. CONCLUSIONS: SOC can be interpreted as an autonomous internal resource contributing to a favorable development of subjective state of health. SOC data should, however, be regarded as complementary to and not a substitute for information already known to be associated with increased risk of future ill health.