原发性震颤的诊断和治疗指南

原发性震颤(essential tremor,ET)也称特发性震颤,是一种常见的运动障碍性疾病,临床上以上肢远端的姿势性或动作性震颤为特点,可伴有头部、口面部或声音震颤,30%～50%的ET患者有家族史.传统观点认为ET是良性、家族遗传性、单症状性疾病,但目前认为ET是缓慢进展的、可能与家族遗传相关的复杂性疾病[1].     

原发性震颤的遗传学及治疗进展

原发性震颤（essential tremor，ET）是一种临床最常见的运动障碍性疾病，多发于40岁以上的中老年人群，临床表现以震颤为主。传统观点认为，原发性震颤是良性、家族性、轻微的单症状性疾病，随着研究的深入，目前认为原发性震颤为缓慢进展、可能与家族遗传相关的复杂性疾病（complex disease）。近年来，其遗传特点和治疗理念、方法都有了新的进展，笔者将围绕上述两方面的研究进展简要综述。     

特发性震颤非运动症状研究进展

特发性震颤是以姿势性或动作性震颤为主要表现的运动障碍性疾病,也是临床最为常见的神经系统疾病之一。传统观点认为,特发性震颤是一种单一症状的良性运动障碍性疾病,近年来,相关研究表明其亦可表现出非运动症状。对特发性震颤非运动症状的研究和探索,为临床诊断与治疗以及疾病机制的研究提供了新视角。     

原发性直立性震颤1例报道

正直立性震颤(orthostatic tremor,OT)是一种罕见的临床疾病。典型临床特征是患者由卧位变为立位时出现下肢震颤,当患者处于端坐位、平躺、或行走时,这种震颤可以明显减轻甚至消失。根据国外文献报道,脑干或小脑损伤、自身免疫性疾病、副肿瘤综合征、维生素缺乏以及使用多巴胺阻断性药物会引起继发性直立性震颤。但关于原发性直立性     

非典型性帕金森样病

非典型性帕金森样病亦称帕金森综合征,为一组临床表现多样的疾病症候群,除了覆盖原发性帕金森病(PD)的主要临床症状[静止性震颤、肌强直、运动不能和(或)运动迟缓、姿势反射障碍]外,还具有进展迅速、对左旋多巴反应不佳或其他特征性表现,如疾病早期易跌倒。与原发性帕金森病相比,非典型性帕金森样病包括原发性神经变性疾病,以及由药物、中毒、代谢性疾病或脑血管事件等导致的继发性症候群,而典型的原发性帕金森病是     

原发性震颤三家系分析

目的探讨原发性震颤的临床表现、家系特点及治疗。方法对3例原发性震颤患者的临床特点和家族史进行分析。结果ET在同一家族中男女均可发病,3例先证者一级、二级亲属患病率分别为：41.2%、40.0%和50.0%。结论原发性震颤是具有家族遗传性的疾病,以姿势性或意向性震颤为主,手及头部受累明显,多数患者饮酒试验阳性,β受体阻滞剂普奈洛尔治疗有效。     

氯硝基安定成功地治疗运动性震颤为主的原发性震颤

原发性震颤的特征是手、头、声音及偶尔腿或躯干的位置性和运动性震颤。作者报告运动为主的原发性震颤与典型原发震颤病人,对药物治疗反应的区别。     

原发性震颤的分子生物学研究进展

原发性震颤(essential tremo,ET)是一种常见的运动功能障碍疾病,是典型的姿势性和运动性混合的震颤,表现为头,面部,下颌,舌及上、下肢的震颤或节律性不自主运动.经典的ET属于单一的症状性运动障碍,但是在很多病例中常合并帕金森病(PD)、肌阵挛等其他疾病.     

原发性震颤的临床特点

原发性震颤 (ＥＴ)是一种病因未明、具有遗传倾向的常见运动障碍性疾病 ,人群患病率为 4 1‰～ 39 2‰。然而有关ＥＴ的临床分析及研究国内报道很少 ,现将 80例ＥＴ病人并对其临床特征进行分析总结如下。材料与方法 :ＥＴ的诊断综合了Ｂａｉｎ和Ｌｏｕｉｓ所提出的标准 ,并略作修改。①出现可见的和持续性的上肢姿势性震颤 ,伴有或不伴有动作性震颤 ,上肢的震颤可不对称 ,也可对称 ;或出现头部震颤。②排除与震颤相关的全身性和其他神经系统疾病 ,如帕金森病 (ＰＤ)、小脑病变、甲状腺功能亢进、心因性等因素以及药物、酒精所致的震颤。③病…     

原发性震颤的外科治疗

原发性震颤 (essentialtremor ,ET)是一种常见的发病原因和机理不明的运动障碍病 ,又称良性震颤或家族性震颤 ,约 6 0 %的患者有家族史 ,呈常染色体显性遗传特征。临床上ET主要表现为执行动作时出现震颤 ,症状单一。多数累及上肢、下颌和头颈 ,可以不对称 ,罕见下肢和躯干受累。药物治疗ET疗效不佳。症状严重的患者影响工作和生活 ,甚至失去自理生活能力。一、原发性震颤的流行病学和遗传学ET在老年人群中发病率的报道从 1 %到 2 2 %不等。最保守的估计 ,在 70岁以上人群中ET的发病率也有 1 .2 %到 2 %。一项研究证实 ,ET的发病率比PD…     

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.     

Summary of legislation of 1935 of interest to the department of Mental Hygiene

Psychiatric Quarterly -     

Summary of legislation of 1941 of interest to the Department of Mental Hygiene

Psychiatric Quarterly -     

Asymmetry of on- and off-pathways of blue-sensitive cones of the retina of macaques

Macaque retinal ganglion cells whose receptive-field center recieves input from blue-sensitive cones show an overt asymmetry of the frequency of ON-center and OFF-center varieties, an asymmetry not present in ganglion cells whose center receives input from the other two cone types. A similar asymmetry of ON/OFF responses is found in the local electrotetinogram (d-wave) mediated by signals from blue-sensitive cones. ‘Blue-ON-center’ ganglion cells have larger receptive-field centers and shorter conduction latencies than other opponent-color varieties, suggesting an appreciable degree of receptor convergence and presumably large cell bodies. Intracellular stainings of these neurons with Procion Yellow show that they correspond to diffuse stratified (Parasol) ganglion cells whose flat-topped dendritic arborization stratifies in the sclerad half of the inner plexiform layer. In view of the known characteristics of macaque bipolar cells and of the ON/OFF asymmetry, it is proposed that these ganglion cells are postsynaptic to cone-specific flat bipolars possibly mediating sign-inverting synaptic contacts. The results also indicate a reversal, for the blue-cone pathway, of the ON/OFF lamination of the inner plexiform layer that has recently been described in other species.     

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.