关键词： 脱髓鞘性   环孢霉素   多发性神经炎   谷浓度   红细胞糖苷脂   正中神经   临床症状   神经生理学   葡糖醛酸基   总蛋白量  

摘要： To investigate the therapeutic efficacy of cyclosporin A (CyA) in the treatmen t of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), a microem ulsion form of this drug (Neoral) was orally given to seven patients with the disease who were unresponsive or resistant to conventional *** daily d ose of CyA was carefully controlled in order to keep the plasma trough concentra tion between 100 and 150 ng/ml. Within 1 month of initiation of CyA, all patient s subjectively showed improvement of clinical symptoms, while both modified Rank in and INCAT disability scores were significantly decreased (p < 0.05) and grip strength was significantly increased (p < 0.05) 3 months after initiation compar ed with before. Total protein in the cerebrospinal fluid was significantly decre ased 3 and 6 months after starting CyA (p < 0.05). Although the maximal motor ne rve conduction velocity showed a significant improvement in the median nerve 1 t o 1.5 years after commencement of CyA (p < 0.05), there were no significant chan ges in any other neurophysiological parameters. One patient with anti sulphoglu curonyl paragloboside IgM antibodies gradually became resistant to CyA, but the rest have since been in good neurological con dition without complications ascribable to this drug. These results suggest that oral CyA may be effective even for refractory cases with CIDP. CyA should be ac tively considered as a therapeutic option when patients with CIDP are resistant to conventional treatment.

关键词： GDAP1   基因突变   奠基者效应   轴突损伤   髓鞘   腓肠神经活检   常染色体隐性   多发神经病   神经生理学   足畸形  

摘要： Background: Mutations in a gene encoding a novel protein of unknown function the ganglioside induced differentia tion associated protein 1 gene (GDAP1) are associated with the autosomal recessive Charcot Marie Tooth disease type 4A (CMT4A). Objective: To investigate the role of GDAP1 mutations in causing au tosomal recessive neuropathies in an Italian population. Methods and results: 76 patients with severe early onset polyneuropathy and possible autosomal recessiv e inheritance were screened for mutations. A T > G transversion (c.347 T > G) at codon 116 (M116R) was detected in four affected subjects from three apparently unrelated families. All patients had early onset of disease with pronounced foot deformities and impaired walking. Neurophysiological studies showed an extremel y variable expression. Sural nerve biopsies revealed signs of both de remyelina tion and axonal impairment, the most prominent feature being a severe loss of la rger fibres. Haplotype analysis of the GDAP1 locus demonstrated a common disease haplotype. Conclusions: The association of the mutation with a common haplotype suggested a common ancestor.

关键词： 帕金森病   运动皮质   经颅磁刺激   左旋多巴   评分量表   侧运动   临床评估   药物治疗   神经生理学   电生理特征  

摘要： Objective: To assess the effects of focal motor cortex stimulation on motor pe rformance and cortical excitability in patients with Parkinsons disease (PD). Methods: Repetitive transcranial magnetic stimulation (rTMS) was performed on th e left motor cortical area corresponding to the right hand in 12 offdrugpati ents with PD. The effects of subthreshold rTMS applied at 0.5 Hz (600 pulses) or at 10 Hz (2000 pulses) using a realor a shamcoil were compared to those obtained by a single dose of l dopa. The assessment included a clinical evalua tion by the Unified Parkinsons Disease Rating Scale and timed motor tasks, and a neurophysiological evaluation of cortical excitability by single and paired pulse TMS techniques. Results:RealrTMS at 10 or0.5 Hz, but notshamstim ulation, improved motor performance. High frequency rTMS decreased rigidity and bradykinesia in the upper limb contralateral to the stimulation, while low fre quency rTMS reduced upper limb rigidity bilaterally and improved walking. Concom itantly, 10 HzrTMS increased intracortical facilitation, while 0.5 HzrTMS restor ed intracortical inhibition. Conclusions: Low and highfrequency rTMS of the pri mary motor cortex lead to significant but differential changes in patients with PD both on clinical and electrophysiological grounds. The effect s on cortical excitability were opposite to previous observations made in health y subjects, suggesting a reversed balance of cortical excitability in patients w ith PD compared to normals. However, the underlying mechanisms of these changes remain to determine, as well as the relationship with clinical presentation and response to l dopa therapy. Significance: The present study gives some clues to appraise the role of the primary motor cortex in PD. Clinical improvement induc ed by rTMS was too short lasting to consider therapeutic application, but these results support the perspective of the primary motor cortex as a possible targe t for neuromodulation in

关键词： CRT monitor   duty cycle   flicker sensitivity   photometry   luminance   OSCILLOSCOPIC DISPLAYS   PHOSPHOR PERSISTENCE   TEMPORAL CONTRAST   CORTICAL-NEURONS   VISUAL DISPLAYS   SENSITIVITY   FLICKER   CONE   CALIBRATION   VISIBILITY  

摘要： We demonstrate that cathode-ray-tube (CRT) monitors commonly used as stimulus generators in visual neuroscience produce signal artefacts. This arises from two factors, one being the finite time needed for the raster scan of the CRT to cross the receptive field being stimulated. and the other being the restraint imposed by the impulse response of the phosphor itself. Together these factors result in smearing or blurring that manifests as high frequency noise, distorting the desired signal applied by the investigator. Our analysis identifies those conditions that promote these artefacts and we describe methods for their minimisation. We suggest that a monitor frame rate greater than or equal to 100 Hz provides a reasonable trade-off between refresh and the generators of high frequency noise. (C) 2004 Elsevier B.V.. All rights reserved.

关键词： 记忆   扣带回/病理学   情绪   神经生理学   疼痛/心理学   综述   人类  

摘要： 感觉分辨和情绪体验是疼痛的两个基本成分。痛的感觉分辨是对刺激的部位、强度和性质作出判断;痛情绪是疼痛刺激引起的不愉快或厌恶的情感体验。过去四十年间,对疼痛的感觉分辨成分在整体、细胞、分子和基因等各个层次上的研究取得了巨大的进展,然而,对痛的不愉快的情绪体验和相关的记忆究竟是怎样产生的,还知之甚少。“只考虑痛的感觉特性而忽视其情绪情感特性,那只是看到问题的一部分,而且不是最重要的部分。”越来越多的临床观察表明,慢性痛患者所遭受的恶性情绪,如焦虑、恐惧、孤独、甚至厌世等给病人造成的心身伤害远比疼痛本身更为严重。因此,对痛情绪和相关记忆形成机制的研究近年来逐步受到人们的关注。为进一步加深对疼痛本质的全面理解,本文简要综述了痛情绪和痛记忆的相关研究在神经解剖学、神经生理学和生物化学等方面的进展。

关键词： POSITRON-EMISSION-TOMOGRAPHY   CEREBRAL-BLOOD-FLOW   EVENT-RELATED FMRI   WORD FORM AREA   TRANSCRANIAL MAGNETIC STIMULATION   INFERIOR PREFRONTAL CORTEX   HUMAN EXTRASTRIATE CORTEX   NEAR-INFRARED SPECTROSCOPY   CROSS-MODAL PLASTICITY   PRIMARY VISUAL-CORTEX  

摘要： Functional neuroimaging methods have reached maturity. It is now possible to start to build the foundations of a physiology of language. The remarkable number of neuroimaging studies performed so far illustrates the potential of this approach, which complements the classical knowledge accumulated on aphasia. Here we attempt to characterize the impact of the functional neuroimaging revolution on our understanding of language. Although today considered as neuroimaging techniques, we refer less to electroencephalography and magnetoencephalography studies than to positron emission tomography and functional magnetic resonance imaging studies, which deal more directly with the question of localization and functional neuroanatomy. This review is structured in three parts. 1) Because of their rapid evolution, we address technical and methodological issues to provide an overview of current procedures and sketch out future perspectives. 2) We review a set of significant results acquired in normal adults ( the core of functional imaging studies) to provide an overview of language mechanisms in the "standard" brain. Single-word processing is considered in relation to input modalities ( visual and auditory input), output modalities ( speech and written output), and the involvement of "central" semantic processes before sentence processing and nonstandard language ( illiteracy, multilingualism, and sensory deficits) are addressed. 3) We address the influence of plasticity on physiological functions in relation to its main contexts of appearance, i.e., development and brain lesions, to show how functional imaging can allow fine-grained approaches to adaptation, the fundamental property of the brain. In closing, we consider future developments for language research using functional imaging.

关键词： chemical   biological   weapons of mass destruction   clinical neurophysiology   PUBLIC-HEALTH MANAGEMENT   BIOLOGICAL WEAPON   BOTULISM   NERVE   ANTHRAX   ELECTROMYOGRAPHY   DIAGNOSIS   CYANIDE   ACETYLCHOLINESTERASE   MANIFESTATIONS  

摘要： Chemical and biological agents have been used as weapons of mass destruction for a long time and presents as a serious threat to mankind. They have been used in many great wars and terrorist attacks with devastating results. The knowledge about these weapons of mass destruction is crucial to health care providers. Early recognition of the clinical characteristics of poisoning as a result of these chemical and biological agents is important to initiate appropriate therapy and minimizing casualties. Neurophysiological investigations when integrated with clinical features are helpful in early identification of some of these agents, especially when serological confirmation is not rapidly available. In this review, we have focused on chemical and biological weapons, which affect the nervous system and the role of clinical neurophysiology in such conditions.

关键词： PEDIATRIC ONCOLOGY   BRAIN   PAIN  

摘要： Three papers of special interest to researchers and clinicians alike have recently appeared in the general scientific and medical literatures. Two of these papers are original research studies that employ brain-imaging technologies, one using Magnetic Resonance Imaging (MRI), the other position emission tomography (PET). A third paper is a comprehensive review of the empirical findings on the clinical use of hypnosis in pediatric oncology. The research study using MRI technology is extraordinary, because it is the first to document differences in brain morphology between high hypnotizable and low hypnotizable individuals. Arguably, if its findings replicate, the study could be one of the most important developments in scientific hypnosis since the genesis of the Stanford scales 45 years ago. The PET study notes differences in brain activation during intentionally simulated and hypnotically experienced paralysis. The review article examines empirical work addressing the efficacy of hypnosis for procedural pain in pediatric oncology.

关键词： 神经生理学  

ISBN: (纸本)781067658X

摘要： 本书首先介绍了常用动物实验方法和神经病学研究常用动物模型的制备，然后分别介绍了组织病理学、生物化学、分子神经生物学等方面的有关技术方法，并对细胞培养、神经干细胞、流式细胞术、电子显微镜术等作了介绍。

关键词： 神经生理学 英文 中文  

ISBN: (纸本)781071757X

摘要： 本书由神经系统基本构成、神经细胞基本活动、神经系统主要功能和特殊感官感觉活动等四篇内容组成, 附有参考文献、附图和词汇表。