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Is hyperexcitability really guilty in amyotrophic lateral sclerosis?

Neural Regeneration Research 2015年第9期10卷 1413-1415页

作者： Felix Leroy Daniel Zytnicki Centre de Neurophysique Physiologie et Pathologie UMR 8119 Université Paris Descartes UMR 8119 45 rue des Saints-Pères 752070 Paris Cedex 06 France

Amyotrophic lateral sclerosis （ALS） is a lethal disorder char- acterized by the gradual degeneration of brainstem and spinal motoneurons as well as lateral cortico-spinal tracts. The onset generally occurs during the adult age except for some juvenile aggressive forms. Until recently, the vast majority of the cases （90%） were deemed sporadic. Mutations in the SOD1 gene have been for a long time the only ones reported in familiar forms of ALS. However, the recent implication of new genes of little known function cast a new view on this disease. C9ORF72, for example, is now recognized to account for 30% of the familial cases. Overall, ALS has been linked to 20 different genes, many also associated with other degenerative diseases （frontotemporal dementia, Alzheimer or ataxia）. Some of these genes are in- volved in RNA maturation （FUS, TARBD）. Clinical observation of human patients and mouse models suggests that all motor pools and motoneurons are not equally affected （see Kanning et al., 2010）. The disease usually starts in motor pools controlling the limbs or in the bulbar area before expanding to other motor pools, with the exception of a few resistant ones （Onuf＇s and oculomotor nuclei）. Within a vulnerable motor pool, motoneu- tons subtypes also exhibit differential vulnerability and follow an orderly degeneration; starting with the motoneurons innervating fast-contracting fatigable motor units （IZF motoneurons） and followed by the ones innervating fast-contractile fatigue-resistant motor units （FR motoneurons）. The motoneurons in- nervating slow motor units （S motoneurons） appear resistant to the disease. Although the mechanisms leading to the orderly degeneration are not known, many hypotheses have been raised （Ilieva et al., 2009）.

关键词： hyperexcitability amyotrophic sclerosis

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Oxaliplatin induced hyperexcitability syndrome in patient with appendiceal adenocarcinoma

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Case Reports in Clinical Medicine 2013年第4期2卷 236-238页

作者： Nicola Jabbour Maher Alchreiki Mohammad Hussain Harlan ARH Hospital Harlan USA Hazard ARH Cancer Center Hazard USA Johnson Medical Center Smithfield USA

Oxaliplatin is a novel platinum compound with clinical activity against several solid tumors. It has been associated with sensory neuropathies and, rarely, a neuromyotonia-like hyperexcitability syndrome;a form of peripheral neuropathy manifested by tremor and twitching activity of muscles. We present a case of hyperexcitability syndrome developed during the treatment of stage III appendiceal adenocarcinoma with oxaliplatin-containing regimen and was successfully treated with Pregabalin.

关键词： Oxaliplatin Peripheral Neuropathy Neuromyotonia hyperexcitability Syndrome Appendiceal Adenocarcinoma Sodium Channels

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Inflammatory Changes in Paravertebral Sympathetic Ganglia in Two Rat Pain Models

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Neuroscience Bulletin 2018年第1期34卷 85-97页

作者： Ai-Ling Li Jing-Dong Zhang Wenrui Xie Judith A. Strong Jun-Ming Zhang Department of Anesthesiology University of Cincinnati College of Medicine Cincinnati OH 45267 USA Department of Psychological and Brain Science Indiana University Bloomington 702 N Walnut Grove Ave. Bloomington IN 47405 USA

Injury to peripheral nerves can lead to neuropathic pain, along with well-studied effects on sensory neurons, including hyperexcitability, abnormal spontaneous activity, and neuroinflammation in the sensory ganglia. Neuropathic pain can be enhanced by sympathetic activity. Peripheral nerve injury may also damage sympathetic axons or expose them to an inflammatory environment. In this study, we examined the lumbar sympathetic ganglion responses to two rat pain models： ligation of the L5 spinal nerve, and local inflammation of the L5 dorsal root ganglion （DRG）, which does not involve axotomy. Both models resulted in neuroinflammatory changes in the sympathetic ganglia, as indicated by macrophage responses, satellite glia activation, and increased numbers of T cells, along with very modest increases in sympathetic neuron excitability （but not spontaneous activity） measured in ex vivo recordings. The spinal nerve ligation model generally caused larger responses than DRG inflammation. Plasticity of the sympathetic system should be recognized in studies of sympathetic effects on pain.

关键词： Neuropathic pain Sympathetic Macrophage T cell Satellite glia Inflammation hyperexcitability

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The interplay between metabolichomeostasis and neurodegeneration:insights into the neurometabolic nature ofamyotrophic lateral sclerosis

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Cell Regeneration 2015年第1期4卷 47-60页

作者： S.T.Ngo F.J.Steyn Queensland Brain Institute The University of QueenslandSt LuciaBrisbane 4072Australia School of Biomedical Sciences The University of QueenslandSt LuciaBrisbane 4072Australia Department of Neurology Royal Brisbane and Women’s HospitalHerstonBrisbane 4006Australia University of Queensland Centre for Clinical Research The University of QueenslandHerstonBrisbane 4029Australia

Amyotrophic lateral sclerosis (ALS) is a fatal, neurodegenerative disease that is characterized by the selectivedegeneration of upper motor neurons and lower spinal motor neurons, resulting in the progressive paralysis of allvoluntary muscles. Approximately 10 % of ALS cases are linked to known genetic mutations, with the remaining 90 %of cases being sporadic. While the primary pathology in ALS is the selective death of upper and lower motor neurons,numerous studies indicate that an imbalance in whole body and/or cellular metabolism influences the rate ofprogression of disease. This review summarizes current research surrounding the impact of impaired metabolicphysiology in ALS. We extend ideas to consider prospects that lie ahead in terms of how metabolic alterations mayimpact the selective degeneration of neurons in ALS and how targeting of adenosine triphosphate-sensitive potassium(K_(ATP)) channels may represent a promising approach for obtaining neuroprotection in ALS.

关键词： Amyotrophic lateral sclerosis Metabolism Neurometabolism hyperexcitability Ion channels K_(ATP) channels

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BmK I directly activates Nav1.8 in primary sensory neurons to induce neuronal hyperexcitability in rats

BmK I directly activates Nav1.8 in primary sensory neurons t...

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第12届生物毒素研究及医药应用学术大会

作者： Pin Ye Li-Ming Hua Yong-Hua Ji Laboratory of Neuropharmacology and Neurotoxicology Shanghai University

Voltage-gated sodium channels(VGSCs) in primary sensory neurons play a key role in transmitting pain signals to the central nervous system.BmK I,a site-3 sodium channel-specific toxin from scorpion Buthus martensi Karsch,induces pain behaviors in rats.However,the subtypes of VGSCs targeted by BmK I were not entirely clear.We therefore investigated the effect of BmK I on the current and kinetic properties of Na1.8,which is associated with neuronal hyperexcitability in DRG neurons.It was found that BmK I dose-dependently increase Na1.8 current in small-sized(<25μm) acute dissociated DRG neurons,which correlated with its inhibition on both fast and slow inactivation.Moreover,voltage-dependent activation and steady-state inactivation curves of Na1.8were shifted in a hyperpolarized direction.Thus,BmK I reduced the threshold of neuronal excitability and increased action potential firing in DRG neurons.In conclusion,the data fruited in the study clearly demonstrated that BmK I modulated Na1.8remarkably,suggesting BmK I as a valuable probe for studying Na1.8,and Na1.8 is an important target related to BmK I-evoked pain.

关键词： BmK I DRG neuron Nav1.8 hyperexcitability

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Transcraneal Magnetic Stimulation Improves Sleep Parameters in Patients Affected with Imsomnia Associated to Electroencephalographic Abnormalities

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Neuroscience & Medicine 2014年第1期5卷 72-77页

作者： Oscar Sánchez-Escandón Yoaly Arana-Lechuga Guadalupe Terán-Pérez ángel Ruiz-Chow Enrique Esqueda-Leon Rosa Obdulia González-Robles Paul Shkurovich-Bialik Miguel ángel Collado-Corona Javier Velázquez-Moctezuma American British Cowdray Medical Center Neurological Center Santa Fe Campus Ciudad de México México Mathematics Department Universidad Autónoma Metropolitana-Iztapalapa Ciudad de México México Psychiatry Department Hospital General Naval de Alta Especialidad Ciudad de México México Sleep Disorders Clinic Universidad Autónoma Metropolitana-Iztapalapa Ciudad de México México

Insomnia is one of the most frequently observed sleep disorders, affecting nearly 10% of the general population. It has multiple etiological factors. Recently, it has been reported that EEG abnormalities are associated with insomnia in patients previously diagnosed as idiopathic insomniacs. In addition, transcranial magnetic stimulation (TMS) has shown to be effective in the treatment of disorders characterized by neural hyper-excitability. Method: In the present study, patients with insomnia and EEG abnormalities were submitted to slow repetitive trans-cranial magnetic stimulation, for 15 minutes daily during 10 days. Polysomnographic recordings were performed before and after TMS. Results: The results indicate that the presence of EEG abnormalities significantly decreased after TMS. In addition, most of the sleep parameters showed significant improvement. Conclusions: These data support the notion that TMS is a reliable therapeutic tool for patients affected with abnormalities linked to neuronal hyper-excitability.

关键词： Insomnia Transcraneal Magnetic Stimulation EEG Abnormalities hyperexcitability Sleep Architecture Polysomnographic

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Case Report: A 72 Years Old Man with Isaacs’ Syndrome: A Rare Entity with Different Outcomes

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World Journal of Neuroscience 2016年第2期6卷 82-84页

作者： Akam A. Saeed Zana A. Mohammed Rebeen R. Saeed Shar Teaching Hospital of Sulaimania Sulaimania Iraq General University Teaching Hospital of Sulaimania Sulaimania Iraq School of Medicine University of Sulaimania Sulaimania Iraq

Neuromyotonia is a neuromuscular hyperexitability disorder characterized by muscle stiffness caused by continuous muscle fiber activity. It is an immune mediated disorder with elevated antibody level against presynaptic, voltage gated potassium channels, either as isolation or as a paraneoplastic process. Symptoms usually include muscle twitching during rest (myokymia), cramps, peudomyotonia (delayed relaxation), increased sweating, and sometimes motor weakness. In this case report, we present a seventy-two years old man who presented with pain in both thighs for one month. It gradually became worse to involve feet and chest. His brain CT scan showed features of brain atrophy. EMG showed fasciculation along neuromyotonic discharges with characteristic wave in frequency and amplitude typical of Isaacs’ syndrome. Potassium channel antibodies were very high. Diagnosis of Isaacs’ syndrome was made. He was followed up for two months with treatment by three-day course of methyl prednisolone followed by oral steroid and methotrexate with much improvement. This is the first case of Isaacs’ syndrome in Kurdistan.

关键词： Issacs Syndrome Myokymia Neuromyotonia Neurology Motor Disorders hyperexcitability

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Prediction of post-progression survival in patientswith advanced hepatocellular carcinoma treated with sorafenib by using time-dependent changes inclinical characteristics

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Hepatoma Research 2018年第3期4卷 1-8页

作者： Yoshiyuki Wada Yuko Takami Hajime Matsushima Masaki Tateishi Tomoki Ryu Munehiro Yoshitomi Hideki Saitsu Department of Hepato-Biliary-Pancreatic Surgery Clinical Research InstituteNational Hospital Organization Kyushu Medical CenterFukuoka 810-8563Japan

Aim: Sorafenib has been shown to improve time to tumor progression (TTP) and overall survival (OS) in patients with hepatocellular carcinoma (HCC);however, post-progression survival (PPS) has not been well characterized in these patients. This study aimed to evaluate the predictors of PPS by using time-dependent and dynamic changes in radiologic progression patterns, liver function, and performance status (PS) in patients with advanced HCC receiving sorafenib treatment. Methods: We retrospectively analyzed the clinical characteristics of 128 advanced HCC patients with Child-Pugh scores ≤ 7 at the initiation of sorafenib treatment. Results: The median TTP, OS, and PPS were 3.8, 15.6, and 9.9 months, respectively. At the time of confirmation of radiologic progressive disease (PD), a total of 46 (35.6%) patients showed impairments in their PS of ≥ +1 points over time. For the Child-Pugh score, 27 (21.1%) and 26 (10.9%) patients exhibited an impairment of ≥ +1 and ≥ +2 points, respectively. Multivariate analysis identified the following independent predictors of PPS: impairment in the PS score of ≥+1 point [hazard ratio (HR) 1.81, 95% confidence interval (CI) 1.16-2.82], impairment in the Child-Pugh score of ≥ +2 points (HR 3.70, 95% CI 1.68-8.15), radiologic pattern of progression (target lesion growth and emergence of a new lesion) (HR 2.91, 95% CI 1.79-2.91), a TTP < 4 months(HR 1.87, 95% CI 1.21-2.91), second-line treatment after radiologic confirmation of PD (HR 0.16, 95% CI 0.08-0.32), and continuous sorafenib treatment after radiologic confirmation of PD (HR 1.76, 95% CI 1.06-3.00). Conclusion: PPS in patients with advanced HCC can be characterized by using time-dependent dynamic changes in clinical parameters.

关键词： Contactin-associated protein-2 Isaac neuromuscular hyperexcitability neuromyotonia voltage-gated potassium channel

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Steroids and plasma exchange in Isaacs' syndromewith anti-Caspr2 antibodies

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Neuroimmunology and Neuroinflammation 2018年第3期5卷 5-9页

作者： Daniele Orsucci Gianfranco Cafforio Gianluca Moscato Alessandro Napolitano Marco Margelli Monica Mazzoni Unit of Immunohematology and Transfusional Medicine San Luca HospitalLucca55100Italy

Isaacs' syndrome is a disease characterized by nerve hyperexcitability. The patients are commonly treated with symptomatic therapies and immunomodulatory approaches, but no clinical trials are available to date. Here, we report the case of an anti-Caspr2-positive patient, presenting with continuous muscle twitches and diffuse muscle pain. He experienced a nearly complete clinical response to intravenous high-dose steroids combined with plasma exchange, sustained for at least 1 year. Our experience suggests that methylprednisolone 1000 mg/day × 5 days and consecutive tapering followed by plasma exchange may be efficient and well tolerated in patient with Isaacs' syndrome due to anti-Caspr2 antibodies.

关键词： Contactin-associated protein-2 Isaac neuromuscular hyperexcitability neuromyotonia voltage-gated potassium channel

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Cholinergic Grb2-associated-binding protein 1 regulates cognitive function

Cholinergic Grb2-associated-binding protein 1 regulates cogn...

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中国神经科学学会第十二届全国学术会议

作者： Nan-Nan Lu Chao Tan Ying-Mei Lu Feng Han Institute of Pharmacology and Toxicology College of Pharmaceutical SciencesZhejiang University School of Medicine Zhejiang University City College

Objective Grb2-associated-binding protein 1（Gab1） is a docking/scaffolding molecule known to play an essential role in cell growth and survival signaling pathway. However, the precise role of Gab1 in Central Neuron System（CNS） is much less known. The aim of present study is to explore and uncover the expression pattern of Gab1 in CNS as well as its role in cognitive function. Methods The study was designed and performed by taking advantages of Gab1 conditional knockout mice（Ch AT-Cre;Gab1 f/f） and a mouse model of AD（APPswe/PS1）, combined with immunofluorescence imaging, electrophysiological recording, gene manipulation by virus system and a series of cognition behavior test. Results Gab1 is selectively expressed in central cholinergic neurons（especially 93.3% ± 3.1% in medial septum） and is downregulated in human Alzheimer’s disease（AD） patients and in a mouse model of AD. In mice, selective ablation of Gab1 in cholinergic neurons（Ch AT-Cre;Gab1 f/f） in the medial septum impaired learning and memory and hippocampal long-term potentiation（LTP） in compared with Gab1 f/f mice. Gab1 ablation also inhibited SK channels, leading to an increase in firing in septal cholinergic neurons. By applying a flip-excision（Flex） switch-based AAV system, selectively reexpression Gab1 in cholinergic neurons, significantly improved cognitive function of Ch AT-Cre;Gab1 f/f mice in Morris water maze, novel object recognition test and Y-Maze test. More importantly, Gab1 overexpression in medial septum sufficiently ameliorated cognitive defects and restored hippocampal Ca MKII autophosphorylation in AD mice. Conclusion Together, the above findings suggest that Gab1 plays a critical role in the pathophysiology of AD and may represent a novel therapeutic target for diseases involving cholinergic dysfunction.

关键词： Cholinergic neurons Cognitive dysfunction Gab1 hyperexcitability SeptoHippocampal System

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