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      • KCI등재

        Sensory and motor axons are different: implications for neurological disease

        David Burke,James Howells,Matthew C. Kiernan 대한임상신경생리학회 2017 Annals of Clinical Neurophysiology Vol.19 No.1

        Using threshold tracking, differences have been established between large myelinated sensory and α motor axons in humans. Major differences are that sensory axons are relatively depolarised at rest such that they have a greater persistent Na+ current, and have greater activity of hyperpolarisation-activated cyclic nucleotide-gated (HCN) channels. Sensory axons may thereby be protected from hyperpolarising stresses, and are less likely to develop conduction block. However, the corollary is that sensory axons are more excitable and more likely to become ectopically active.

      • KCI등재

        Sensory and motor axons are different: implications for neurological disease

        Burke, David,Howells, James,Kiernan, Matthew C. The Korean Society of Clinical Neurophysiology 2017 Annals of Clinical Neurophysiology Vol.19 No.1

        Using threshold tracking, differences have been established between large myelinated sensory and ${\alpha}$ motor axons in humans. Major differences are that sensory axons are relatively depolarised at rest such that they have a greater persistent $Na^+$ current, and have greater activity of hyperpolarisation-activated cyclic nucleotide-gated (HCN) channels. Sensory axons may thereby be protected from hyperpolarising stresses, and are less likely to develop conduction block. However, the corollary is that sensory axons are more excitable and more likely to become ectopically active.

      • KCI등재

        The Puzzling Case of Hyperexcitability in Amyotrophic Lateral Sclerosis

        배종석,Neil G. Simon,Parvathi Menon,Steve Vucic,Matthew C. Kiernan 대한신경과학회 2013 Journal of Clinical Neurology Vol.9 No.2

        The development of hyperexcitability in amyotrophic lateral sclerosis (ALS) is a well-known phenomenon. Despite controversy as to the underlying mechanisms, cortical hyperexcitability appears to be closely related to the interplay between excitatory corticomotoneurons and inhibitory interneurons. Hyperexcitability is not a static phenomenon but rather shows a pattern of progression in a spatiotemporal aspect. Cortical hyperexcitability may serve as a trigger to the development of anterior horn cell degeneration through a ‘dying forward’process. Hyperexcitability appears to develop during the early disease stages and gradually disappears in the advanced stages of the disease, linked to the destruction of corticomotorneuronal pathways. As such, a more precise interpretation of these unique processes may provide new insight regarding the pathophysiology of ALS and its clinical features. Recently developed technologies such as threshold tracking transcranial magnetic stimulation and automated nerve excitability tests have provided some clues about underlying pathophysiological processes linked to hyperexcitability. Additionally, these novel techniques have enabled clinicians to use the specific finding of hyperexcitability as a useful diagnostic biomarker, enabling clarification of various ALS-mimic syndromes, and the prediction of disease development in pre-symptomatic carriers of familial ALS. In terms of nerve excitability tests for peripheral nerves, an increase in persistent Na+ conductances has been identified as a major determinant of peripheral hyperexcitability in ALS, inversely correlated with the survival in ALS. As such, the present Review will focus primarily on the puzzling theory of hyperexcitability in ALS and summarize clinical and pathophysiological implications for current and future ALS research.

      • KCI등재

        Dissociation of Structural and Functional Integrities of the Motor System in Amyotrophic Lateral Sclerosis and Behavioral-Variant Frontotemporal Dementia

        배종석,Michele Ferguson,Rachel Tan,Eneida Mioshi,Neil Simon,James Burrell,Steve Vucic,John R. Hodges,Matthew C Kiernan,Michael Hornberger 대한신경과학회 2016 Journal of Clinical Neurology Vol.12 No.2

        Background and Purpose Tis study investigated the structural and functional changes in the motor system in amyotrophic lateral sclerosis (ALS; n=25) and behavioral-variant frontotemporal dementia (bvFTD; n=17) relative to healthy controls (n=37). Methods Structural changes were examined using a region-of-interest approach, applying voxel-based morphometry for gray-matter changes and difusion tensor imaging for white-matter changes. Functional changes in the motor system were elucidated using threshold-tracking transcranial magnetic stimulation (TMS) measurements of upper motor-neuron excitability. Results The structural analyses showed that in ALS there were more white-matter changes in the corticospinal and motor-cortex regions and more gray-matter changes in the cerebellum in comparison to controls. bvFTD showed substantial gray- and white-matter changes across virtually all motor-system regions compared to controls, although the brainstem was afected less than the other regions. Direct comparisons across patient groups showed that the gray- and white-matter motor-system changes inclusive of the motor cortex were greater in bvFTD than in ALS. By contrast, the functional integrity of the motor system was more adversely afected in ALS than in bvFTD, with both patient groups showing increased excitability of upper motor neurons compared to controls. Conclusions Cross-correlation of structural and functional data further revealed a neural dissociation of diferent motor-system regions and tracts covarying with the TMS excitability across both patient groups. Te structural and functional motor-system integrities appear to be dissociated between ALS and bvFTD, which represents useful information for the diagnosis of motor-system changes in these two disorders.

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