• Title/Summary/Keyword: Neuromuscular disorders

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Critical Illness Myopathy (위험질병 근병증)

  • Lee, Dong Kuck
    • Annals of Clinical Neurophysiology
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    • v.4 no.2
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    • pp.91-97
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    • 2002
  • The field of critical care medicine has flourished, but an unfortunate result of improved patient survival in the intensive care unit is the occurrence of certain acquired neuromuscular disorders. During the last two decades, various neuromuscular disorders were recognized as common causes of weakness occurring in critically ill patients. The two most common disorders are an acute quadriplegic myopathy predominantly associated with the use of intravenous corticosteroids and neuromuscular junction blocking agents and severe systemic illness termed critical illness myopathy(CIM), and an axonal sensorimotor polyneuropathy termed critical illness polyneuropathy. I will review briefly about general components of the CIM.

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Integrated diagnostic approach of pediatric neuromuscular disorders

  • Lee, Ha Neul;Lee, Young-Mock
    • Journal of Genetic Medicine
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    • v.15 no.2
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    • pp.55-63
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    • 2018
  • Clinical and genetic heterogeneity in association with overlapping spectrum is characteristic in pediatric neuromuscular disorders, which makes confirmative diagnosis difficult and time consuming. Considering evolution of molecular genetic diagnosis and resultant upcoming genetically modifiable therapeutic options, rapid and cost-effective genetic testing should be applied in conjunction with existing diagnostic methods of clinical examinations, laboratory tests, electrophysiologic studies and pathologic studies. Earlier correct diagnosis would enable better clinical management for these patients in addition to new genetic drug options and genetic counseling.

Adverse Events Associated with Intravenous Immunoglobulin Therapy in Neuromuscular Disorders (신경근질환의 정맥 내 면역글로불린 치료와 연관된 유해사례)

  • Na, Sang-Jun;Choi, Young-Chul
    • Annals of Clinical Neurophysiology
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    • v.8 no.1
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    • pp.48-52
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    • 2006
  • Background: Intravenous immunoglobulin (IVIg) has been administered for various immune-mediated neurological diseases such as autoimmune neuropathy, inflammatory myopathies, and other autoimmune neuromuscular disorders. The purpose of this study is to investigate side effects and complications of IVIg therapy in neuromuscular disorders. Methods: We enrolled 29 patients (age 8~63 years) with IVIg therapy for various neurological diseases including Guillain-Barre syndrome, myasthenia gravis, dermatomyositis, polymyositis, chronic inflammatory demyelinating polyneuropathy, and multifocal motor neuropathy. IVIg therapy was used at a dose of 0.4 g/kg body weight/day for 5 consecutive days. Results: 10 patients (34%) had adverse events. There are adverse events in 16 courses (11%) among total 145 courses. The majority of patients presented with mild side effects, mostly asymptomatic laboratory changes. Rash or mild headache occurred in 3 patients. One patient showed a serious side effect of deep vein thrombosis. Conclusions: IVIg therapy is safe for a variety of immune-mediated neurological diseases in our study.

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The Effects of Kinesio Taping and Neuromuscular Rehabilitation Exercise for Patients with Acute Whiplash-Associated Disorder (급성 편타성-관련손상 환자에게 키네지오 테이핑과 신경근 재활 운동이 미치는 영향)

  • Lee, Yun-sang;Ahn, Seung-won
    • The Journal of Korean Academy of Orthopedic Manual Physical Therapy
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    • v.22 no.2
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    • pp.41-49
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    • 2016
  • Background: The purpose of this study was to investigate the effects of kinesio taping and neuromuscular rehabilitation exercise for patients with acute whiplash-associated disorders. Methods: Twenty acute whiplash-associated disorders patients were recruited for the study. Subjects were randomly allocated into two groups. Neuromuscular rehabilitation group (NRG) received neuromuscular rehabilitation exercise, kinesio taping group (KTG) received kinesio taping. All subject was evaluated before and after intervention by their range of motion (ROM), neck disability index (NDI), numeric pain rating scale (NPRS) and fatigability. Results: First, the ROM was significantly increased in all group (p<.05). The NDI, NPRS and fatigability were significantly decreased in all group (p<.05). Secondly, NRG had significantly more increased ROM (flexion, both rotation) than KTG (p<.05). NRG had significantly more decreased NDI, NPRS and fatigability than KTG (p<.05). Conclusion: According to the results above, increased in range of motion in the neck, decreased in NDI, NPRS, fatigability were more effective in the NRG that received neuromuscular rehabilitation exercise than the KTG that received kinesio taping.

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Nerve conduction studies: basic principal and clinical usefulness

  • Ahn, Suk-Won;Yoon, Byung-Nam;Kim, Jee-Eun;Seok, Jin Myoung;Kim, Kwang-Kuk;Lim, Young Min;Kwon, Ki-Han;Park, Kee Duk;Suh, Bum Chun;Korean Society of Clinical Neurophysiology Education Committee
    • Annals of Clinical Neurophysiology
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    • v.20 no.2
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    • pp.71-78
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    • 2018
  • Nerve conduction study (NCS) is an electrophysiological tool to assess the overall function of cranial and peripheral nervous system, therefore NCS has been diagnostically helpful in the identification and characterization of disorders involving nerve roots, peripheral nerves, muscle and neuromuscular junction, and are frequently accompanied by a needle Electromyography. Furthermore, NCS could provide valuable quantitative and qualitative results into neuromuscular function. Usually, motor, sensory, or mixed nerve studies can be performed with using NCS, stimulating the nerves with the recording electrodes placed over a distal muscle, a cutaneous sensory nerve, or the entire mixed nerve, respectively. And these findings of motor, sensory, and mixed nerve studies often show different and distinct patterns of specific abnormalities indicating the neuromuscular disorders. The purpose of this special article is to review the neurophysiologic usefulness of NCS, to outline the technical factors associated with the performance of NCS, and to demonstrate characteristic NCS changes in the setting of various neuromuscular conditions.

Movement Disorders Affecting the Larynx (후두운동장애)

  • Kwon, Tack-Kyun;Son, Hee-Young
    • Journal of the Korean Society of Laryngology, Phoniatrics and Logopedics
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    • v.21 no.1
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    • pp.22-26
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    • 2010
  • Neuromuscular Disorders Affecting the Larynx are steadily important topics at laryngology. Physiology of larynx is controlled by the frame structure and neuromuscular dynamics to acting on the specialized soft tissue, Therefore, for a proper understanding of the larynx, it is needed the voice and swallowing, a series of prayers on the regulation of neurologic function and the correlation between systemic neuromuscular disease and laryngeal symptoms and clinical knowledge, We described that clinical findings and treatments of the 3 neurological diseases causing dysphonia well (Parkinson's disease, laryngeal tremor, spasmodic dysphonia) and vocal impairments for stoke patients.

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Comprehensive Relevance of AMPK in Adaptive Responses of Physical Exercise, Skeletal Muscle and Neuromuscular Disorders

  • Lee, Jun-Ho
    • Journal of the Korean Society of Physical Medicine
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    • v.13 no.3
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    • pp.141-150
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    • 2018
  • PURPOSE: This study was conducted to understand the adaptive responses of different modes of physical exercises utilizing skeletal muscle and the comprehensive relevance of AMPK signaling that can be activated by physical exercise as a potential molecular target in human health problems such as neuromuscular disorders (NMDs). METHODS: Most of the contents in this review article are based on recent publications concerning the main topics of interest. The reference literatures cited were obtained by basic searches of overseas academic databases such as PubMed and ScienceDirect using EndNote X7.8. RESULTS: The phenotypic adaptive responses of skeletal muscle during endurance- and resistance-based exercise training (ET and RT respectively) appear to be distinct. To explain the adaptive responses in each single mode of exercises (ET, RT) along with combined exercise training (CT), AMPK signaling is proposed as an important molecular link among those differential modes of exercise and a promising molecular target of NMDs. CONCLUSION: Based on the available evidence, intracellular AMPK signaling activated by diverse stimuli including physical exercise can be a potential and promising therapeutic target for the prevention, amelioration or cure of various human health problems including NMDs and may also be beneficial for physical rehabilitation and emergency situations that may elicit acute metabolic stresses.

Therapeutic Potential of CKD-504, a Novel Selective Histone Deacetylase 6 Inhibitor, in a Zebrafish Model of Neuromuscular Junction Disorders

  • Jeong, Hui Su;Kim, Hye Jin;Kim, Deok-Ho;Chung, Ki Wha;Choi, Byung-Ok;Lee, Ji Eun
    • Molecules and Cells
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    • v.45 no.4
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    • pp.231-242
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    • 2022
  • The neuromuscular junction (NMJ), which is a synapse for signal transmission from motor neurons to muscle cells, has emerged as an important region because of its association with several peripheral neuropathies. In particular, mutations in GARS that affect the formation of NMJ result in Charcot-Marie-Tooth disease and distal hereditary motor neuropathy. These disorders are mainly considered to be caused by neuronal axon abnormalities; however, no treatment is currently available. Therefore, in order to determine whether the NMJ could be targeted to treat neurodegenerative disorders, we investigated the NMJ recovery effect of HDAC6 inhibitors, which have been used in the treatment of several peripheral neuropathies. In the present study, we demonstrated that HDAC6 inhibition was sufficient to enhance movement by restoring NMJ impairments observed in a zebrafish disease model. We found that CKD-504, a novel HDAC6 inhibitor, was effective in repairing NMJ defects, suggesting that treatment of neurodegenerative diseases via NMJ targeting is possible.

Ultrasonographic evaluation of common compression neuropathies in the upper limb

  • Seok, Jung Im
    • Annals of Clinical Neurophysiology
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    • v.22 no.1
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    • pp.1-7
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    • 2020
  • Neuromuscular ultrasonography has emerged over the last decade as a useful tool for diagnosing peripheral nerve disorders. It has been studied extensively with a particular focus on the assessment of compression neuropathies. Neuromuscular ultrasonography complements electrodiagnostic studies well by visualizing both the nerve anatomy and surrounding structures, providing useful data that cannot be obtained using the latter methodology only. This review article summarizes and synthesizes the literature focusing on the diagnostic role of neuromuscular ultrasonography in common compression neuropathies of the upper limb.