• 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.

• Annals of Rehabilitation Medicine
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• v.42 no.6
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• pp.833-837
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• 2018

Objective To investigate and demonstrate persistent increase of peak cough flow after mechanical in-exsufflator application, in patients with neuromuscular diseases and pneumonia. Methods A mechanical in-exsufflator was applied with patients in an upright or semi-upright sitting position (pressure setting, +40 and $-40cmH_2O$; in-exsufflation times, 2-3 and 1-2 seconds, respectively). Patients underwent five cycles, with 20-30 second intervals to prevent hyperventilation. Peak cough flow without and with assistive maneuvers, was evaluated before, and 15 and 45 minutes after mechanical in-exsufflator application. Results Peak cough flow was 92.6 L/min at baseline, and 100.4 and 100.7 L/min at 15 and 45 minutes after mechanical in-exsufflator application, respectively. Assisted peak cough flow at baseline, 15 minutes, and 45 minutes after mechanical in-exsufflator application was 170.7, 179.3, and 184.1 L/min, respectively. While peak cough flow and assisted peak cough flow increased significantly at 15 minutes after mechanical in-exsufflator application compared with baseline (p=0.030 and p=0.016), no statistical difference was observed between 15 and 45 minutes. Conclusion Increased peak cough flow after mechanical in-exsufflator application persists for at least 45 minutes.

• 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.

• Annals of Clinical Neurophysiology
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• v.22 no.2
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• pp.51-60
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• 2020

Muscle pathology findings may guide the diagnosis of neuromuscular disorders since they are helpful for understanding the pathological processes causing muscle weakness and also provide significant clues for the diagnosis of muscle diseases. Recent advances in molecular genetics mean that a muscle biopsy can be omitted when diagnosing inherited muscle diseases. However, the muscle pathology can still play a role in those cases and its findings are also required when diagnosing inflammatory myopathies.

• Clinical and Experimental Pediatrics
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• v.51 no.12
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• pp.1295-1299
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• 2008

Inherited muscle diseases are heterogeneous with varying genetic etiologies and present with common symptoms and signs, including weakness, motor developmental delay, and hypotonia. To diagnose these various diseases, a meticulous family and clinical history, physical and neurological examinations, laboratory findings with electromyography, muscle biopsy, and genetic testing are needed. Here, I review several inherited muscle diseases, with a focus on muscular dystrophy in children and its genetics and general management.

• 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.

• PNF and Movement
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• v.12 no.2
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• pp.51-61
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• 2014

Purpose: This study aimed to review articles related to PNF and easily provide information to readers who have difficulty reading papers or using PNF in clinics without a background in statistical knowledge. Methods: We used PubMed to collect papers on international studies and we conducted a search on April 28, 2014. Keywords used for the PubMed search were "PNF" and "Proprioceptive Neuromuscular Facilitation". The search results identified 765 papers that had been published since 1954. To target the papers for review, we selected 66 papers, excluding papers that were not published in online journals and papers that were written in a second language. Results: The reviewed papers consisted of studies that addressed basic principles and procedures, combined with other interventions, including PNF stretching concepts, and other diseases with PNF concepts. The articles related to basic procedures and principles reported on optimal contraction intensity, patterns, and irradiation. The articles related to stretching and strengthening reported on stretching the hamstring muscles in healthy subjects, in combination with other interventions such as neuromobilization, clinical massage, kinesio taping, and water immersion. The articles related to specific diseases reported on stroke, low back pain, athletic injury, total knee replacement, etc. Conclusion: In the 66 articles reviewed for this study, there was little evidence that PNF has therapeutic value. However, in future research on the application of PNF, investigators could conduct a more in-depth analysis of this topic and present more objective and useful information on the therapeutic benefits of PNF.

• Physical Therapy Korea
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• v.25 no.3
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• pp.60-67
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• 2018

Background: Progressive muscle weakness is aggravated not only in the skeletal muscles but also in the respiratory muscles in many patients with neuromuscular diseases (NMD). Inspiratory muscle training (IMT) has been reported as therapy for pulmonary rehabilitation to improve respiratory strength, endurance, exercise capacity, and quality of life, and to reduce dyspnea. Objects: The purpose of this study was to determine the effect of playing harmonica for 5 months on pulmonary function by assessing the force vital capacity (FVC), peak cough flow (PCF), maximal inspiratory pressure (MIP), maximal expiratory pressure (MEP), and maximal voluntary ventilation (MVV) in patients with NMD. Methods: Six subjects with NMD participated in this study. The subjects played harmonica once a week for 2 hours at a harmonica academy and twice a week for 1 hour at home. Thus, training was performed thrice a week for 23 weeks. The examiner assessed pulmonary function by measuring FVC in the sitting and supine positions and PCF, MIP, MEP, and MVV in the sitting position at the beginning of training and once a month for 5 months. Results: Both sitting and supine FVC significantly increased after playing harmonica (p=.042), as did MIP (p=.043) and MEP (p=.042). Conclusion: Playing harmonica can be used as an effective method to improve pulmonary function in patients with NMD.

• 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.

• Journal of Korean Physical Therapy Science
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• v.5 no.3
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• pp.617-624
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• 1998

A variety of neuromuscular diseases in children cause muscle imbalance. impaired function, and resultant deformity of the lower extremities. Equinovarus, equinos, adductor contracture are common deformity of lower extremities in the children. Generally, the transfer was successful in improving or maintaining range of motion, stability of the joint and gait. Postoperative physical therapy is important. Physical therapy was instituted after cast removal. All children received stretching exercise, range of motion exercise for the joint, proprioceptive neuromuscular fascilitation and gait training was administered. This article describes our experience with the physical therapy for tendon transfered spastic cerebral palsy and discusses the therapeutic protocol.