• Title/Summary/Keyword: Neuromuscular disease

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Familial congenital myopathy with prominent decremental responses in repetitive nerve stimulation testing

  • Kim, Dayoung;Sunwoo, Il Nam;Oh, Jeeyoung
    • Annals of Clinical Neurophysiology
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    • v.23 no.1
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    • pp.53-55
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    • 2021
  • Congenital myasthenic syndromes (CMSs) are rare genetic disorders characterized by weakness and fatigue resulting from impaired neuromuscular transmission. Genetic testing can confirm the diagnosis for some types of CMS; however, variations in genotype, clinical phenotypes, age at disease onset, and responses to treatment make diagnosis very difficult. Here we present two adult patients who had significant decremental responses in repetitive nerve stimulation testing and multi-minicore pathology, and who responded to treatment with a cholinesterase inhibitor.

Effects of Combined Breathing Exercise and Neuromuscular Electrical Stimulation on Patients with Severe COPD (중증 만성폐쇄성폐질환 환자를 위한 복합호흡운동과 신경근전기자극의 효과)

  • Kang, Jeong-Il;Park, Jun-Su;Jeong, Dae-Keun
    • Journal of Korea Entertainment Industry Association
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    • v.13 no.7
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    • pp.539-548
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    • 2019
  • This study was designed to examine the effects of complex breathing exercise and neuromuscular electrical stimulation of Quadriceps Femoris muscle on pulmonary function and cerebral cortex activity in patients with severe chronic obstructive pulmonary disease. After collecting samples from 20 patients with severe chronic obstructive pulmonary disease aged 60 to 80, 10 patients each were randomly placed in an experimental group and a control group. The experimental group conducted complex breathing exercise and neuromuscular electrical stimulation of Quadriceps Femoris muscle, and the control group only conducted complex breathing exercise. As a pretest, pulmonary function and cerebral cortex activity were measured. The intervention program was applied to each group for 30 minutes, once a day, for 4 days a week, for 6 weeks, and the posttest was carried out the same way as the pretest. As a result, both groups showed significant differences in FEV1.0(Forced Expiratory Volume in One Second)(p<.001)(p<.05), and there were significant differences between the groups as well(p<.05). When comparing alpha waves in each domain of cerebral cortex, both of the experimental and control groups showed significant differences in Fp1, Fp2, F3 and F4 domains (p<.01)(p<.05). During the 6-week experiment, complex breathing exercise and neuromuscular electrical stimulation of Quadriceps Femoris muscle improved pulmonary function of patients with severe chronic obstructive pulmonary disease, and in relation to cerebral cortex activity, a positive breathing change was found due to the increase of alpha waves in the forehead domain. Therefore, it is considered that applying neuromuscular electrical stimulation of Quadriceps Femoris muscle to patients with severe chronic obstructive pulmonary disease additionally along with complex breathing exercise will bring a better therapeutic effect.

Current status and challenges in disease surveillance and epidemiological investigation systems for companion animals in South Korea

  • Beom Jun Lee;Kyung-Duk Min
    • Korean Journal of Veterinary Research
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    • v.64 no.2
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    • pp.18.1-18.5
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    • 2024
  • The surveillance and epidemiological investigation systems for companion animals in South Korea are significantly underdeveloped compared to those for humans and livestock. Recent outbreaks, such as idiopathic neuromuscular syndrome and highly pathogenic avian influenza among cats, have highlighted the need for reliable systems. This short review conducts situation analysis regarding disease surveillance and epidemiological investigation for companion animals in South Korea. The current challenges include an absence of administrative leadership, a lack of legal support, and unreliable medical data. The recommendations for future directions include clear leadership by the Animal and Plant Quarantine Agency, amending the Act on the Prevention of Contagious Animal Diseases to include companion animals, and enhancing the quality of medical data through standardized coding systems, such as Systematized Nomenclature of Medicine Clinical Terms. In addition, sentinel surveillance rather than universal systems should be established to provide adequate incentives for local practitioners to provide data and develop sustainable public-private networks. These recommendations could be important for developing a comprehensive and sustainable system for disease surveillance and epidemiological investigation in the companion animal field.

Surgical Management of Myasthenia Gravis (근무력증의 외과적 치료에 대한 임상적 고찰)

  • 김주현
    • Journal of Chest Surgery
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    • v.13 no.3
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    • pp.301-305
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    • 1980
  • Myasthenia gravis is a disorder that affects neuromuscular transmission in a way that is still poorly understood. some think that myasthenia gravis results from a reduction of available acetylcholine receptors in neuromuscular junctions, consequent to some form of autoimmune injury. Surgical interest in this disease was first aroused in 1939 when Blalock observed that some patients with thymic tumors and myasthenia gravis improved following thymectomy. This report represents two cases of myasthenia gravis. The 14-year-old girl was admitted to Korea Universtiy Hospital with chief complaintment of bilateral ptosis, diplopia, swallowing difficulty, and mastication difficulty, which were relieved by administration of edrophonium (Tensilon) chloride, given intravenously. Myasthenica gravis was confirmed and thymectomy was given. After thymectomy, symptoms were relieved but the administration of neostigmine was contijued to be needed till following 3 months. After that period, she was free from this symptoms without anticholinesterase drugs. Second case is 57 year old male who has the symptoms of diplopia, bilatreal ptosis, walking disturbance, and speech difficulty. He had thymectomy too but in thymic tissue, malignant thymoma was included. He has subjective improvement only, with no major reduction of medication requirements after thymectomy.

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Surgical treatment of myasthenia gravis: a report of 10 cases (중증 근무력증의 외과적 요법: 10례 보)

  • Jo, Dae-Yun;Kim, Yong-Jin;Kim, Ju-Hyeon
    • Journal of Chest Surgery
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    • v.17 no.2
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    • pp.292-298
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    • 1984
  • Myasthenia 8ravis is a disorder of neuromuscular function due to a reduction of available acetylcholine receptors at the neuromuscular junction. Typically, the muscle weakness is worse after effort and improved by rest. In 1939, Blalock and associated reported a case of patient with myasthenia gravis who was successfully treated by thymectomy. Since then, operation has become increasingly important in the management of this disease. From 1968 to 1983, 10 cases of myasthenia gravis were operated at the Department of Thoracic and Cardiovascular Surgery,College of Medicine, Seoul National University. 1.Among 10 cases, 4 were male and 6 were female. 2.Thymectomy was performed in 9 cases, and 1 case of malignant thymoma with deeply infiltration to adjacent structure was not resectable. 3.There was 1 operative mortality with respiratory failure. 4.During follow-up period, most of them showed symptomatic improvement, but complete remission was not noticed in any case.

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The Change of Spinal Motor Neuron Excitability by Neuromuscular Electrical Stimulation (신경근전기자극에 의한 척수운동신경원의 흥분성 변화)

  • Lee, Jeong-Woo;Kim, Tae-Youl;Lee, In-Hak;Lee, Joon-Hee
    • Journal of the Korean Academy of Clinical Electrophysiology
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    • v.1 no.1
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    • pp.1-15
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    • 2003
  • The purpose of this study was to determine the effect of neuromuscular electrical stimulation(NMES) on the alteration of spinal motor neuron excitability. In this article, I would like to experiment on a standard capacity of clinical electrophysiology, a difference in applying methods and a clinical efficiency of NMES by Nerve conduction velocity. We used normal eight subjects without neuromuscular disease and all subjects participated 3 session, which at least 1 week between session. Participants classified according to each group in Antagonist, Agonist, Antagonist-Agonist by the NMES. The test was measured continuously pre test, post-test, post 20 minute test by EMG including H reflex, F wave, motor nerve conduction velocity(MNCV). The following results were obtained; 1. H-reflex latencies and H/M intervals were significantly increased in agonist and antagonist-agonist group(p<.01). 2. H-reflex amplitudes and H/M ratios were significantly decreased in agonist and antagonist-agonist group(p<.01). In agonist group, H-reflex amplitudes and H/M ratios were more significantly decreased than antagonist group. 3. F-wave latencies were significantly increased in agonist and antagonist-agonist group(p<.01). F/M intervals were significantly increased in antagonist-agonist group(p<.01). F wave conduction velocities were significantly increased in agonist and antagonist-agonist group(p<.01) but F/M ratios were not significant. 4. MNCV were significantly decreased in agonist(p<.01). These results lead us to the conclusion that agonist and Antagonist-agonist was significantly decreased excitability of spinal motor neuron. Conversely, Antagonist does not decreased. Therefore, A further direction of this study will be to provide more evidence that NMES have an effect on excitability of spinal motor neurons in UMN syndrome.

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A case of spinal muscular atrophy typeⅡ (제 2 형 척수근위축증(SMA type II; Spinal muscular atrophy typeⅡ) 환아 1례에 대한 증례보고)

  • Jo Hyeong-Jun;Lee Jin-Yong;Kim Deok-Gon
    • The Journal of Pediatrics of Korean Medicine
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    • v.14 no.1
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    • pp.197-204
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    • 2000
  • Neuromuscular disorders are common causes of weakness and hypotonia in the infantile period and in childhood. Accurate diagnosis of specific neuromuscular disorders depends first on identification of which aspect of the peripheral neuromuscular system is affected-the motor neuron in the spinal cord, the nerve root or peripheral nerve, the neuromuscular junction, or the muscle-and then on the determination of the etiology and specific clinical entity. Spinal muscular atrophy(SMA) is the most common autosomal-recessive genetic disorder lethal to infants. The three major childhood-onset forms of SMA are now usually called type I, type II and typeⅢ. Progression of the disease is due to loss of anterior horn cells, thought to be caused by apoptosis. Diagnosis is based on the course of the illness, as well as certain changes seen on nerve and muscle biopsy and electrodiagnostic studies. More recently, our understanding of the genetics of this disorder has provided a noninvasive approach to diagnosis. We report on a 3-year-old male patient with spinal muscular atrophy type II. He had progressive muscular weakness since 18 months of age. The upper arms were slightly, and the thighs moderately atrophic. There was muscle weakness of both the upper and lower limbs, being more proximal in distribution. Electromyogram revealed a neurogenic pattern.

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The Effect of Proprioceptive Neuromuscular Facilitation Exercise on the Range of Motion, Pain, and Functional Activity of Total Knee Arthroplasty Patients (고유수용성신경근촉진법 운동이 무릎관절 전치환술환자의 관절가동범위와 통증 및 기능적 활동에 미치는 영향)

  • Kim, Chang-Heon;Kang, Tae-Woo;Kim, Beom-Ryong
    • PNF and Movement
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    • v.16 no.1
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    • pp.75-83
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    • 2018
  • Purpose: This study investigated the effects of proprioceptive neuromuscular facilitation (PNF) exercise on the range of motion (ROM), pain, and functional activity of patients who received total knee arthroplasty (TKA). The purpose of this study was to provide fundamental data regarding the use of PNF exercise among patients with musculoskeletal disease. Methods: Fourteen patients who received TKA were randomly divided into an experimental group (n=7) that took part in PNF exercise and a control group (n=7) that performed general rehabilitation exercise. Both groups performed the respective exercises for 30 minutes, five times a week for 2 weeks. For the measurement of ROM, the range of knee flexion was measured using a clinometer smartphone application. A visual analogue scale (VAS) was used for the measurement of the level of pain. The timed up and go test (TUG) was conducted to measure functional activity. A paired t-test was performed to compare within-group changes before and after the PNF exercise. Differences between the experimental group and control group were analyzed by an independent t-test. For all tests, the level of statistical significance was set at ${\alpha}=0.05$. Results: After the exercise, there was a significant within-group change in VAS and TUG scores in the experimental group and control group (p<0.01). There was also a significant between-group difference in VAS and TUG scores after the intervention (p<0.05). Conclusion: General rehabilitation exercise is commonly applied as a treatment for TKA patients and is relatively effective. The application of PNF exercise may be useful in such patients, considering its effects on ROM improvement, pain reduction, and functional enhancement.

Exercise program in cardiac rehabilitation (심장재활에서의 운동 프로그램)

  • Kim, Jwa-Jun;Kim, Dae-Kyeong;Kim, Min-Soo
    • PNF and Movement
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    • v.8 no.3
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    • pp.17-25
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    • 2010
  • Cardiac disease is the class of diseases related to the heart that plays an important role in supplying blood to our body and the number of deaths is increasing every year. Cardiac Rehabilitation has been conducted as treatment and prevention in such patients with cardiac disease. Cardiac rehabilitation programs in general contain pat~ient education and consulting service in order to improve physical strength in patients with cardiac disease, decrease cardiac symptoms, promote fitness, and minimize the risk of following cardiac problems including cardiac arrest. Among them therapeutic exercise is the mainstream of cardiac rehabilitation, however, to accomplish more efficient patient care, standardized guideline based on each disease and researches from a physical therapy perspective are required.

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