• 재활복지공학회논문지
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• 제10권2호
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• pp.107-112
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• 2016

본 연구는 수중 경사로 보행 훈련이 제2형 척수성근위축증(spinal muscular atrophy type II) 아동의 넙다리네갈래근 활성화와 보행 및 일상생활동작에 미치는 영향을 알아보고자 하였다. 본 연구의 대상자는 만 5세의 제2형 척수성근위축증으로 진단받은 아동이었다. 본 연구는 단일 대상자 반전 연구 설계(A-B-A)를 이용하여 일주일에 3번 12회기 실행하였고 기초선 설정기간(A), 실험 적용기간(B), 관찰기간(A)으로 나누어 진행하였다. 기초선 설정기간, 관찰기간에서는 일반적인 수중 치료가 적용되었고 실험 적용기간에는 일반적인 수중 치료와 수중 경사로 보행훈련(60m 경사로 걷기)이 추가되었다. 제2형 척수성근위축증 환자의 보행에 큰 영향을 끼치는 넙다리네갈래근 활성화 측정을 위해 표면 근전도 검사가 실시되었고, 동적인 기능평가를 위해 일어나 걷기 검사와 일상생활 기능평가(ACTIVLIM)를 실시하였다. 그 결과 기초선 설정기간에 비해 실험 적용기간에서 넙다리네갈래근의 활성화가 감소하였고 보행 시간도 감소하였다. 일상생활 기능은 변화가 나타나지 않았다. 하지만, 관찰기간동안 다시 넙다리네갈래근의 활성화 증가, 보행시간 증가, 일상생활 기능 감소가 나타난 것으로 보아 수중 경사로 보행 훈련이 본 아동에게 기능적으로 영향을 끼쳤음을 알 수 있었다.

• 한국전문물리치료학회지
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• 제22권2호
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• pp.70-80
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• 2015

The spinal cord is highly complex, consisting of a specialized neural network that comprised both neuronal and non-neuronal cells. Any kind of injury and/or insult to the spinal cord leads to a series of damaging events resulting in motor and/or sensory deficits below the level of injury. As a result, muscle paralysis (or paresis) leading to muscle atrophy or shrinking of the muscle along with changes in muscle fiber type, and contractile properties have been observed. Traditionally, histology had been used as a gold standard to characterize spinal cord injury (SCI)-induced adaptation in spinal cord and skeletal muscle. However, histology measurements is invasive and cannot be used for longitudinal analysis. Therefore, the use of conventional magnetic resonance imaging (MRI) is promoted to be used as an alternative non-invasive method, which allows the repeated measurements over time and secures the safety against radiation by using radiofrequency pulse. Currently, many of pathological changes and adaptations occurring after SCI can be measured by MRI methods, specifically 3-dimensional MRI with the advanced diffusion tensor imaging technique. Both techniques have shown to be sensitive in measuring morphological and structural changes in skeletal muscle and the spinal cord.

• The Journal of Korean Physical Therapy
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• 제22권3호
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• pp.9-15
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• 2010

Purpose: To evaluate, in patients with degenerative disc disease (DDD), the efficacy of using spinal stabilizing exercises for the reversal? of atrophy of the multifidus and psoas major, reductions in pain and disability, and for increases in paraspinal muscle strength. Methods: Nineteen patients diagnosed with DDD participated for 10 weeks in a spinal stabilization exercise program. Pain and disability were measured before and after exercise using, respectively, a visual analogue scale (VAS) and the Oswestry Disability Index (ODI). Paraspinal muscular strength in four directions was evaluated using CENTAUR. Both before and after exercise we used computed tomography (CT) too measure cross-sectional areas (CSAs) of both the left and right multifidus and the psoas major at the upper & lower endplate of L4. Results: After 10 weeks of a spinal stabilization exercise program, pain was significantly decreased from $5.7{\pm}0.9$ to $2.5{\pm}0.9$ (p<0.01); the ODI score decreased from $16.7{\pm}4.9$ to $7.3{\pm}3.1$. Paraspinal muscle strength was significantly increased (p<0.01) and the CSAs of the left and right multifidus and psoas major muscles were significantly increased (p<0.01). Conclusion: Spinal stabilization exercise is effective in reversing atrophy in DDD patients, in reducing pain and disability, and in increasing paraspinal muscle strength. It is an effective treatment foro aiding rehabilitation in these cases.

• 한방재활의학과학회지
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• 제19권4호
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• pp.151-163
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• 2009

Objectives : In the assessment of the lumbar spine by magnetic resonance imaging (hereinafter, "MRI"), changes in the paraspinal muscles are overlooked. The purpose of our study is to examine the correlation between the multifidus muscle atrophy on MRI findings and the clinical findings in low back pain (hereinafter, "LBP") patients. Methods : The retrospective study on 38 LBP patients, presenting either with or without associated leg pains, was undertaken. The MRI findings on the patients were visually analysed to find out a lumbar multifidus muscle atrophy, disc herniation, disc degeneration, spinal stenosis and nerve root compressions. The clinical history in each case was obtained from their case notes and pain drawing charts. Results : The lumbar multifidus muscle atrophy has occurred from more than 80% of the patients with LBP. Most of lumbar multifidus muscle atrophies have increased from lower level of lumbar spine. It was bilateral in the majority of the cases. In addition, multifidus muscle atrophy was correlated to the patient's age, disc degenerations and spinal stenosis. On the contrary, gender, the duration of LBP, referred leg pain, disc herniation and nerve root compressions had no relevance to multifidus muscle atrophies. Therefore, when assessing the MRIs of the lumbar spine, we should have more attetion on multifidus muscle, because it has lot's of information about spinal neuropathy problems. Conclusions : Therefore, the examination of multifidus muscle atrophies should be considered when assessing the MRIs of the lumbar spine. In addition, it helps to evaluate and plan the treatment modalities of LBP. Moreover, it prevents from LBP by discovering the importance between the multifidus muscle and the spine stabilization exercise.

• 대한소아신경학회지
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• 제26권4호
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• pp.189-196
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• 2018

목적: 늘어지는 영아 증후군은 중추신경계 이상, 말초 신경계 이상 혹은 둘 모두의 이상으로 발생할 수 있다. 늘어지는 영아에서 원인을 진단하는 것은 환아의 치료와 발달 예후를 결정하는 중요한 요소로 현재까지 다양한 진단 알고리듬이 제안되고 있다. 본 논문에서는 늘어지는 영아 증후군의 원인에 대한 새로운 분류 및 증상 발현 시기에 따른 원인, 그리고 이들의 발달 예후에 대해 연구하였다. 방법: 2005년부터 2016년까지 세브란스병원에 내원한 늘어지는 영아들을 대상으로 EMR 차트를 후향적으로 분석하여 진단 및 임상적 특징을 분석하였고 환아들의 발달에 대해 보호자에게 일대일 전화인터뷰를 통해 조사하였다. 결과: 전체 116명의 환아 중에 원인에 대한 확진을 받은 경우가 69명으로 전체 진단율이 59.5%이었고 이들 중 Prader-Willi syndrome, myotonic dystrophy, spinal muscular atrophy가 가장 흔한 진단이었다. 전 연령대에 걸쳐 Prader-willi syndrome이 가장 흔한 진단이었고 특히 1개월 미만 증상 발현군에서는 Prader-willi syndrome, myotonic dystrophy, early infantile epileptic encephalopathy가 흔한 3가지의 진단이었다. 발달 예후 면에서 원인군 중 combined hypotonia에서 전 영역에 걸쳐 가장 나쁜 예후를 보였다. 결론: 현재까지의 논문과 본 논문에서의 늘어지는 영아 증후군에 대한 진단율은 유사했고 각 연령에 따른 흔한 진단에 대해서도 알아보았다. 발달 예후가 가장 나쁜 combined hypotonia군에 속하는 진단으로 확진되거나 의심되는 경우 초기 진단시부터 발달에 대해 체계적이고 단계적인 추적관찰이 필요하다.

• Annals of Clinical Neurophysiology
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• 제8권1호
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• pp.36-39
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• 2006

Background: Floppy infant syndrome has a number of different etiologies. Methods: One hundred twenty-three consecutive patients of floppy infant syndrome were included in this study. We reviewed all the electrophysiologic tests of these patients and the medical record of patients showing abnormalities in the electrophysiologic studies. Results: Of the 123 patients, twenty-six (21.1%) showed definite abnormalities in electrophysiologic tests; 8 myopathies, 14 neuropathies and 4 unclassified. The neuropathy was further classified as 5 neuronopathies and 9 sensorimotor polyneuropathies. With muscle or sural nerve biopsy and genetic test, a final diagnosis was made of Duchenne muscular dystrophy in 4, Becker muscular dystrophy in 1, spinal muscular atrophy in 2, and metachromatic leukodystrophy in 1. Conclusions: About 21% of patients presented with floppy infant syndrome showed abnormalities in the neuromuscular system. The electrophysiologic test is valuable to guide further investigations in diagnosing the cause of floppy infant syndrome.

• 대한간호학회지
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• 제41권4호
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• pp.520-527
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• 2011

Purpose: The purpose of this study was to examine effects of nitric oxide synthase (NOS) inhibitor on muscle weight and myofibrillar protein content of affected and unaffected hindlimb muscles in rats with neuropathic pain induced by unilateral peripheral nerve injury. Methods: Neuropathic pain was induced by ligation and cutting of the left L5 spinal nerve. Adult male Sprague-Dawley rats were randomly assigned to one of two groups: The NOSI group (n=19) had NOS inhibitor (L-NAME) injections daily for 14 days, and the Vehicle group (n=20) had vehicle injections daily for 14 days. Withdrawal threshold, body weight, food intake and activity were measured every day. At 15 days all rats were anesthetized and soleus, plantaris and gastrocnemius muscles were dissected from hindlimbs. Muscle weight and myofibrillar protein content of the dissected muscles were determined. Results: The NOSI group showed significant increases as compared to the Vehicle group for body weight at 15 days, muscle weight and myofibrillar protein content of the unaffected soleus and gastrocnemius. The NOSI group demonstrated a higher pain threshold than the vehicle group. Conclusion: NOSI for 14 days attenuates unaffected soleus and gastrocnemius muscle atrophy in neuropathic pain model.

• 대한한의학방제학회지
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• 제25권4호
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• pp.499-508
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• 2017

Objectives : Skeletal muscle atrophy occurs in response to a variety of conditions. The unloading to muscle occurs clinically in limb immobilization, bed rest, spinal cord injury and peripheral nerve damage, resulting in significant loss of muscle mass and force production. Muscle disuse is accompanied by an increase in apoptotic signaling, which mediates some of the responses to unloading in the muscle. In this study we tested the hypothesis that Daeyeoung-jeon extract would improve muscle recovery after reloading following disuse. Method : Twenty young male Sprague-Dawley rats were used for the studies. The hindlimb immobilization was performed with casting tape to keep the left ankle joint in a fully extended position. No intervention was performed on the right leg and used as intact region. The Rats in Daeyeoung-jeon treated group (DYJ) were orally administrated Daeyeoung-jeon water extract, and rats of Control group were given with saline only. After 2 weeks of immobilization, all animals were sacrificed, and the whole gastrocnemius muscles were dissected from both legs. The morphology of right and left gastrocnemius muscles in both DYJ and Control groups were assessed by hematoxylin and eosin staining. Moreover, to investigate the immobilization-induced muscular apoptosis, the immunohistochemical analysis of Bax and Bcl-2 was carried out. Results : Daeyeoung-jeon represented the significant protective effects against the reductions of the left gastrocnemius muscles weight and average cross section area to compared with Control group. The treatment with Daeyeoung-jeon extract significantly reduced the immunoreactivity of BAX and increased the immunoreactivity of Bcl-2 in gastrocnemius muscle compared with Control group. Conclusion : Daeyeoung-jeon has protective effects against immobilization-induced muscle atrophy by regulating the activities of apoptosis-associated BAX/Bcl-2 proteins in gastrocnemius muscle.

• Journal of Korean Biological Nursing Science
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• 제10권1호
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• pp.88-95
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• 2008

Purpose: The purpose of this study was to examine the effect of neuropathic pain by peripheral nerve injury on mass and Type I and II fiber cross-sectional areas on hindlimb muscles of the neuropathic pain model rat. Method: Adult male Sprague-Dawley rats (body weight 200-220 g) were assigned to one of two groups: a neuropathic pain group (n=7) that had a ligation of the left L5 spinal nerve, a control group (n=5), a naive rat without any procedures. Withdrawal threshold, activity, body weight and food intake were measured daily. At 8 days after neuropathic pain, all rats were anesthetized and the soleus and plantaris muscles were dissected from the both hindlimbs. Body weight, food intake, muscle weight and Type I and II fiber cross-sectional area of the dissected muscles were determined. Result: The neuropathic pain group showed a significant decreases (p<.05) as compared with the control rats, in diet intake, body weight, muscle weight and Type II fiber cross-sectional area of the left (affected side) soleus and plantaris muscles, and the right (unaffected side) muscle weight of plantaris and Type II fiber cross-sectional area of the soleus muscle. Conclusion: The hindlimb muscle atrophy occurs in both affected and unaffected side due to neuropathic pain by the peripheral nerve injury. The hindlimb muscle atrophy of the affected side is more pronounced than that of the unaffected side.

• Molecules and Cells
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• 제46권1호
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• pp.10-20
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• 2023

Antisense oligonucleotide (ASO) technology has become an attractive therapeutic modality for various diseases, including Mendelian disorders. ASOs can modulate the expression of a target gene by promoting mRNA degradation or changing pre-mRNA splicing, nonsense-mediated mRNA decay, or translation. Advances in medicinal chemistry and a deeper understanding of post-transcriptional mechanisms have led to the approval of several ASO drugs for diseases that had long lacked therapeutic options. For instance, an ASO drug called nusinersen became the first approved drug for spinal muscular atrophy, improving survival and the overall disease course. Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause cystic fibrosis (CF). Although Trikafta and other CFTR-modulation therapies benefit most CF patients, there is a significant unmet therapeutic need for a subset of CF patients. In this review, we introduce ASO therapies and their mechanisms of action, describe the opportunities and challenges for ASO therapeutics for CF, and discuss the current state and prospects of ASO therapies for CF.