• Journal of Genetic Medicine
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• 제1권1호
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• pp.33-37
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• 1997

Spinal muscular atrophy (SMA) is the second most common fatal disease of childhood with autosomal dominant mode of inheritance, and in its less severe form the third most common neuromuscular disease of childhood after Duchenne muscular dystrophy. The genetic defect was found to be on the long arm of chromosome 5 (5q11.2-q13.3) where many genes and microsatellite markers were missing. One of the most important genes is the Survival Motor Neuron (SMN) gene which is homozygously missing in 90% of SMA patients. Another important gene, the Neuronal Apoptosis Inhibitory Protein (NAIP) gene was found to be defective in 67% of SMA type I patients. Studies so far suggest SMA occurs when the genes on the long arm of chromosome 5 are mutated or deleted. Recently our hospital encountered 2 SMA patients of type I and II respectively. These patients both had homozygously defective SMN genes but intact NAIP genes. We are reporting these cases with bibliographic review and discussion. Korean SMA patients presumably have defects in SMN genes similar to those found in European patients, although the significance of NAIP genes remains to be established. SMN gene defects can be easily diagnosed using PCR and restriction enzymes, and this method could be applied towards convenient prenatal diagnosis and towards screening for family members at risk.

• Journal of Acupuncture Research
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• 제28권3호
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• pp.33-42
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• 2011

Objectives : This study was performed to evaluate the effects of Scutellariae radix (SR) water extract on locomotor dysfunction induced by spinal cord injury (SCI) in rats. Methods : SCI was induced mechanical contusion following laminectomy of 10 th thoracic vertebra in Sprague-Dawley rats. SR was orally given once a day for 7 days after SCI. Neurological behavior was examined with the Basso-Beattie-Bresnahan locomotor rating scale. Tissue damage and nerve fiber degeneration were examined with cresyl violet and luxol fast blue (LFB) histochemistry. Using immunohistochemistry, cellular damages to neurons and nerve fibers were examined MAP-2. Results : 1. SR significantly ameliorated the locomotor dysfunction of the SCI-induced rats. 2. SR significantly reduced the number of motor neurons in the ventral horn of the SCI-induced rat spinal cord. 3. SR attenuated the reduction of nerve fiber shirnakage and degeneration of the SCI-induced rat spinal cord. 4. SR attenuated the reduction of MAP-2 positive cells in the peri-lesion of the SCI-induced rat spinal cord. Conclusions : These results suggest that SR improves the locomotor dysfunction of SCI by reducing degeneration of nerve fibers and motor neuron shrinkage in the ventral horn.

• 한국임상수의학회지
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• 제21권2호
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• pp.177-180
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• 2004

Lower motor neuron signs of hind limbs, anus and bladder were identified by history taking and physical examination in the 6.8 year-old mongrel dog and 2.6 year-old Cocker spaniel. The Cocker spaniel, also showed gradual cranial migration of neurologic deficit including respiratory paralysis. On plain radiography and myelography, intervertebral disc extrusion between L2 and L3, the infiltration of contrast medium into the spinal cord and cord swelling were found in the mongrel dog, and infiltration of contrast medium like hollowness of cord parenchyma was observed in the Cocker spaniel. On the basis of clinical signs and radiographic findings, they were diagnosed tentatively as acute myelomalacia. The Cocker spaniel died of respiratory paralysis on the following day. Decompressive surgery was performed on the mongrel dog and the extensive necrosis and hemorrhage were found at surgery. It was euthanized with the owner's consent because of the perceived poor prognosis. Histopathologic examination after autopsy confirmed acute diffuse hemorrhagic myelomalacia with the swelling and the inflammation of axon, showing hemorrhagic changes in the white matter and the grey matter.

• 동의생리병리학회지
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• 제17권5호
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• pp.1305-1308
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• 2003

To evaluate the neurotoxicity of reactive oxygen species (ROS) in cultured cultured spinal dorsal root(DRG) neurons derived from neonatal mouse, Cytotoxicity was measured by MTS assay after cultured cells were grown for 3 hours in the media containing 1～60 μM hydrogen peroxide (H₂O₂). In addition the neuroprotective effect of Salviae Miltiorrhzae Radix (SMR) was measured in these cultrures. Cell viability was positively decreased in a dose- and time-dependent manner after exposure of cultured mouse DRG neurons to 30 tt M H202 for 3 hours. In the neuroprotective effect of SMR on H₂O₂-mediated toxicity, SMR prevented the H₂O₂-induced neurotoxicity in these cultures. From these results. it suggests that H₂0₂ is toxic in cultured mouse spinal motor neurons and selective herb extract such as Uncariae Ramulus Cum Uncis is effective in prevetion of the neurotoxicity induced by H₂O₂.

• 대한의용생체공학회:의공학회지
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• 제22권3호
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• pp.249-257
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• 2001

본 논물에서는 척수 손상으로 인하여 암이 수축 및 이완시 미약한 근전도, 신호를 발생시키는 환자로부터 명확한 수축 및 이완 패턴을 추출하기 위한 신호 처리 기법을 제안하였다. 제안한 방법은 비선형 고정 필터의 일종인 FatBear 필터를 이용하여 거대 운동단위 활동전위로 의심되는 충격 잡음을 제거하고 웨이브렛 평면에서 비선형 멀티 스케일 필터링 기법을 이용하여 가산 잡음을 제거하는 것으로서 횡단성 척수염으로 인한 마미 증후근을 보이는 환자들에게 적용하여 명확한 수축 및 이완 패턴을 추출할 수 있었다.

• Advances in Traditional Medicine
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• 제1권2호
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• pp.59-65
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• 2000

To clarify the toxic effect of oxidative stress, hydrogen peroxide $(H_{2}O_{2})-induced$ neurotoxicity was examined in cultured newborn mouse spinal motor neurons after spinal motor neurons were grown in the media containing various concentrations of glucose oxidase (GO). And also, the protective effect of Rhizoma gastrodiae extract against GO-induced neurotoxicity was evaluated. Cytotoxicity was expressed as a cell viability by 3-(4,5-dimethylthiazol-2-yl) -2,5-diphenyltetrazolium bromide (MTT) assay. In this study, exposure of motor neurons to GO-induced cell death significantly, in a dose- and time-dependent manners in spinal motor neuron cultures. The decrease in cell viability of motor neurons damaged by GO was proventioned by Rhizoma gastrodiae extract. These results suggest that the neuroprotective effect of Rhizoma gastrodiae extract on GO-induced neurotoxicity may result from a attenuation of $H_{2}O_{2}-induced$ oxidative stress.

• 대한의용생체공학회:학술대회논문집
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• 대한의용생체공학회 1997년도 춘계학술대회
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• pp.398-401
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• 1997

The EMG signal of spinal cord injured patient is very feeble because that the information from central nervous system is not sufficiently transmitted to molter neuron or muscle fiber. Therefore the observer can not observe contraction and relaxation movement of muscle from the raw EMG signal. In this paper, we propose the muscle contraction and relaxation pattern analysis method of spinal cord injured patient whose EMG signal is composed of the sum of motor unit action potential train with additive white Gaussian noise and impulsive noise. From the EMG model, we denoise impulsive noise using median filter which is a kind of nonlinear filter and the output of median filter is transformed to wavelet transform domain for denoising additive white Gaussian noise using threshold level removal technique. As a result, we can obtain the clear contraction and relaxation pattern.

• 한방재활의학과학회지
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• 제20권2호
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• pp.1-15
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• 2010

Objectives : This study was performed to evaluate the effects of root of Cibotii rhizoma(CR) ethanol extract on the tissue and neuronal damage of the spinal cord injury(SCI). Methods : SCI was induced by mechanical contusion following laminectomy of 10th thoracic vertebra in Sprague-Dawley rats. CR was orally given once a day for 7 days after SCI. Tissue damage and nerve fiber degeneration were examined with cresyl violet and luxol fast blue(LFS) histochemistry. HSP72(as neuronal damage marker), MAP2(as nerve fiber degeneration marker), c-Fos(immediate early gene), and Bax(pro-apoptotic molecule) expressions were examined using immuno-histochemistry. Individual immuno-positive cells expressing HSP72, MAP2, c-Fos and Bax were observed on the damaged level and the upper thoracic and lower lumbar spinal segments. Results : 1. CR reduced degeneration of nerve fibers and motor neuron shrinkage in the ventral horn of the lower lumbar spinal segment, but generally it did not seem to ameliorate the tissue injury following SCI. 2. CR reduced demyelination in the ventral and lateral funiculus of the lower lumbar spinal segment. 3. CR reduced HSP72 expression on the neurons in the peri-central canal gray matter adjacent to the damaged region. 4. CR strengthened MAP2 expression on the motor neurons in the ventral horn and on nerve fibers in the lateral funiculus of the lower lumbar spinal segment. 5. CR reduced c-Fos positive cells in the peri-lesion and the dorsal horn of the damaged level and in the ventral horn of the lower lumbar spinal segment. 6. CR reduced Bax positive cells in the peri-lesion and the dorsal horn of the damaged level and in the ventral horn of the lower lumbar spinal segment. Conclusions : These results suggest that CR plays an inhibitory role against secondary neuronal damage and nerve fiber degeneration. following SCI.

• 대한한방소아과학회지
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• 제14권1호
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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.

• 한국임상수의학회지
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• 제30권2호
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• pp.142-145
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• 2013

말운동신경세포질환은 척수와 뇌간에 존재하는 운동신경의 변성에 의해 자발적으로 발병하는 말의 신경성 질환이다. 8년령의 제주 조랑말 거세마 1두가 체중감소, 근육진전, 잦은 횡와, 낮은 두위, 발한, 사지를 모아 서있는 증상으로 내원하였다. 환마는 장기간 동일한 마방 내에서 초지 방목 없이 건초와 농후사료로 사육되었다. 혈청 생화학검사 결과 혈청 비타민 E(0.14 ${\mu}g/mL$; 정상 > 1.5 ${\mu}g/mL$)의 감소, CK(402 IU/L; 정상 119-287 IU/L) 및 AST(876 IU/L; 정상 226-336 IU/L)의 증가가 관찰되었다. 포도당 경구흡수시험에서는 부분적 흡수장애를 나타내었다. 미근부 근육생검 후 조직검사 결과 근섬유 위축과 비대, 핵 중심화, 변성 및 괴사된 근섬유가 관찰되었다. 상기의 내용을 종합하여 환마는 말운동신경세포질환으로 진단되었다. 경구 비타민 E 투여 5주 후, 환마는 정상적으로 개선된 기립자세와 두위, 횡와 빈도의 감소 및 체중증가를 보였다. 본 증례는 한국의 제주 조랑말에서 발생한 말운동신경세포질환으로서 비타민 E 치료에 성공한 최초의 보고이다.