• Journal of Genetic Medicine
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• v.1 no.1
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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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• v.28 no.3
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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.

• Journal of Veterinary Clinics
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• v.21 no.2
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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.

• Journal of Physiology & Pathology in Korean Medicine
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• v.17 no.5
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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.

• Journal of Biomedical Engineering Research
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• v.22 no.3
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• pp.249-257
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• 2001

The voluntary contracted EMG signal of spinal cord injured patients is very small because the information from central nervous system is not sufficiently transmitted to $\alpha$ motor neuron or muscle fiber. Therefore the acquisited EMG signal from needle or surface electrodes can not be identified obvious voluntary contraction pattern by muscle movement. In this paper we propose the extraction technique of voluntary muscle contraction and relaxation pattern from EMG signal of spinal cord injured patient whose EMG signal is composed of the linear sum of mo색 unit action potentials with two noise sources, additive noise assumed to be white Gaussian noise and high frequency discharge assumed to be not motor unit action potential but impulsive noise. In order to eliminate impulsive noise and additive noise from voluntary contracted EMG signal, we use the FatBear filter which is a nonarithmetic piecewise constant filter, and multiscale nonlinear wavelet denoising processing, respectively. The proposed technique is applied to the EMG signal acquisited from transverse myelitis patients to extract voluntary muscle contraction pattern.

• Advances in Traditional Medicine
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• v.1 no.2
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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.

• Proceedings of the KOSOMBE Conference
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• v.1997 no.05
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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.

• Journal of Korean Medicine Rehabilitation
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• v.20 no.2
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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.

• 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.

• Journal of Veterinary Clinics
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• v.30 no.2
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• pp.142-145
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• 2013

Equine motor neuron disease (EMND) is a spontaneous neurologic disorder of horses, which results from the degeneration of motor neurons in the spinal cord and brain stem. An 8-year-old Jeju pony gelding presented with weight loss, muscle tremors, frequent recumbence, low head carriage, sweating, and standing with four limbs close together. The gelding has been on the same stable and limited access to pasture for several years. The gelding has been fed with dried hays and commercial concentrated feeds. Laboratory test revealed very low serum vitamin E level (0.14 ${\mu}g/mL$; reference range > 1.5 ${\mu}g/mL$), mildly elevated creatine kinase (402 IU/L; reference range 119-287 IU/L) and aspartate aminotransferase (876 IU/L; reference range 226-336 IU/L). Oral glucose absorption test showed decreased glucose absorption. Histopathologic examination using a biopsy specimen from sacrocaudalis dorsalis medialis muscle revealed atrophic and hypertrophic muscle fiber, centralization of nucleus, degenerating and necrotic muscle fibers. Taken together, the gelding was diagnosed as EMND. After oral vitamin E administrated for 5 weeks, the gelding showed normally improved stance, decreased periods of recumbency, improved head carriage and weight gain except consistent tremors. This is the first report that successfully treated EMND case occurred in a Jeju pony in Korea.