• 혜화의학회지
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• 제8권1호
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• pp.371-378
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• 1999

Through the thought of etiology and pathogenesis of convulsion disease in past document, we concluded as follow. 1. Convusion disease brings about some symptoms such as myotonia, neck stiffness, myospasm of four limbs, and in the ancient times it was called in Gye-Jong, Chu-Pung(抽風), Chi. 2. Etiologies of convulsion disease are external invasion of Pung-Han-Seub(風寒濕) and Ybul-Sa(熱邪), mistreatment, great loss of blood, deficiency of Gi-Hyul(氣血), stagnation of phlegm and blood. 3. There are four pathologic cases which arise convulsion disease. They are muscular denutrition from meridian stagnation by external invasion, muscular denutrition of heat injury, stagnation of phlegm and thrombus in meridian, muscular denutrition with deficiency of Gi-Hyul(氣血). 4. The treatment methods of convulsion disease are divided into three. If caused by external invasion, the methods are San-Han-Hae-Gi(散寒解肌), Hwa-Yung-Jo-Joong(和營調中). If caused by deficiency of Gi-Hyul(氣血), the method is Bo-Gi-Ik-Hyul(補氣益血). If caused by stagnation of phlegm and blood, the methods are Hwal-Hyul-So-Eo(活血消瘀), Do-Dam-Gun-Bi(導痰健脾).

• Annals of Clinical Neurophysiology
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• 제8권2호
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• pp.125-134
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• 2006

Among the various physiological factors that affect nerve conduction velocity (NCV), temperature is the most important. Because the influence of temperature is the most important source of error. It is known from animal experiments that conduction is eventually completely blocked at low temperatures, the myelinated A fibers being the first affected and the thin fibers of group C the last. Many studies showed that the NCV decreases linearly with lowering temperature within the physiological range. The distal motor latency increased by $0.2msec/^{\circ}C$ drop in temperature between $25^{\circ}C$and $35^{\circ}C$ in the median, ulnar and peroneal nerves. The temperature affect the neuromuscular transmission; The miniature endplate potential (MEPP) and endplate potential (EPP) are increase with increasing temperature. In myasthenia gravis, the reduction in the decremental response is observed following cooling. The lowering temperature make increase the amplitude of sensory compound action potential; make enlarge the surface area of compound muscle action potential with very little increase in amplitude; make diminish the fibrillation potential and increase the myotonia in needle electromyography (EMG). Because of these findings mentioned above, the skin temperature should be routinely monitored and controlled during nerve conduction tests and needle EMG and should be taken into account when interpreting the findings.

• 대한근전도전기진단의학회지
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• 제20권2호
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• pp.130-134
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• 2018

A 36-year-old male patient developed diffuse low back pain. His past medical history was unremarkable and had no family history of neuromuscular disease. He had no bladder and bowel problems. Creatine kinase was 172 U/L (normal < 170). Other fluid and blood chemistry tests were normal. Manual muscle test grades of extremities and sensory examination were normal. Muscle stretch reflexes were normal. Fasciculations and myotonia were not detected. Straight leg raising test was negative. There was no spinal root compression, spinal stenosis, or signal intensity change of spinal cord on magnetic resonance imaging (MRI). Fatty change and atrophy of the cervical, thoracic and lumbar paraspinal muscles were noted on MRI. Nerve conduction studies were normal. Electromyography showed 1+ positive sharp waves in the lumbar paraspinal muscles. Electromyography of upper and lower extremity muscles revealed no abnormal spontaneous activity. We report a rare case of severe paraspinal muscle atrophy with fatty degeneration in a Young Adult.

• The Korean Journal of Physiology and Pharmacology
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• 제9권1호
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• pp.1-8
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• 2005

Myotonic Dystrophies type 1 and 2 (DM1/2) are neuromuscular disorders which belong to a group of genetic diseases caused by unstable CTG triplet repeat (DM1) and CCTG tetranucleotide repeat (DM2) expansions. In DM1, CTG repeats are located within the 3' untranslated region of myotonin protein kinase (DMPK) gene on chromosome 19q. DM2 is caused by expansion of CCTG repeats located in the first intron of a gene coding for zinc finger factor 9 on chromosome 3q. The CTG and CCTG expansions are located in untranslated regions and are expressed as pre-mRNAs in nuclei (DM1 and DM2) and as mRNA in cytoplasm (DM1). Investigations of molecular alterations in DM1 discovered a new molecular mechanism responsible for this disease. Expansion of un-translated CUG repeats in the mutant DMPK mRNA disrupts biological functions of two CUG-binding proteins, CUGBP and MNBL. These proteins regulate translation and splicing of mRNAs coding for proteins which play a key role in skeletal muscle function. Expansion of CUG repeats alters these two stages of RNA metabolism in DM1 by titrating CUGBP1 and MNBL into mutant DMPK mRNA-protein complexes. Mouse models, in which levels of CUGBP1 and MNBL were modulated to mimic DM1, showed several symptoms of DM1 disease including muscular dystrophy, cataracts and myotonia. Mis-regulated levels of CUGBP1 in newborn mice cause a delay of muscle development mimicking muscle symptoms of congenital form of DM1 disease. Since expansion of CCTG repeats in DM2 is also located in untranslated region, it is predicted that DM2 mechanisms might be similar to those observed in DM1. However, differences in clinical phenotypes of DM1 and DM2 suggest some specific features in molecular pathways in both diseases. Recent publications suggest that number of pathways affected by RNA CUG and CCUG repeats could be larger than initially thought. Detailed studies of these pathways will help in developing therapy for patients affected with DM1 and DM2.

• The Journal of Korean Physical Therapy
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• 제35권6호
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• pp.177-184
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• 2023

Purpose: The purpose of this study was to compare the concurrent validity and test-retest reliability of 'KPIMT Torticollis Protractor', a smart phone and I-pad application for convenient range of motion measurement, and 'Image J', an analysis software with high reliability and validity, according to head tilt and active cervical rotation angle. This was done to determine the clinical utility of 'KPIMT Torticollis Protractor'. Methods: Head tilt and active cervical spine rotation angles of 40 children with congenital muscular torticollis were measured using Image J and KPIMT Torticollis Protractor, respectively. The level of concurrent validity and inter-rater and intra-rater reliability between the two measurement methods were analyzed. Results: For forty participants, the concurrent validity between Image J and KPIMT Torticollis Protractor showed very high validity with ICC of ICC 0.977 (0.995-0.999), 0.994 (0.994-0.998), CV_ME% 0.71-0.72%, SEM% 0.31-0.34%, MDC% 0.86-0.94%. The test-retest intra-rater reliability showed very high reliability ICC 0.911 (0.911-0.966), CV_ME% 0.71%, SEM% 0.34-0.36%, MDC% 0.81-0.94%. The test-retest inter-rater showed very high reliability ICC 0.936 (0.933-0.957), CV_ME% 0.70%, SEM% 0.34-0.35%, MDC% 0.81-0.83%. Conclusion: The KPIMT Torticollis Protractor, a smart phone and IPD application, is a highly reliable and valid device for angle measurement in children with congenital myotonia and can be easily used in clinical practice.