• Journal of Acupuncture Research
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• v.20 no.3
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• pp.209-216
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• 2003

목적 : 대표적인 motor neuron disease(MND)이면서 가장 치명적인 신경퇴행성 질환인 ALS의 발생 양상과 한방치료효과에 대하여 연구하였다. 방법: 상지대학교 부속 한방병원에 래원한 ALS 환자들의 성별, 연령, 발병일, 발병양상, 래원 당시의 병태양상 등을 분석하였고, 이 중 3개월 이상 입원치료를 통하여 치료과정 평가가 가능하였던 18명의 환자들의 치료 전과 치료 후의 변화양상을 평가하였다. 결과 : 외국의 보고에 비하여 여성 환자가 많았고 발병 연령도 유의하게 낮았으며 상지에서 최초의 증상이 발현되는 비율이 상대적으로 높았다. Bulbar From의 비율도 높았고, 대부분의 환자가 여성인 것도 특이하였다. 환자들이 생각하는 질병의 발생원인 중 약 80%가 stress나 정신적 충격 등을 지적하여 정신적 불안상태가 유관함을 추정할 수 있었다. 한방치료가 ALS를 호전시키지는 못하였으나 진행을 억제하였고, 국소적인 주소증을 완화시키며 심리적 안정 상태를 유지하는데 도움이 된다고 평가되었다.

• 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.

• Annals of Clinical Neurophysiology
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• v.3 no.1
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• pp.37-39
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• 2001

Although the etiology and pathogenesis of amyotrophic lateral sclerosis(ALS) is unknown, increasing evidence supports a role for autoimmune mechanisms in motor neuron degeneration. The coexistence of immune disease in ALS supports that an altered immune system may contribute to disease pathogenesis. A 55-year-old woman was admitted to our department due to dysarthria and gait disturbance. On physical and neurologic examination, she showed thyroid enlargement, tongue atrophy, muscle weakness, fasciculation, and increased deep tendon reflex. The electrophysiological studies are compatible with motor neuron disease. Cytological findings of thyroid were compatible with Hashimoto's thyroiditis. Thus, we report a case of ALS combined with Hashimoto's thyroiditis. And the simultaneous presentation with ALS and Hashimoto's thyroiditis led us to consider whether this was simply a chance association or not.

• Annals of Clinical Neurophysiology
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• v.20 no.1
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• pp.26-30
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• 2018

Background: The motor unit number index (MUNIX) and motor unit size index (MUSIX) refer to the electrophysiological measurement of the motor units using the surface electromyographic interference pattern (SIP) recorded during graded muscle contraction. In order to improve the reliability and reproducibility of MUNIX by the systematization of the graded muscle contractions, we applied a digital hand instrument to the procedure of recording SIP signals. Methods: We tested the applicability of the digital instrument in the MUNIX technique by assessing the mean values and the reproducibility of the MUNIX involving the abductor pollicis brevis (APB) and the abductor digiti minimi (ADM) muscles in 30 healthy adults. Results: The digital dynamometer was successfully applied to the MUNIX measurements of the APB and ADM muscles, and showed high reproducibility across trials. Conclusions: Application of the digital instrument would be useful in improving the reliability and reproducibility of MUNIX.

• 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.

• Annals of Clinical Neurophysiology
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• v.4 no.2
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• pp.98-107
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• 2002

Multifocal motor neuropathy (MMN) is a chronic immune-mediated peripheral myelinopathy. The major clinical features include slowly progressive, painless, and asymmetric weakness, usually of distal limb muscle. Early in the course of the disease, weakness is not necessarily associated with muscle atrophy, owing to the initial primary involvement of peripheral myelin. Chronic progressive weakness is often associated with some degree of concurrent axonal loss and subsequent muscle atrophy. Sensory symptoms are usually mild or absent, and involvement of cranial and respiratory muscles is rare. The findings of multifocal motor conduction block, abnormal temporal dispersion, and focal conduction slowing at segments not at risk for common entrapment or compression injury, associated with normal sensory conduction studies along the same segments, are the hallmark electrophysiologic features of MMN. The slow progression and absence of upper motor neuron signs are the major clinical points that separate MMN from amyotrophic lateral sclerosis. The role of GM1 antibodies, found in high titers in 22~84% of MMN patients, remains uncertain. The contention that MMN is an autoimmune disorder is largely based on the often dramatic improvement in symptoms following the administration of intravenuos immunoglobulin or cyclophosphamide.

• Clinical and Experimental Pediatrics
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• v.51 no.12
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• pp.1350-1354
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• 2008

Spinal muscular atrophy (SMA) is an autosomal recessive disease characterized by diffuse proximal and distal weakness due to deletion of the survival motor neuron (SMN) gene localized on chromosome 5 (5q11.2-13.3). SMA has been considered as a pure lower motor neuron disorder, and a definitive diagnosis can be established by molecular genetic testing. Here, we describe two patients with severe hypotonia and frequent aspirations at early infancy. Nerve conduction studies showed more extensive sensory involvement in these patients diagnosed to have SMA by genetic study than in classical cases of SMA. To the best of our knowledge, this is the first report of SMA Type 1 with sensory nerve involvement in Korea.

• Annals of Clinical Neurophysiology
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• v.19 no.1
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• pp.34-39
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• 2017

Background: Automated nerve excitability testing is used to assess various peripheral neuropathies and motor neuron diseases. Comparing these excitability parameters with normal data provides information regarding the axonal excitability properties and ion biophysics in diseased axons. This study measured and compared normal values of axonal excitability parameters in both the distal motor and sensory axons of normal Koreans. Methods: The axonal excitability properties of 50 distal median motor axons and 30 distal median sensory axons were measured. An automated nerve excitability test was performed using the QTRACW threshold-tracking software (Institute of Neurology, University College London, London, UK) with the TRONDF multiple excitability recording protocol. Each parameter of stimulus-response curves, threshold electrotonus, current-voltage relationship, and recovery cycle was measured and calculated. Results: Our Korean normal data on axonal excitability showed ranges of values and characteristics similar to previous reports from other countries. We also reaffirmed that there exist characteristic differences in excitability properties between motor and sensory axons: compared to motor axons, sensory axons showed an increased strength-duration time constant, more prominent changes in threshold to hyperpolarizing threshold electrotonus (TE) and less prominent changes in threshold to depolarizing TE, and more prominent refractoriness and less prominent subexcitability and superexcitability. Conclusions: We report normal data on axonal excitability in Koreans. These data can be used to compare various pathological conditions in peripheral nerve axons such as peripheral neuropathies and motor neuron disease.

• Annals of Clinical Neurophysiology
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• v.5 no.1
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• pp.11-15
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• 2003

Backgrounds: Kennedy disease is a X-linked recessive disease characterized by bulbar symptoms, proximal muscle weakness, and gynecomastia. Methods: We analyzed clinical symptoms and performed electrodiagnostic studies on 6 patients. Results: We found following features: 1) proximal muscle weakness 2) bulbar symptoms, as dysarthria, facial and tongue atrophy 3) hyporeflexia or areflexia 4) fasciculations, predominantly on face, and proximal upper extremities 5) decreased sensory nerve action potentials(SNAPs) 6) chronic neurogenic changes in needle EMG. Conclusions: Kennedy disease is characterized by degenerative process of anterior horn cell and dorsal root ganglion without upper motor neuron dysfunction. Increased triple nucleotide CAG repeats(>38) in androgen receptor gene of Xp21 will confirm early stage of this disease.

• Journal of Yeungnam Medical Science
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• v.39 no.3
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• pp.181-189
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• 2022

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative condition characterized by loss of motor neurons, resulting in motor weakness of the limbs and/or bulbar muscles. Pain is a prevalent but neglected symptom of ALS, and it has a significant negative impact on the quality of life of patients and their caregivers. This review outlines the epidemiology, clinical characteristics, underlying mechanisms, and management strategies of pain in ALS to improve clinical practice and patient outcomes related to pain. Pain is a prevalent symptom among patients with ALS, with a variable reported prevalence. It may occur at any stage of the disease and can involve any part of the body without a specific pattern. Primary pain includes neuropathic pain and pain from spasticity or cramps, while secondary pain is mainly nociceptive, occurring with the progression of muscle weakness and atrophy, prolonged immobility causing degenerative changes in joints and connective tissue, and long-term home mechanical ventilation. Prior to treatment, the exact patterns and causes of pain must first be identified, and the treatment should be tailored to each patient. Treatment options can be classified into pharmacological treatments, including nonsteroidal anti-inflammatory drugs, antiepileptic drugs, drugs for cramps or spasticity, and opioid; and nonpharmacological treatments, including positioning, splints, joint injections, and physical therapy. The development of standardized and specific assessment tools for pain-specific to ALS is required, as are further studies on treatments to reduce pain, diminish suffering, and improve the quality of life of patients with ALS.