• Annals of Clinical Neurophysiology
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• v.2 no.2
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• pp.107-113
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• 2000

Background & Objectives : Motor evoked potentials(MEPs) to magnetic trans cranial stimulation were performed to evaluate upper motor neuron involvement and relationship to lower motor neuron involvement in motor neuron disease patients. Method : MEPs were obtained in the 17 consecutive patients with motor neuron disease. These patients were divided into three group based on clinical evidence of upper and lower motor neuron involvement, bulbar symptom; amyotrophic lateral sclerosis(ALS), progressive muscular atrophy(PMA), progressive bulbar palsy(PBP). MEPs were recorded from abductor pollicis brevis and abductor hallucis muscles. Abnormal MEPs were defined by delayed central motor conduction time or absent MEP. Results : MEPs were abnormal in 64%(11/17) of patients; 100%(7/7) in ALS, 64%(4/7) in PMA, 0%(0/3) in PBP respectively. In 68 total recording muscles, 34 muscles had evidence of motor weakness and showed abnormal responses in 59%(20/34). Whereas 34 muscles with normal strength, only 3%(1/34) of muscles showed abnormal response. Conclusion : MEPs are well correlated with upper motor neuron signs in ALS and may detect masking upper motor neuron signs in PMA. The muscles with lower motor neuron sign(weakness) usually relate with abnormal MEPs reponses.

• Annals of Clinical Neurophysiology
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• v.17 no.1
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• pp.1-16
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• 2015

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that is characterized by progressive death of motor neurons in the cortex, brainstem, and spinal cord. Until now, many treatment strategies have been tested in ALS, but so far only Riluzole has shown efficacy of slightly slowing disease progression. The pathophysiological mechanisms underlying ALS are multifactorial, with a complex interaction between genetic factors and molecular pathways. Other motor neuron disease such as spinal muscular atrophy (SMA) and spinobulbar muscular atrophy (SBMA) are also progressive neurodegenerative disease with loss of motor neuron as ALS. This common thread of motor neuron loss has provided a target for the development of therapies for these motor neuron diseases. A better understanding of these pathogenic mechanisms and the potential pathological relationship between the various cellular processes have suggested novel therapeutic approaches, including stem cell and genetics-based strategies, providing hope for feasible treatment of ALS.

• Annals of Clinical Neurophysiology
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• v.11 no.2
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• pp.74-77
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• 2009

We report a 23-year-old woman with adult Sandhoff disease, who presented with motor neuron disease phenotype. The patient had experienced progressive motor weakness in four extremities since 1 year prior to admission. Electrophysiological study revealed wide-spread denervation potentials, and the assay of total hexosaminidase involving A and B activities showed decreased levels of these activities, which was consistent with Sandoff disease. This is the first Korean case of adult Sanhoff disease presented as a motor neuron disease phenotype.

• Annals of Clinical Neurophysiology
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• v.21 no.1
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• pp.61-65
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• 2019

$Sj{\ddot{o}}gren^{\prime}s$ syndrome (SS)-associated polyradiculopathy is rarely reported. A 51-year-old woman presented with a history of gradual weakness in all four extremities for several months. Based on electrophysiological studies, spinal magnetic resonance imaging and cerebrospinal fluid examination, inflammatory polyradiculopathy was confirmed. During a search for the aetiology, the patient was ultimately diagnosed with SS. This study introduces SS-associated polyradiculopathy that primarily presented with motor symptoms, thus mimicking motor neuron disease.

• KSBB Journal
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• v.28 no.5
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• pp.269-274
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• 2013

Motor neuron disease (MND) is a fatal neurodegenerative disorder caused by progressive and selective degeneration of motor neurons (MNs). Because of the versatile nature, stem cells have the potential to repair or replace the degenerated cells. In this review, we discussed stem cell based therapies including the use of embryonic stem cells (ESCs), neural stem cells (NSCs), induced pluripotent stem cells (iPSCs) and genetically engineered cells to produce the neurotrophic factors for the treatment of MND. To achieve this goal, the knowledge of specificity of the cell target, homing and special markers are required.

• Annals of Clinical Neurophysiology
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• v.18 no.1
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• pp.18-20
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• 2016

Amyotrophic lateral sclerosis (ALS) is a progressive motor neuron degenerative disease that clinically manifests both upper and lower motor neuron signs. However, it is unknown where and how the motor neuron degeneration begins, and conflicting hypotheses have been suggested. Recent advanced radiological techniques enable us to look into ALS neuropathology in vivo. Herein, we report a case with upper motor neuron-predominant ALS in whom the results of brain magnetic resonance imaging (MRI) and myelin water fraction MRI suggest axonal degeneration.

• Journal of Korean Neurosurgical Society
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• v.56 no.3
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• pp.254-256
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• 2014

Weakness of the dorsiflexor muscles of the ankle or toe, referred to as foot drop, is a relatively common presentation. In most cases, foot drop is caused by a lower motor neuron disease such as peroneal peripheral neuropathy, L4-5 radiculopathic sciatic neuropathy, or polyneuropathy. Although upper motor neuron lesions can present as foot drop, the incidence is very rare. Here, we report an extremely rare case in which foot drop was the only presenting symptom of cerebral infarction.

• Annals of Clinical Neurophysiology
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• v.5 no.1
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• pp.21-26
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• 2003

Background & Objectives: Hyperexcitablity of motor system is a well-established characteristic pathophysiologic finding of amyotrophic lateral sclerosis (ALS). Whereas little is known about the source of excitability according to the progression of the disease. We evaluated the excitability and its source in advanced ALS patients using transcranial magnetic stimulation (TMS). Meterial & Methods: Motor evoked potentials (MEP) by TMS were recorded for abductor pollicis brevis muscles in 20 patients, 11 men and 9 women, with ALS. Mean age was $54.2{\pm}12.1years$, and mean disease duration was $13.9{\pm}13.4years$. Serial magnetic stimulations were applied to get the parameters; excitability threshold (ET), amplitude and latency of MEP. We also had a facilitated MEP (fMEP). Results: The parameters were analyzed according to the clinical settings. ET was higher in ALS(mean $63.5{\pm}18.1$) than normal control (mean $46.0{\pm}8.4$, p<0.01). Amplitudes of MEP were reduced in ALS ($2.6{\pm}3.6mV$; control $6.5{\pm}3.1mV$, p<0.01). Duration of the disease and ET showed significant inverse correlation (Spearson correlation coefficient = -0.57, p<0.01). Duration of the disease and fMEP/MEP ratio showed less but also significant inverse correlation (Spearson correlation coefficient, r = -0.52, p < 0.05). Conclusions: Lower ET in advanced ALS patients, in spite of decreased fMEP/MEP ratio, may indicate the hyperexcitability of lower motor neurons in these patients. This study suggests that lower motor neurons is hyperexcitable due to upper motor neuron dysfunction at advanced stage.

• Journal of the Korean Academy of Clinical Electrophysiology
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• v.4 no.1
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• pp.39-47
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• 2006

Functional Electrical Stimulation(FES) cause paralysed muscles to contract in some clinical circumstances. Generally, FES has been thought of as a valuable tool in activating any skeletal muscle paralysed as a result of upper motor neuron damage. But, the function of cardiac and smooth muscle is also affected by upper motor neuron damage. Today, various applications of FES are investigated, including conditioning cardiovascular exercise, caugh and breathing assistant, improving bowel and bladder control, hand grasp, standing and walking etc. This review will focus on the literature reporting application of FES to control respiratory capabilities and internal organ function as well as increase muscular strength, hand grasp, standing and walking in patients with upper motor diseases.

• 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.