• 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.

• Journal of International Academy of Physical Therapy Research
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• v.10 no.2
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• pp.1785-1790
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• 2019

Background: Lumbar disc herniation (LDH) causes neurological symptoms by compression of the dura mater and nerve roots. Due to the changed in proprioception inputs that can result in abnormal postural pattern, delayed reaction time, and changed in deep tendon reflex. Objective: To investigate the effects of lumbar stabilization exercises on motor neuron excitability and neurological symptoms in patients with LDH. Design: Randomized Controlled Trial (single blind) Methods: Thirty patients with LDH were recruited; they were randomly divided into the balance center stabilization resistance exercise group (n=15) and the Nordic walking group (n=15). Each group underwent their corresponding 20-minute intervention once a day, four times a week, for four weeks. Participants' motor neuron excitability and low back pain were assessed before and after the four-week intervention. Results: There were significant differences in all variables within each group (p<.05). There were significant differences between the experimental and control groups in the changes of upper motor neuron excitability and pain (p<.05), but not in the changes of lower motor neuron excitability and Korean Oswestry Disability Index. Conclusion: Lumbar stabilization exercises utilizing concurrent contraction of deep and superficial muscles improved low back function in patients with LDH by lowering upper motor neuron excitability than compared to exercises actively moving the limbs. Lumbar stabilization exercises without pain have a positive impact on improving motor neuron excitability.

• PNF and Movement
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• v.7 no.4
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• pp.45-53
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• 2009

Purpose : The purpose of this article was to review the literature on mirror neuron system with reference to its functional diversity in stroke rehabilitation.. Method : This review outlines scientific findings regarding different neurophysiological properties in mirror neurons, and discusses their involvement in process of stroke rehabilitation. Result & Conclusions : Mirror neurons were first discovered in macaque monkey. These neurons, like most neurons in F5 areas in premotor cortex, fired when an individual performs an action, as well as when he/she observes a similar action done by another individual, although originally fired only during action execution. Mirror neurons form a network for motor planning and initiating of motor action. Thus, in stroke rehabilitation based on the mirror neuron-action observation, motor imagery, observation with intent to imitate and imitation-may help activate mirror neuron system for improved outcome of physical therapy. These studies provide a scientific theoretical basis and discuss for the use of mirror neuron system as a complement to clinical physical therapy in stroke rehabilitation.

• Annals of Clinical Neurophysiology
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• v.8 no.1
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• pp.63-70
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• 2006

Background: Testosterone is reported to have neuroprotective effect in various neurological diseases. Recently, the mechanism involved in nitric oxide (NO)-mediated motor neuron death is under extensive investigation. The Cu/Zn-superoxide dismutase (SOD1) mutations has been implicated in selective motor neuron death of amyotrophic lateral sclerosis (ALS) and it is said to play an important role in NO-mediated motor neuron death. However, neuroprotective effect of testosterone on motor neuron exposed to NO has rarely been studied. Methods: Motor neuron-neuroblastoma hybrid cells expressing wild-type or mutant (G93A or A4V) SOD gene were treated with $200{\mu}M$ S-nitrosoglutathione. After 24 hr, cell viability was measured by MTT assay. To see the neuroprotective effect of testosterone, pretreatment with 1 nM testosterone was done 1 hr before S-nitroglutathione treatment. To study the mechanism of protective effect, $20{\mu}M$ flutamide (androgen receptor antagonist) was also pretreated with testosterone 1 hr before S-nitroglutathione treatment. Results: S-nitrosoglutathione showed significant neurotoxic effect in all three cell lines. Percentage of cell death was somewhat different in each cell line. 1 nM testosterone showed neuroprotective effect in G93A and wild-type cell line. In A4V cell line, testosterone did not showed neuroprotective effect. The neuroprotective effect of testosterone was reversed by $20{\mu}M$ flutamide. Conclusions: These results indicate that testosterone induces neuroprotection in NO-mediated motor neuron death directly through the androgen receptor. This neuroprotective effect of testosterone varies according to the types of SOD1 gene mutation. These data suggest that testosterone may be of therapeutic value against ALS.

• 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.

• Journal of the Korean Academy of Clinical Electrophysiology
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• v.2 no.3
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• pp.37-49
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• 2004

The purpose of this study was to determine the effect of spinal motor neuron excitability by cranial electrostimulation(CES). The fifteen Sparague-Dawley adult male rats were assigned to the three groups; GroupI(control), GroupII(low rate CES), GroupIII(high rate CES). Spinal motor neuron excitability was measured to use a computerized H reflex. The results of this study was as follows; M latency, M amplitude and H latency were no significant difference in all groups on repeated measured ANOVA(p>.05) but low rate CES and high rate CES groups were lower than ether group in comparative measurement of H amplitude and Hmax/Mmax ratio(p<.05). These results lead to the conclusion that spinal neuron excitability was influenced by CES. These results suggest that CES had the capability to lower spinal motor neuron excitability used synaptic blockade in spinal segment.

• 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.

• 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.

• 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.

• 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.