• Annals of Clinical Neurophysiology
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• 제2권2호
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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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• 제17권1호
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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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• 제11권2호
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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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• 제21권1호
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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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• 제28권5호
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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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• 제18권1호
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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.

• Biomolecules & Therapeutics
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• 제32권5호
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• pp.523-530
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• 2024

Parkinson's disease (PD) is a neurodegenerative disease caused by the death of dopaminergic neurons in the nigrostriatal pathway, leading to motor and non-motor dysfunctions, such as depression, olfactory dysfunction, and memory impairment. Although levodopa (L-dopa) has been the gold standard PD treatment for decades, it only relieves motor symptoms and has no effect on non-motor symptoms or disease progression. Prior studies have reported that 6-shogaol, the active ingredient in ginger, exerts a protective effect on dopaminergic neurons by suppressing neuroinflammation in PD mice. This study investigated whether cotreatment with 6-shogaol and L-dopa could attenuate both motor and non-motor symptoms and dopaminergic neuronal damage. Both 6-shogaol (20 mg/kg) and L-dopa (80 mg/kg) were orally administered to 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine/probenecid-induced PD model mice for 26 days. The experimental results showed that L-dopa alleviated motor symptoms, but had no significant effect on non-motor symptoms, loss of dopaminergic neuron, or neuroinflammation. However, when mice were treated with 6-shogaol alone or in combination with L-dopa, an amelioration in both motor and non-motor symptoms such as depression-like behavior, olfactory dysfunction and memory impairment was observed. Moreover, 6-shogaol-only or co-treatment of 6-shogaol with L-dopa protected dopaminergic neurons in the striatum and reduced neuroinflammation in the striatum and substantia nigra. Overall, these results suggest that 6-shogaol can effectively complement L-dopa by improving non-motor dysfunction and restoring dopaminergic neurons via suppressing neuroinflammation.

• Journal of Korean Neurosurgical Society
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• 제56권3호
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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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• 제5권1호
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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.

• 대한임상전기생리학회지
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• 제4권1호
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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.