• Molecules and Cells
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• v.45 no.4
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• pp.231-242
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• 2022

The neuromuscular junction (NMJ), which is a synapse for signal transmission from motor neurons to muscle cells, has emerged as an important region because of its association with several peripheral neuropathies. In particular, mutations in GARS that affect the formation of NMJ result in Charcot-Marie-Tooth disease and distal hereditary motor neuropathy. These disorders are mainly considered to be caused by neuronal axon abnormalities; however, no treatment is currently available. Therefore, in order to determine whether the NMJ could be targeted to treat neurodegenerative disorders, we investigated the NMJ recovery effect of HDAC6 inhibitors, which have been used in the treatment of several peripheral neuropathies. In the present study, we demonstrated that HDAC6 inhibition was sufficient to enhance movement by restoring NMJ impairments observed in a zebrafish disease model. We found that CKD-504, a novel HDAC6 inhibitor, was effective in repairing NMJ defects, suggesting that treatment of neurodegenerative diseases via NMJ targeting is possible.

• Journal of Yeungnam Medical Science
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• v.40 no.4
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• pp.328-334
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• 2023

An aging population and changes in dietary habits have increased the incidence of diabetes, resulting in complications such as diabetic foot ulcers (DFUs). DFUs can lead to serious disabilities, substantial reductions in patient quality of life, and high financial costs for society. By understanding the etiology and pathophysiology of DFUs, their occurrence can be prevented and managed more effectively. The pathophysiology of DFUs involves metabolic dysfunction, diabetic immunopathy, diabetic neuropathy, and angiopathy. The processes by which hyperglycemia causes peripheral nerve damage are related to adenosine triphosphate deficiency, the polyol pathway, oxidative stress, protein kinase C activity, and proinflammatory processes. In the context of hyperglycemia, the suppression of endothelial nitric oxide production leads to microcirculation atherosclerosis, heightened inflammation, and abnormal intimal growth. Diabetic neuropathy involves sensory, motor, and autonomic neuropathies. The interaction between these neuropathies forms a callus that leads to subcutaneous hemorrhage and skin ulcers. Hyperglycemia causes peripheral vascular changes that result in endothelial cell dysfunction and decreased vasodilator secretion, leading to ischemia. The interplay among these four preceding pathophysiological factors fosters the development and progression of infections in individuals with diabetes. Charcot neuroarthropathy is a chronic and progressive degenerative arthropathy characterized by heightened blood flow, increased calcium dissolution, and repeated minor trauma to insensate joints. Directly and comprehensively addressing the pathogenesis of DFUs could pave the way for the development of innovative treatment approaches with the potential to avoid the most serious complications, including major amputations.

• Journal of the Korean Society of Radiology
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• v.81 no.1
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• pp.81-100
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• 2020

Magnetic resonance neurography (MRN) has been increasingly used in recent years for the assessment of peripheral neuropathies. Fat suppression T2-weighted imaging (T2WI) and diffusion-weighted imaging (DWI) have typically been used to provide high contrast MRN. Isotropic 3-dimensional (3D) sequences with fast spin echo, post-processing imaging techniques, and fast imaging methods, among others, allow good visualization of peripheral nerves that have a small diameter, complex anatomy, and oblique course within a reasonable scan time. However, there are still several issues when performing high contrast and high resolution MRN including standard sequence; fat saturation techniques; balance between resolution, field of view, and slice thickness; post-processing techniques; 2D vs. 3D image acquisition; different T2 contrasts between proximal and distal nerves; high T2 signal intensity of adjacent veins or joint fluid; geometric distortion; and appropriate p-values on DWI. The proper understanding of these issues will help novice radiologists evaluate peripheral neuropathies using MRN.

• Journal of Korean Academy of Fundamentals of Nursing
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• v.13 no.2
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• pp.225-234
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• 2006

Purpose: This study was done to investigate the effect of self-foot reflexology on peripheral blood circulation, peripheral neuropathy and to determine the feasibility of self-foot reflexology as a nursing intervention. Method: This was nonequivalent control pretest-posttest study with 76 patients with type 2 diabetes mellitus (ages between 40-79) recruited from public health centers in Busan city. Intervention was a 6 week self-foot reflexology, and outcome variables were peripheral blood circulation and peripheral neuropathy(tactile response to monofilament, intensity of symptoms of peripheral neuropathy). ANCOVA was used to do the statistical analysis. A.05 significance level was set for evaluating the effects of self-foot reflexology. Results: The self-foot reflexology was relatively effective not only in reducing peripheral neuropathy(especially tingling sensation and pain) but also in improving ability to sense the 10-g force monofilament. Conclusion: Even though self-foot reflexology was not effective in improving peripheral circulation, it had good effect on improving peripheral neuropathy. Therefore self-foot reflexology can be used as a nursing intervention program for promoting foot care for patients with DM patients.

• Annals of Clinical Neurophysiology
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• v.6 no.1
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• pp.48-51
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• 2004

The occurrence of polyneuropathy in association with monoclonal gammopathy of undetermined significance (MGUS) is quite common. However, reports of MGUS associated cranial neuropathies are rare. A 63 year-old women was presented with diplopia and swallowing difficulty. Neurological examination showed limitation of abduction of right eye, right peripheral facial palsy, decreased hearing and gag reflex, left side deviation of uvula, and decreased DTR. Sensorimotor polyneuropathy were observed with elctrophysiological studies. Protein and immunoelectrophoresis revealed IgG ${\kappa}$monoclonal gammopathy. She was treated with intravenous immunoglobulin and steroid, and her symptoms and signs were improved. This case suggested that she had sensorimotor polyneuropathy and multiple cranial neuropathies associated with IgG ${\kappa}$MGUS.

• Annals of Clinical Neurophysiology
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• v.19 no.2
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• pp.79-92
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• 2017

Paraproteinemia is caused by a proliferation of monoclonal plasma cells or B lymphocytes. Approximately 10% of idiopathic neuropathies are associated with paraproteinemia, where a certain paraprotein acts like an antibody targeted at constituents of myelin or axolemma in peripheral nerves. The relationship between paraproteinemia and peripheral neuropathy remains unclear despite this being of interest for a long time. Neurologists frequently find paraproteinemia during laboratory examinations of patients presenting with peripheral neuropathy, especially in the elderly. The possibility of a relationship with paraproteinemia should be considered in cases without an explainable cause. We review the causal association between paraproteinemia and neuropathy as well as clinical, laboratory, and electrophysiologic features, and the treatment options for paraproteinemic neuropathy.

• 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.

• Korean Journal of Adult Nursing
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• v.28 no.3
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• pp.343-353
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• 2016

Purpose: This study aimed to identify the levels of oxaliplatin-induced peripheral neuropathy (OXLIPN) and the quality of life (QOL) related to OXLIPN in patients with digestive system cancer. Methods: A total of 83 patients with chemotherapy-induced peripheral neuropathy (CIPN)-related symptoms participated in this study. Data were collected through self-reported questionnaire which were constructed to include general and clinical characteristics, EORTC QLQ-C30, Patient Neurotoxicity Questionnaire (PNQ), and EORTC QLQ-CIPN20. Results: The average scores of OXLIPN upper and lower extremity scale were 30.01 and 29.16, respectively. The average scores of PNQ sensory and motor scale were 2.11 and 1.70, respectively. The mean score of the QLQ-C30 global health status was 54.85, and the range of mean score of the functional and symptom subdomains was 34.85~73.29 and 17.67~53.54, respectively. The CIPN-related symptoms positively correlated with the global health status scale and all subdomains of functional scale, respectively and negatively correlated with fatigue, pain, dyspnea, insomnia, and financial problem subdomains of the symptom scale, respectively. Conclusion: Oncology nurses should pay attention and provide remedies for CIPN symptoms reported by their patients. Nursing interventions should be developed for patients with digestive system cancer to alleviate CIPN and enhance their QOL.

• Annals of Clinical Neurophysiology
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• v.22 no.1
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• pp.13-18
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• 2020

The electrodiagnostic findings in Guillain-Barré syndrome (GBS) play important roles in both understanding its pathophysiology and its diagnosis. Only demyelinating neuropathies were thought to be present when GBS patients were first diagnosed in Western countries, but the concept changed when many axonal GBS patients were reported in Asia. Reversible conduction failure was subsequently revealed, and it was recognized as a pathophysiologic continuum of axonal GBS. Thus, the electrodiagnostic findings in GBS have had a profound effect on the history of this disease.

• Molecules and Cells
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• v.23 no.1
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• pp.39-48
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• 2007

Charcot-Marie-Tooth (CMT) disease and hereditary neuropathy with liability to pressure palsies (HNPP) are frequent forms of genetically heterogeneous peripheral neuropathies. Reciprocal unequal crossover between flanking CMT1A-REPs on chromosome 17p11.2-p12 is a major cause of CMT type 1A (CMT1A) and HNPP. The importance of a sensitive and rapid method for identifying the CMT1A duplication and HNPP deletion is being emphasized. In the present study, we established a molecular diagnostic method for the CMT1A duplication and HNPP deletion based on hexaplex PCR of 6 microsatellite markers (D17S921, D17S9B, D17S9A, D17S918, D17S4A and D17S2230). The method is highly time-, cost- and sample-saving because the six markers are amplified by a single PCR reaction and resolved with a single capillary in 3 h. Several statistical and forensic estimates indicated that most of these markers are likely to be useful for diagnosing the peripheral neuropathies. Reproducibility, as determined by concordance between independent tests, was estimated to be 100%. The likelihood that genotypes of all six markers are homozygous in randomly selected individuals was calculated to be $1.6{\times}10^{-4}$, which indicates that the statistical error rate for this diagnosis of HNPP deletion is only 0.016%.