• Journal of Genetic Medicine
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• v.18 no.2
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• pp.83-93
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• 2021

Charcot-Marie-Tooth disease (CMT) is the most common hereditary motor and sensory peripheral neuropathy. CMT is usually classified into two categories based on pathology: demyelinating CMT type 1 (CMT1) and axonal CMT type 2 (CMT2) neuropathy. CMT1 can be distinguished by assessing the median motor nerve conduction velocity as greater than 38 m/s. The main clinical features of axonal CMT2 neuropathy are distal muscle weakness and loss of sensory and areflexia. In addition, they showed unusual clinical features, including delayed development, hearing loss, pyramidal signs, vocal cord paralysis, optic atrophy, and abnormal pupillary reactions. Recently, customized treatments for genetic diseases have been developed, and pregnancy diagnosis can enable the birth of a normal child when the causative gene mutation is found in CMT2. Therefore, accurate diagnosis based on genotype/phenotypic correlations is becoming more important. In this review, we describe the latest findings on the phenotypic characteristics of axonal CMT2 neuropathy. We hope that this review will be useful for clinicians in regard to the diagnosis and treatment of CMT.

• Journal of Acupuncture Research
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• v.30 no.5
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• pp.155-167
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• 2013

Objectives : This research was conducted to investigate the factors that affect the level of facial nerve damage. Methods : From October 2009 to September 2013, the total number of 581 patients of Bell's palsy and Ramsay-Hunt syndrome visited Facial Palsy Center in Kyung Hee University Hospital at Gangdong for Traditional Korean and Western combined medical treatment. Of these, 453 patients of peripheral facial nerve palsy were selected for the research. After reviewing the medical records that have details of age, gender, diagnosis(Bell's palsy and Ramsay-Hunt syndrome), onset, underlying diseases(DM, HTN), and HbAlc value, the analysis on the influence factors on the level of facial nerve damage was drew out. Results : The axonal loss rate of oris branch and nasal branch were significantly higher than the axonal loss rate of frontal branch and oculi branch. In addition, the frequency of becoming a major damaged branch was also high in the oris branch nasal branch. The factors by month, weather, smoking, and alcohol did not influence EMG axonal loss rate. Male rather than female and patient with Rasmay-Hut syndrome rather than Bell's palsy had a higher axonal loss rate in all branches. Of those, front of branch of male was remarkably higher than female. Patient with DM as P/H had high axonal loss rate in all branches. Patient with HTN as P/H had high axonal loss rate in all branches except for oris branches. Patients with DM and HTN group had significantly higher value from the average of axonal loss rate than patients who are only with HTN and without DM/HTN. DM alone group had significantly higher value than patients who are without DM/HTN. However, HTN alone was not significantly high. By analysing HbAlc of the patients who were hospitalised regardless DM, axonal loss rate was high in the order of DM group, preDM group, normal group. Nevertheless, only DM group showed higher axonal rate statistically than normal group. Considering DM and HbA1c value, the patients can be divided into 4 different groups of hkDM, lkDM, hfDM and nDM. By analysing those groups, the average damaged value of the groups with diagnosis followed by treatment(lkDM, hkDM) were higher than the average rate of hfDM and statistically higher than the rate of the nDM. Conclusions : The influential factors of increasing the level of EMG damage are male(only for the frontal branch), age above sixties, HTN, DM, and HbAlc value above 6.5. Besides, the negligible factors are month, season, diagnosis, alcohol, and smoking. Further research including clinical prognosis should be conducted.

• Journal of Acupuncture Research
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• v.25 no.2
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• pp.189-197
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• 2008

Objectives : This study was performed in order to investigate the effectiveness of electromyography and the Heart Rate Variability(HRV) test as prognosis factors, and to clarify correlation between Electromyography and the Heart Rate Variability test. Methods : 44 Bell's palsy patients who were graded V on the House-Brackmann scale and underwent HRV and EMG testing were retrospectively reviewed based on medical records. Results from both tests were analyzed via simple linear regression, and bivariate correlation analysis was performed to investigate the correlation between results from the two tests. The severity of the facial palsy at onset and at 2 weeks after treatment were evaluated with the H-B grade and Yanagihara grading system, and was converted into improvement scores. Results : Mean axonal loss according to electromyography showed a statistically significant correlation in predicting peripheral facial palsy improvement(p<0.01). HR, SDNN, TP, LF, HF, VLF, and LF/HF ratio on the Heart Rate Variability test showed no significant correlation in predicting peripheral facial palsy improvement. Mean axonal loss determined by electromyography, and HR, SDNN, TP, LF, HF, VLF, and LF/HF ratio recorded with the Heart Rate Variability test was analyzed with the bivariate correlation analysis method. Mean axonal loss and SDNN showed a statistically significant correlation(p<0.01) Conclusions : The Heart Rate Variability test has no statistical significance in predicting peripheral facial palsy improvement. SDNN has a statistically significant correlation with mean axonal loss as determined by electromyography.

• Journal of Acupuncture Research
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• v.26 no.1
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• pp.29-37
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• 2009

Objectives : This study was performed to categorize and define causes of recurrent peripheral facial nerve palsy. Methods : 54 patients was identified with recurrent peripheral facial nerve palsy among 726 patients who visited the Facial Palsy Center in East-West Neo Medical Center between May 2006 and August 2008. We reviewed the medical records including gender, age, laterality, number of recurrence, primary onset age, interval between recurrences, accompanied disease(e.g. DM, HTN), and axonal loss. Results : Patients whose primary palsy onset was before their second decade had a higher possibility of recurrence and tended to recur more than twice. Double-episode ipsilaterally recurrent group showed definitely worse result of axonal loss compared with non-recurrent group and single-episode ipsilaterally recurrent group. But There was no statistically significant difference between mean axonal loss of the non-recurrent group and single-episode ipsilaterally recurrent group. Conclusions : This study was designed for 54 patients and further studies are necessary.

• Journal of Acupuncture Research
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• v.26 no.6
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• pp.111-119
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• 2009

Objectives : To assess the characteristics of electropyography(EMG) and heart rate variability(HRV) values in idiopathic facial palsy inpatients of different Sasang constitutions. Methods : Medical records of 103 idiopathic facial palsy inpatients who underwent facial EMG testing and HRV testing were retrospectively reviewed. All subjects had been consulted to the department of Sasang constitution for constitution differentiation. The Sasang constitutional and age distribution were initially analyzed, and axonal loss percentage values on EMG were correlated with Sasang constitution and sex. HRV results were also correlated with Sasang constitution and sex. Results : 1. 24.4 percent of idiopathic facial palsy inpatients were of the Soyang constitution, 52.4 percent were of the Taeum, and 23.3 percent were of the Soeum. Patients in their fifties were most common in all constitutions(27.8%). 2. Mean axonal loss values for the temporal, zygomatic, and buccal branches were all statistically significantly lower in the Taeum constitution compared with Soyang or Soeum(p<0.05). 3. Mean axonal loss values for the temporal, zygomatic, and buccal branches were all statistically significantly lower in men than women(p<0.05). 4. The mean LF/HF ratio was significantly lower in idiopathic facial palsy inpatients of the Soeum constitution compared with Soyang and Taeum(p<0.05), but there was no statistically significant difference in mean MHRT, SDNN, TP, VLF, LF, and HF of different constitutions. 5. The mean SDNN was significantly lower in women inpatients compared with men(p<0.05), but there was no statistically significant difference in mean MHRT, TP, VLF, LF, HF, and LF/HF ratio. Conclusions : The Sasang constitutional composition of idiopathic facial palsy inpatients closely adheres to the composition proposed in Dong-eui-su-se-bo-won, but the Taeum and Soeum proportion is slightly larger and the Soyang proportion slightly smaller. Axonal loss value on EMG was significantly lower in the Taeum constitution, and in men(p<0.05, <0.05). Mean LF/HF ratio was significantly lower in the Soeum constitution compared with Soyang and Taeum(p<0.05), and mean SDNN was significantly lower in women(p<0.05).

• The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
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• v.24 no.3
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• pp.55-64
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• 2011

Objectives : This study was performed in order to investigate the effectiveness of electroneuronography as predicting factors for peripheral facial palsy prognosis. Methods : Data were gathered with 32 Bell's palsy patients. The grade of Bell's palsy were measured 2 times; first medical exam and 4 weeks after treatment, with Lucille Daniels's Muscle test. We converted the grade system use on five rating scale. The significance of treatment verified with paired t-test used on first medical exam and 4 weeks after treatment score and predicting factors of electroneuronography verified with simple regression test. Results : The improvement score were statistically significant different before and after treatment(p<.001). Mean axonal loss according to electroneuronography showed a statistically significant correlation in predicting peripheral facial palsy (P<0.01). Conclusion : Axonal loss as determined by electroneuronography has statistical significance as predicting factors for peripheral facial palsy prognosis.

• Journal of Acupuncture Research
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• v.30 no.4
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• pp.115-123
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• 2013

Objectives : This study was performed to confirm the usefulness of EMG and early-performed ENoG as predicting factor for facial palsy by clinical outcome. Methods : We gathered patients who visited the Facial Palsy Center in Kyung Hee University Hospital at Gangdong between January 2010 and September 2012. We used inclusion/exclusion criteria and reviewed 231 patients' medical records including gender, age, HB grade and EMG & ENoG axonal loss. We compared the relation between EMG axonal loss and improvement degree at 4 & 6 weeks after treatment, and between ENoG axonal loss at 3-5 & 6-8 days after onset and EMG axnoal loss using regression analysis. Results : Each analysis had statistical significance, but EMG & improvement degree at 6 weeks after treatment showed statistically higher correlation than that at 4 weeks after treatment, and ENoG at 6~8 days after onset & EMG showed statistically higher correlation than that at 3~5 day after onset. Conclusions : We could get the evidence of the validity of EMG and early-performed ENoG as predicting factor for facial palsy based on collaborative treatment between Korean and western medicine.

• Journal of Acupuncture Research
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• v.33 no.2
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• pp.51-60
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• 2016

Objectives : Although integrative Korean medical treatments have been suggested to be effective for treating Bell's palsy, the effect of Korean medical treatment according to when treatment was started is unknown in the clinical field. Therefore, this study was conducted to determine the results of treating Bell's palsy according to different starting points of integrative Korean medical treatments. Methods : We screened patients who visited the Facial Palsy Center at Kyung Hee University Hospital for Korean and Western combined medical treatment from March 2011 to February 2016. A total of 821 out of 2086 patients were studied, including their basic characteristics and results of an electromyography. Patients who started Korean medical treatment within 3 days of onset were placed in group A, within 9 days of onset in group B, and patients who started treatment after 10 days of onset were placed in group C. We tried to compare the level of facial nerve damage by electromyography between groups. Results : The patients in group C had the highest axonal loss rates in all branches(frontal, oculi, nasal, oris). The post hoc analyses revealed the difference of axonal loss rates between group A and B was not statistically significant. Only group C showed statistically higher axonal loss rates in all branches. Conclusion : The study results showed that the patients who had delayed Korean medical treatments had a higher level of facial nerve damage by electromyography. According to the results of this study, early application of integrative Korean medical treatment is suggested.

• Toxicological Research
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• v.13 no.4
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• pp.317-322
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• 1997

Acute delayed neurotoxicity of KH-502 [O.O-Diethyl O-(1-phenyyl-3-trifluoromethyl-5-pyrazoyl) thiophosphoric acid ester], an insecticide synthesized newly in Korea, was studied in White Leghorn hens. The doses were determined on the basis of preliminary $LD_{50}$ study. High, middle and low doses were determined to be 1123 mg/kg, 762 mg/kg and 518 mg/kg, respectively. The animals were pretreated with atropine (30 mg/kg) prior to administration of KH-502. The chemical was administrated at the first and 21st day of the study. As positive controls, animals were admlnistrated with triorthocresylphosphate (TOCP 1000 mg/kg and 500 mg/kg). Animals administrated with TOCP or KH-502 were sacrificed by perfusion-fixation at 21st and 42nd day of the study, respectively. The central and peripheral nerve tissues were routinely treated for microscopic observation. As results, eight, three, one, and one chickens died within 2 day after adminiatration with signs of cholinergic acute toxicity in high, middle low and TOCP dose-group (500 mg/kg), respectively. No abnormal clinical signs were observed in the survived chickens administrated with KH-502 in the duration of the study. The chickens in positive control groups showed ataxia and incoordination at the 14th day after administration of TOCP. From necropsy, macroscopic changes were not observed in all groups including positive control groups. Histopathologically, oxonal swelling with myelin loss, focal gliosis, distention around axonal space were observed in the spinal cords of the chickens administrated with TOCP 1000 mg/kg. The lesions were distinct in the dorsal and lateral funiculi of cervical spinal cord, in the lateral and ventral funiculi of thoracic spinal cord and in ventral funiculi of lumbosacral spinal cord. Axonal swelling and mlcrogliosis were infrequently observed in the chickens of other groups including negative control one. However, they were nonspecifically distributed in the spinal cords. In this study, we concluded that the new chemical, KH-502 did not have acute delayed neurotoxicity in White Leghorn hens.

• Journal of Acupuncture Research
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• v.29 no.6
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• pp.23-34
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• 2012

Objectives : This study was performed to define clinical character of peripheral facial nerve injury. Methods : 36 patients was identified with peripheral facial nerve injury among 1128 patients who visited the Facial Palsy Center in Kyung Hee University Hospital at Gang-dong between January 2010 and November 2011. We reviewed the medical records including gender, age, cause, symptom, period of treatment, and axonal loss. Results : Most common cause of peripheral facial nerve injury was iatrogenic surgery, followed by direct trauma, neoplastic disorders. Patients with facial nerve injury commonly complain about facial palsy(ipsilateal or bilateral), followed by paresthesia, facial spasm, facial pain, auricular pain. Peripheral facial nerve injury group showed worse electrophysiological pattern and younger onset age compared with Bell's palsy group. Conclusion : This study was designed for 36 patients and further studies are necessary.