• Journal of Medicine and Life Science
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• v.21 no.1
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• pp.11-14
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• 2024

Excess of pyridoxine, in contrast to other nutrients, may result in neuropathy. Case reports are sparse, and little is known about the clinical and electrophysiological findings. Eight patients with pyridoxine-induced neuropathy were investigated, and a review of the literature was undertaken. Nerve conduction studies showed axonal sensory or sensorimotor polyneuropathy. And the blood levels of vitamin B6 were markedly elevated. After discontinuation of vitamin supplements, all patients showed no significant improvement in clinical and electrophysiological findings. Supplementation with pyridoxine at doses greater than 50 mg/day for extended durations may be harmful and should be discouraged.

• The Journal of the Korean dental association
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• v.38 no.4 s.371
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• pp.378-384
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• 2000

• Annals of Clinical Neurophysiology
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• v.13 no.1
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• pp.44-47
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• 2011

Background: Miller-Fisher syndrome (MFS) is characterized by the clinical triad of ophthalmoplegia, ataxia, and areflexia, and is considered a variant form of Guillain-Barre syndrome. Although some cases of delayed-onset facial palsy in MFS have been reported, the characteristics of this facial palsy are poorly described in the literature. Methods: Between 2007 and 2010, six patients with MFS were seen at our hospital. Delayed facial palsy, defined as a facial palsy that developed while the other symptoms of MFS began to improve following intravenous immunoglobulin treatment, was confirmed in four patients. The clinical and electrophysiological characteristics of delayed facial palsy in MFS, as observed in these patients, are described here. Results: Four patients with delayed-onset facial palsy were included. Delayed facial palsy developed 8-16 days after initial symptom onset (5-9 days after treatment). Unilateral facial palsy occurred in three patients and asymmetric facial diplegia in one patient. The House-Brackmann score of facial palsy was grade III in one patient, IV in two patients, and V in one patient. None of the patients complained of posterior auricular pain. Facial nerve conduction studies revealed normal amplitude in all four patients. The blink reflex showed abnormal prolongation in two patients and the absence of action potential formation in two patients. Facial palsy resolved completely in all four patients within 3 months. Conclusions: Delayed facial palsy is a frequent symptom in MFS and resolves completely without additional treatment. Thus, standard treatment and patient reassurance are sufficient in most cases.

• The Korean Journal of Physiology and Pharmacology
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• v.21 no.4
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• pp.439-447
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• 2017

Myotonia congenita (MC) is a genetic disease that displays impaired relaxation of skeletal muscle and muscle hypertrophy. This disease is mainly caused by mutations of CLCN1 that encodes human skeletal muscle chloride channel (CLC-1). CLC-1 is a voltage gated chloride channel that activates upon depolarizing potentials and play a major role in stabilization of resting membrane potentials in skeletal muscle. In this study, we report 4 unrelated Korean patients diagnosed with myotonia congenita and their clinical features. Sequence analysis of all coding regions of the patients was performed and mutation, R47W and A298T, was commonly identified. The patients commonly displayed transient muscle weakness and only one patient was diagnosed with autosomal dominant type of myotonia congenita. To investigate the pathological role of the mutation, electrophysiological analysis was also performed in HEK 293 cells transiently expressing homo-or heterodimeric mutant channels. The mutant channels displayed reduced chloride current density and altered channel gating. However, the effect of A298T on channel gating was reduced with the presence of R47W in the same allele. This analysis suggests that impaired CLC-1 channel function can cause myotonia congenita and that R47W has a protective effect on A298T in relation to channel gating. Our results provide clinical features of Korean myotonia congenita patients who have the heterozygous mutation and reveal underlying pathophyological consequences of the mutants by taking electrophysiological approach.

• Clinical and Experimental Pediatrics
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• v.54 no.12
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• pp.507-511
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• 2011

Purpose: The aim of the present study was to evaluate the characteristics of electrophysiologic studies (EPS) and radiofrequency ablation (RFA) performed in subjects aged less than 30 years with Wolff-Parkinson-White (WPW) syndrome, particularly pediatric patients under 18 years of age, based on our experience. Methods: Two hundred and one consecutive patients with WPW syndrome were recruited and divided to 3 groups according to age: group 1, 6 to 17 years; group 2, 18 to 29 years; and group 3, 30 to 60 years. The clinical, electrophysiological, and therapeutic data for these patients were evaluated by a retrospective medical record review. Results: A total of 73 (36%) of these patients were <30 years of age. Although there were more males than females in group 2 (male:female, 31:11), there was no sex difference in group 1 (male:female, 16:15). Left accessory pathway was detected less frequently in group 1 (32%, 10/31) than in group 2 (57%, 24/42) and group 3 (63%, 81/128) (P=0.023 and P=0.002, respectively). Conclusion: The present study describes several different electrophysiological characteristics in children and adolescents with WPW syndrome. Therefore, when EPS and RFA are performed in children and adolescence with WPW syndrome, we recommend that these characteristics be considered.

• The Journal of the Korea Contents Association
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• v.10 no.10
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• pp.237-245
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• 2010

Nerve conduction studies (NCS) are the most objective measure of nerve function and essential for the diagnosis of sub-clinical neuropathy in diabetes mellitus and diabetic polyneuropathy (DPN). This study evaluates the characteristic of electrophysiological abnormalities in DPN. Electrodiagnostic data from 120 patients with diabetic polyneuropathies and 77 control subjects were reviewed. Motor nerve conduction velocities (MNCV), distal motor latencies (DML), compound muscle action potential (CMAP) amplitudes, No potential frequency and conduction block were analyzed. Data were normalized based on normative reference values, and the proportion of nerves with abnormal values in the lower and upper limbs were evaluated. DPN was systemic demyelinating peripheral polyneuropathy and more severe abnormal nerve conduction was found in lower limbs than in upper limbs. The abnormal degree was more severe in peroneal nerve. It was no statistically significant difference of conduction block in control and DPN group. Our findings suggest that DPN had more common and severe peroneal nerve involvement in the motor nerve conduction studies (MNCS). These findings have important implications for the electrophysiological evaluation of DPN.

• The Korean Journal of Physiology and Pharmacology
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• v.4 no.3
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• pp.169-175
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• 2000

To learn the developmental changes in intrinsic electrophysiological properties of the second order taste neurons, whole cell recordings from the developing nucleus of the solitary tract neurons were done in brainstem slices of postnatal rats. Rats aged from postnatal 0 to 21 days (P0-P21) were used, being divided into 3 age groups: postnatal first week (P0-P7 days), second week (P8-P14 days), and third week (P15-P21 days). Slices containing gustatory NTS were cut horizontally in the thickness of $300\;{\mu}m.$ Whole cell recordings were obtained from neurons in response to a series of hyperpolarizing and depolarizing current pulses. The intrinsic electrophysiological properties of the rostral NTS (rNTS) neurons were compared among the age groups. Depolarizing current pulses evoked a train of action potentials in all neurons of all age groups. The resting membrane potential and input resistance of the neurons did not show any significant differences during the postnatal 3 weeks. The time constant, however, decreased during the development. Duration of action potential measured at half maximum amplitude was longer in younger age groups. Both the maximum rate of rise and the maximum rate of fall in the action potential increased during the first 3 weeks postnatal. Electrophysiologically more than half neurons were type III. In summary, it is suggested that developmental changes in electrophysiological properties in rNTS occur during the first three weeks in rats.

• The Korean Journal of Physiology and Pharmacology
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• v.19 no.6
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• pp.533-542
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• 2015

Little human tissue data are available for slow waves and migrating motor complexes, which are the main components of small bowel motility. We investigated the electrophysiological and mechanical characteristics of human ileal motility, in vitro. Ileum was obtained from patients undergoing bowel resection. Electrophysiological microelectrode recordings for membrane potential changes and mechanical tension recordings for contraction from smooth muscle strips and ileal segments were performed. Drugs affecting the enteric nervous system were applied to measure the changes in activity. Slow waves were detected with a frequency of 9~10/min. There were no cross-sectional differences in resting membrane potential (RMP), amplitude or frequency between outer and inner circular muscle (CM), suggesting that electrical activities could be effectively transmitted from outer to inner CM. The presence of the interstitial cell of Cajal (ICC) at the linia septa was verified by immunohistochemistry. Contractions of strips and segments occurred at a frequency of 3~4/min and 1~2/min, respectively. The frequency, amplitude and area under the curve were similar between CM and LM. In segments, contractions of CM were associated with LM, but propagation varied with antegrade and retrograde directions. Atropine, $N^W$-oxide-L-arginine, and sodium nitroprusside exhibited different effects on RMP and contractions. There were no cross-sectional differences with regard to the characteristics of slow waves in CM. The frequency of contractions in smooth muscle strips and ileal segments was lower than slow waves. The directions of propagation were diverse, indicating both mixing and transport functions of the ileum.

• Annals of Clinical Neurophysiology
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• v.4 no.1
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• pp.34-37
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• 2002

Background : The term "mononeuropathy multiplex" means simultaneous or sequential involvement of individual noncontiguous nerve trunks, evolving over days to years. The aim of this study was to delineate the causes, clinical features, and detailed electrophysiological findings in the patients with mononeuropathy multiplex. Methods : We analyzed the medical records of 22 patients with mononeuropathy multiplex confirmed on electrophysiological studies in Inje University Seoul Paik Hospital, Seoul Municipal Boramae Hospital, and Seoul National University Hospital between 1991 to 2000. Results : The number of male and female patients was equal. The mean age was 48 years with a peak incidence in the sixth decade. The etiology could be divided into vasculitis(11 patients) or non-vasculitis group. In vasculitis group, Churg-Strauss syndrome, polyarteritis nodosa, and rheumatoid arthritis were included. The non-vasculitis group included diabetes mellitus, leprosy, and Guillain-Barre syndrome. Ulnar and median nerves were most commonly involved(91%). In descending order of frequency, peroneal, posterior tibial, sural, and radial nerves were also involved. Bilateral involvement occurred most commonly in ulnar nerve. The symptoms and signs of mononeuropathy multiplex were the initial manifestations in 12 patients(55%), which was more frequent in vasculitis group(73%). Nerve conduction abnormalities could be divided into axonal, demyelinating, or mixed type. Most(91%) of the patients in vasculitis group revealed axonal type abnormalities. The location of the nerve lesion was frequently related to potential site of entrapment in demyelinating type. Conclusions : Mononeuropathy multiplex is the presenting features of the etiological disease frequently, especially in vasculitis group. Nerve conduction studies(NCS) reveals not only axonal type but also demyelinating type abnormalities. The etiological diseases were different in each type. Therefore, NCS is very helpful for the early etiological diagnosis and therapeutic implication in the patients with mononeuropathy multiplex.

• Animal cells and systems
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• v.12 no.3
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• pp.137-141
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• 2008

Molecular cloning revealed the three isoforms($Ca_v3.1,\;Ca_v3.2,\;and\;Ca_v3.3$) of the T-type calcium channel subfamily. Expression studies exhibited their distinctive electrophysiological and pharmacological properties, accounting for diverse properties of T-type calcium channel currents previously characterized from isolated cells. However, electrophysiological properties of ion channels have shown to be more diversified by their splice variants. We here searched splice variants of rat $Ca_v3.1$ T-type channel by reverse-transcription-polymerase chain reaction(RT-PCR) to further explore diversity of $Ca_v3.1$. Interestingly, analyses of cloned RT-PCR products displayed that there were at least four splicing variants of rat $Ca_v3.1$ in the loop connecting repeats III and IV. Southern blot analyses indicated that the predominantly detected variant in brain was $Ca_v3.1a$(492 bp), which were rarely detected in most of peripheral tissues. Other two variants($Ca_v3.1c$, 546 bp; $Ca_v3.1d$, 525 bp) were detected in most of the tissues examined. The smallest isoform($Ca_v3.1b$, 471 bp) was rarely detected all the tissues. Electrophysiological characterization of the splicing variants indicated that the splice variants differ in inactivation kinetics and the voltage dependence of activation and inactivation as well.