• Journal of Korean Physical Therapy Science
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• v.3 no.1
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• pp.929-941
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• 1996

This study was conducted to determine the association between weighted needle pinprick sensory threshold(PPT) and sensory nerve conduction studies. The subjects were 53 healthy controls, 31 diabetic patients without peripheral neuropathic symptoms(DM) and 36 diabetic patients with peripheral neuropathic symptoms(DN). PPT was measured on the index and little fingers, bilaterally, as well as under the lateral malleolus, bilaterally. In electrophysiologic assessment the left and right median, ulnar and sural nerves were studied. Mean PPT in DN, DM and controls was high in turn on each sites tested. Age controlled PPT was significantly different among three groups on right little finger(p<0.05) and left malleolus(p<0.05), but on other sites, not statistically significantly different between DN and DM. The results were as follows: Sensory nerve conduction velocity and amplitude on each nerve tested were statistically significantly different among three groups(p<0.05). Correlation of PPT with sensory nerve conduction velocity and amplitude were statistically significant on each site and ranged from -0.4203(left malleolus) to -0.5649(right index finger) and from -0.3897(left index finger) to -0.6200(right index finger), respectively. When electrophysiological study is not feasible, measurement of PPT may be helpful for the assessment of peripheral sensory neurological function.

• Journal of Genetic Medicine
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• v.15 no.2
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• pp.107-109
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• 2018

X-linked Charcot-Marie-Tooth disease type 1 (CMTX1) is caused by the mutation in GJB1 gene, characterized by the transient central nervous system involvement and long standing peripheral polyneuropathy which does not fulfill the criteria of demyelination or axonopathy. We describe a 37-year-old man with progressive bilateral leg weakness since his early teen. He suffered transient right hemiparesis, followed by quadriparesis at 14 years of age. When we examined him at 37 years of age, he presented a distal muscle weakness on lower extremities with a sensory symptom. The nerve conduction study demonstrated a motor conduction velocity between 26 and 49 m/s. The whole exome sequencing revealed a novel variant c.136 G>A in GJB1. This report will raise awareness in this rare disease, which is frequently misdiagnosed early in its course.

• Journal of Yeungnam Medical Science
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• v.6 no.1
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• pp.151-163
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• 1989

Nerve conduction studies help delineate the extent and distribution of the neural lesion. The nerve conduction was studied on upper(median, ulnar and radial nerves) and lower(personal, posterior tibial and sural nerves) extremities in 83 healthy subjects 23 to 66 years of age. and normal values were established(Table 1). The mean motor terminal latency (TL) were : median. 3.6(${\pm}0.6$)milliseconds ; ulnar. 2.9(${\pm}0.5$) milliseconds ; radial nerve. 2.3(${\pm}0.4$) milliseconds. Mean motor nerve conduction velocity(MNCV) along distal and proximal segments: median. 61.2(${\pm}9.1$) (W-E) and 57.8(${\pm}13.2$) (E-Ax) meters per second ; ulnar. 63.7(${\pm}9.1$) (W-E) and 50.(${\pm}10.0$) meters per second. Mean sensory nerve conduction velocity(SNCV) : median. 34.7(${\pm}6.7$) (F-W), 63.7(${\pm}7.1$) (W-E) and 62.8(${\pm}12.3$) (E-Ax)meters per second ; ulnar. 38.0(${\pm}6.7$)(F-W), 63.4(${\pm}7.5$) (W-E) and 57.0(${\pm}10.1$) (E-Ax)meters per second ; radial, 45.3(${\pm}6.8$) (F-W) and 64.2(${\pm}11.0$) (W-E) meters per second ; sural nerve, 43.4(${\pm}6.1$) meters per second. The amplitudes of action potential and H-reflex were also standardized. Mean H latency was 28.4(${\pm}3.2$) milliseconds. And. the fundamental principles, several factors altering the rate of nerve conduction and clinical application of nerve stimulation techniques were reviewed.

• Journal of Electrodiagnosis and Neuromuscular Diseases
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• v.20 no.2
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• pp.91-97
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• 2018

Objective: The aim of this study was to compare the electrodiagnostic outcomes of carpal tunnel release in patients with and without diabetes with carpal tunnel syndrome (CTS), and to evaluate the effect of diabetes mellitus (DM) on electrodiagnostic outcomes. Method: We conducted a retrospective analysis of 67 patients with electro-diagnostic evidence of CTS. Patients were classified into two groups according to the presence of DM. Both groups were evaluated using nerve conduction studies preoperatively and 3 weeks and 3 months postoperatively. Results: There were no statistical differences in any of the electrodiagnostic parameters between groups 3 weeks postoperatively. However, there were statistical differences in the amplitude and the latency of compound muscle action potential, and sensory nerve conduction velocity 3 months postoperatively. Conclusion: Patients with DM did not show a significantly different outcome 3 weeks after surgery but showed a worse electrodiagnostic outcome 3 months after surgery than those without DM.

• Journal of Genetic Medicine
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• v.17 no.2
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• pp.89-91
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• 2020

Charcot-Marie-Tooth (CMT) disease can be divided mainly into demyelination and axonopathy based on the results of the electrophysiological study. Mitofusin 2, encoded by MFN2 gene, has a crucial role in the fusion of mitochondria, which is known to associate with CMT type 2A as one of the axonal forms. We describe a 44-year-old man with progressive weakness on bilateral legs after noticing foot drop in his early teen. When we examined him at 45 years of age, he presented atrophy on entire legs and with distal muscle weakness on limbs. The nerve conduction study revealed severely decreased amplitude on motor nerve ranging from 0.2 to 4.5 mV, while conduction velocity remained more than 30.4 m/s. The whole-exome sequencing revealed a novel variant c.2228G>T in MFN2 by efficient genetic analysis tool, MutationDistiller. This report will not only expand the mutation spectrum of CMT2A but also introduce a time-saving genetic analysis tool.

• Annals of Clinical Neurophysiology
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• v.13 no.1
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• pp.31-37
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• 2011

Background: Carpal tunnel syndrome (CTS) and tarsal tunnel syndrome (TTS) are thought to share a similar pathophysiology, compression of the median and plantar nerve by the carpal tunnel and flexor retinaculum. A few reports introduced the relationship between idiopathic CTS and TTS without definite evidence of coexistence. The current study was designed to analyze the electrophysiologic characteristics of combined idiopathic CTS and TTS by comparing with each idiopathic CTS or TTS. Methods: We retrospectively collected patients with combined idiopathic CTS and TTS (CTS-TTS group) from June 2001 to February 2009. Patients with each idiopathic CTS or TTS were collected as controls. Electrophysiologic data of median and plantar nerves were compared between CTS-TTS group and controls. Results: CTS-TTS group was composed of 31 patients. Control group of each CTS or TTS were 50 CTS and 49 TTS patients. In comparison of median nerve conduction study between CTS-TTS group and CTS control group, decreased compound muscle action potential amplitude (p<0.001), decreased median sensory nerve action potential amplitude (p<0.001) and sensory nerve conduction velocity at finger stimulation (p=0.013) were prominent in CTS-TTS group. Decreased medial plantar sensory nerve action potential amplitude (p=0.034) was indicated when CTS-TTS groups and TTS control group were compared. Conclusions: If the electrophysiology study of patients with CTS or TTS was suggestive of severe degree of nerve injury, concerns about the possibility of combined CTS and TTS would be helpful.

• 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.

• The Korean Journal of Physiology and Pharmacology
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• v.2 no.1
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• pp.9-19
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• 1998

The present study was primarily carried out to characterize the properties of the spinomesencephalic tract (SMT) neurons that project from the upper cervical spinal segments to the midbrain. It was also investigated whether these neurons received convergent afferent inputs from other sources in addition to cervical inputs. Extracellular single unit recordings were made from neurons antidromically activated by stimulation of midbrain. Recording sites were located in lamina $I{\sim}VIII\;of\;C1{\sim}C3$ segments of spinal cord. Receptive field (RF) and response properties to mechanical stimulation were studied in 71 SMT neurons. Response profiles were classified into six groups: complex (Comp, n=9), wide dynamic range (WDR, n=16), low threshold (LT, n=5), high threshold (HT, n=6), deep/tap (Deep, n=10), and non- responsive (NR, n=25). Distributions of stimulation and recording sites were not significantly different between SMT groups classified upon their locations and/or response profiles. Mean conduction velocity of SMT neurons was $16.7{\pm}1.28\;m/sec$. Conduction velocities of SMTs recorded in superficial dorsal horn (SDH, n=15) were significantly slower than those of SMTs recorded in deep dorsal horn (DDH, n=18), lateral reticulated area (LRA, n=21), and intermediate zone and ventral horn (IZ/VH, n=15). Somatic RFs for SMTs in LRA and IZ/VH were significantly larger than those in SDH and DDH. Five SMT units (4 Comps and 1 HT) had inhibitory somatic RFs. About half (25/46) of SMT units have their RFs over trigeminal dermatome. Excitabilities of 5/12 cells and 9/13 cells were modulated by stimulation of ipsilateral phrenic nerve and vagus nerve, respectively. These results suggest that upper cervical SMT neurons are heterogenous in their function by showing a wide range of variety in location within the spinal gray matter, in response profile, and in convergent afferent input.

• Journal of Korean Neurosurgical Society
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• v.55 no.6
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• pp.370-374
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• 2014

To present a case of cauda equina syndrome (CES) caused by chronic inflammatory demyelinating polyneuropathy (CIDP) which seemed clinically similar to Charcot-Marie-Tooth disease type1 (CMT1). CIDP is an immune-mediated polyneuropathy, either progressive or relapsing-remitting. It is a non-hereditary disorder characterized by symmetrical motor and sensory deficits. Rarely, spinal nerve roots can be involved, leading to CES by hypertrophic cauda equina. A 34-year-old man presented with low back pain, radicular pain, bilateral lower-extremity weakness, urinary incontinence, and constipation. He had had musculoskeletal deformities, such as hammertoes and pes cavus, since age 10. Lumbar spine magnetic resonance imaging showed diffuse thickening of the cauda equina. Electrophysiological testing showed increased distal latency, conduction blocks, temporal dispersion, and severe nerve conduction velocity slowing (3 m/s). We were not able to find genetic mutations at the PMP 22, MPZ, PRX, and EGR2 genes. The pathologic findings of the sural nerve biopsy revealed thinly myelinated nerve fibers with Schwann cells proliferation. We performed a decompressive laminectomy, intravenous IgG (IV-IgG) and oral steroid. At 1 week after surgery, most of his symptoms showed marked improvements except foot deformities. There was no relapse or aggravation of disease for 3 years. We diagnosed the case as an early-onset CIDP with cauda equine syndrome, whose initial clinical findings were similar to those of CMT1, and successfully managed with decompressive laminectomy, IV-IgG and oral steroid.

• Annals of Clinical Neurophysiology
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• v.2 no.1
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• pp.21-26
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• 2000

Background: There has been few electrophysiologic studies in median proper palmar digital nerve(PPDN). Bar electrode may be a useful tool to evaluate the pathophysiologic state of the distal peripheral nerves. Objectives : To evaluate sensory nerve conduction velocities(NCVs) of median PPDNs in normal controls and carpal tunnel syndrome(CTS) patients by bar electrode, and clarify the usefulness of the bar electrode. Methods : We checked NCV of each median PPDN of thumb(D1), index(D2) and middle finger(D3) in normal controls(68 hands) and CTS patients(95 hands) by bar electrode. The each mean NCV of both groups were compared to find the correlation between them. Results : The mean NCV of each median PPDN in control group were $38.7{\pm}4.2$(D1), $32.0{\pm}4.6$(D2), $34.2{\pm}4.4$(D3) m/sec, and in CTS group were $35.3{\pm}8.9$(D1), $20.2{\pm}5.2$(D2), $20.2{\pm}5.1$(D3) m/sec orderly. There were significant differences between mean NCV of each finger in control group(p=0.0001), but not between each left and right finger(p>0.05). The differences between each mean NCV of control and CTS were significant in all 3 fingers(p=0.0014, 0.0000, 0.0000). Conclusion : Bar electrode is a useful tool to evaluate the pathophysiologic state of the median PPDNs in normal controls and CTS patients.