Facial diplegia (FD) rarely occurs as a regional Guillain-Barré syndrome (GBS) variant. A 70-yearold male presented with bifacial weakness that had started on the left side and extended to the right after several days. He was then treated using steroids and gradually improved. Serum antiganglioside antibody testing revealed positivity for anti-GM1 IgG antibodies. FD can be idiopathic, but it is an uncommon GBS variant. The ganglioside antibody test may increase the possibility of diagnosing isolated FD.
Nitrous oxide (N2O), an anesthetic gas, has been abused by some people for entertainment purposes. Recently, N2O (under the name of "Happy Balloon") abuse has become problematic among young people in Korea. N2O intoxication can develop a neuropathy, as well as other systemic disorders, either by inactivating vitamin B12 or by a direct neurotoxic mechanism. Here, we report a case of peripheral neuropathy with possible coexisting myelopathy following N2O abuse.
Pain in Guillain-Barré syndrome (GBS) is known as a common symptom, experienced by about 72% of patients. Various types of pain are associated with GBS, including paresthesia, dysesthesia, radicular pain, meningism, myalgia and visceral pain. Pain in GBS can vary from mild to severe, often under-recognized and poorly managed. This article reviews the various pains associated with Guillain-Barré syndrome and their management.
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.
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Multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy is a variant of chronic acquired demyelinating polyneuropathy. A 65-year-old women presented with upper arm weakness. A nerve conduction study showed conduction blocks over intermediate
segments with sparing of distal compound action potentials. Magnetic resonance imaging revealed asymmetric hypertrophy of the brachial plexus on the affected side. These findings
represent important electrophysiological and radiological evidence of MADSAM neuropathy. The condition of the patient began to improve after starting intravenous immunoglobulin administration.
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.
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Chronic Hypoxemia Triggers a Neuropathic Process in Chronic Obstructive Pulmonary Disease: Insight From In Vivo Neurophysiological Assessments Seon Min Yoon, Young Bum Park, Yousang Ko, Jong Seok Bae Journal of Clinical Neurology.2023; 19(2): 186. CrossRef
Does Essential Tremor Alter the Axonal Excitability Properties of Lower Motor Neurons? Yerim Kim, Young Eun Kim, Joo Young Lee, Hyeo-Il Ma, Soon Kyung Shim, Sun min Yoon, Jong Seok Bae Journal of Clinical Neurophysiology.2022; 39(6): 492. CrossRef
Differences in nerve excitability properties between isolated bulbar palsy and bulbar-dominant amyotrophic lateral sclerosis So Young Pyun, Yerim Kim, Seong-Il Oh, Jong Seok Bae Neurological Research.2020; 42(2): 133. CrossRef
It is our differences that make us similar Jong Seok Bae Annals of Clinical Neurophysiology.2018; 20(1): 1. CrossRef
Moving towards synergy: The Korean Society of Pain & Autonomic Disorders is welcome! Jong Seok Bae, Ha Young Shin Annals of Clinical Neurophysiology.2018; 20(2): 55. CrossRef
Hemiplegia cruciata (HC) manifests as paralysis of the ipsilateral arm and contralateral leg. Herein, we report a 64-year-old man with weakness of the right leg and of the left arm after multiple sclerosis (MS). His brain and spine magnetic resonance imaging show a lower medulla lesion, which is extended to posterior part of C1 spine through cervicomedullary junction. HC usually results from stroke or trauma, but it is rare as presenting symptom of MS.
Atopic myelitis (AM) is a relatively mild form of myelitis associated with allergic diathesis, and present with predominant sensory manifestations. Lhermitte’s sign has been considered as a relatively non-specific clinical sign suggesting demyelinating lesion in cervical cord. Here we report a patient with recurrent AM who presented with isolated Lhermitte’s sign, both in first and second attacks. This report suggests that either the diagnosis or recurrence of AM can be frequently underdiagnosed because of its predominant sensory manifestations.
Background Transcranial magnetic stimulation (TMS) is a non-invasive diagnostic method particularly suited to investigation the long motor tracts. The clinical value of TMS in most spinal cord diseases has still to be made. Diagnostic value of magnetic motor evoked potential (MEP) parameters in intramedullary spinal cord lesions was investigated.
Methods MEP elicited by TMS was recorded in 57 patients with clinically and radiologically defined intramedullary myelopathy. Twenty five patients with cervical myelopathy (CM) and 32 thoracic myelopathy (TM) were included. Recordings were performed during resting and minimal voluntary contraction at both abductor pollicis brevis (APB) and tibialis anterior (TA) muscles. Stimulation threshold(ST), amplitude, and central motor conduction time (CCT) were measured at resting and facilitated conditions. CCT was calculated by two means; central motor latency (CML)-M using magnetic transcranial and root stimulation, and CML-F using electrical F-wave study. The results were compared between patient groups and 10 normal control group.
Results Facilitated mean ST recorded at TA was elevated in both CM and TM compared with control group. Resting mean CML-M at TA was significantly prolonged in both CM and TM, and CML-M was absent or delayed in 37.1% of CM and 8% of TM at APB with facilitation. Facilitated mean MEP amplitude at ABP was lower in CM than in TM, while MEP/M ratios were not different significantly between groups.
Conclusions Magnetic motor evoked potential has diagnostic value in intramedullary myelopathy and localizing value in differentiating between CM and TM by recording at APB and TA. It is a noninvasive way to investigate the functional status of motor tracts of spinal cord.
Various immunotherapeutic modalities have been used based on the autoimmune pathogenic mechanisms of myasthenia gravis (MG). Cell-mediated immunity as well as auto-antibodies may play a role in the remission and relapse of MG. We recently experienced two patients with MG who showed spontaneous remission fter inadvertent severe leukopenia. These findings suggest that the cell-mediated immune process is important in the treatment of MG, and selective suppression of leukocyte may induce remission in the patients with intractable MG.
The pathomechanisms involved in both restless legs syndrome (RLS) and complex regional pain syndrome type I(CRPS I) are still controversial whether they are central or peripheral origins. We recently encountered a patient whohad an unusual coexistence of both RLS and CRPS I, and both of which showed good responses to sympathetic block.These findings suggest the role of peripheral mechanisms, especially unmyelinated small autonomic fiber, in both RLSand CRPS I
Chronic low back pain (CLBP) is a distinct disease entity in that its clinical course and response to the treatment arequite different from acute low back pain (ALBP). CLBP is also closely related with systemic or preexisting psychosocialfactors rather than focal or localized factors. Since there has been little consensus in practice regarding the proper managementof CLBP, clinicians tend to approach and manage patients with CLBP in an empirical manner rather than an evidence-based one. In this article, I will review the difficulties of therapeutic choice in CLBP and provide superior aspectsof medical treatment over invasive interventional treatments.
Myotonic dystrophy type 1 (DM1) is an autosomal dominant multisystem disorder caused by the expansion of cytosine-thymine-guanine (CTG) repeats in the myotonic dystrophy protein kinase (DMPK) gene. Some literatures indicated that DM1 had incidentalCNS lesions such as white matter lesions and diffuse gray matter atrophy. We report a patient with DM1 whose brain magneticresonance image (MRI) showed multifocal hyperintense lesions and cystic lesion on both frontotemporoparietal lobes
Recently, we encountered a man with isolated bilateral sixth nerve palsies and areflexia whose titer of anti-GQ1b IgG antibody waselevated. We propose that bilateral sixth nerve palsies can be the sole manifestation of
Chronic graft-versus-host disease (GVHD) is a well-known complication of allogeneic bone marrow transplantation (BMT)and has heterogeneous manifestations, with multi-organ involvement. Recently, polymyositis (PM) was reported to be a raremanifestation of chronic GVHD. Here, we report a 30-year-old woman who was diagnosed with PM after allogeneic BMT.
Eun Hee Sohn, Byung-Jo Kim, Jong Kuk Kim, Jong Seok Bae, Wonki Baek, Bum Chun Suh, Jung-Joon Sung, Suk-Won Ahn, Joong Yang Cho, Yoon-Ho Hong, The Korean ALS/MND R Group
Geographical differences in the incidence of amyotrophic lateral sclerosis (ALS) have been reported, and there are stillmany unresolved problems. The incidence as well as epidemiologic data of ALS is not known in Korea. Web-based multicenterregistry of ALS, the Korean ALS registry, was established at January, 2011. The aim of
Eun Hee Sohn, Byung-Jo Kim, Jong Kuk Kim, Jong Seok Bae, Wonki Baek, Bum Chun Suh, Jung-Joon Sung, Suk-Won Ahn, Joong-Yang Choi, Yoon-Ho Hong, the Korean ALS/MND Research Group
Isolated posterior antebrachial cutaneous (PABC) neuropathy is rare. A 62 year-old man presented with hypesthesia over the skin region of PABC nerve territory after an injection at the proximal to the lateral epicondyle. Antidromic sensory nerve conduction studies of PABC nerve was not evoked on the affected side. Our case showed that sensory nerveconduction of PABC nerve can be performed without much difficulty and that this test is useful in confirming PABC neuropathy.
Two cases of bilateral ulnar neuropathies caused by telephone overuse are described in people engaged in the job of longtime telephone use. They had worked using telephone all through the working hours. Although they were right handed, they usually used the telephone in their left hand so as to leave their right hand free for writing, and would lean his elbow on the desk. Telesales or teleconsultation is a rapidly expanding field of business. These cases highlights the need of proper training and supplies appropriate to do their job.
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