Juwon Lee, J. Park, Seung-min Jeong, J. Noh, G. Kang, S. Baek
{"title":"Granular Cell Tumor: An Unusual Cause of Common Peroneal Neuropathy","authors":"Juwon Lee, J. Park, Seung-min Jeong, J. Noh, G. Kang, S. Baek","doi":"10.18214/jend.2023.00038","DOIUrl":"https://doi.org/10.18214/jend.2023.00038","url":null,"abstract":"Granular cell tumors, also known as Abrikossoff’s tumors, are rare soft-tissue tumors, and their occurrence in the peripheral nerves has been very rarely reported. We present the case of a 35-year-old woman with common peroneal neuropathy due to a granular cell tumor, who complained of foot dragging. Common peroneal neuropathy was confirmed by electrophysiology. The tumor mass was observed using ultrasonography and magnetic resonance imaging. The tumor was surgically excised, the tumor cells stained positive for S-100, and eosinophilic granular cytoplasm was observed using microscopy. To the best of our knowledge, this is the first report of a granular cell tumor involving the common peroneal nerve.","PeriodicalId":440658,"journal":{"name":"Journal of Electrodiagnosis and Neuromuscular Diseases","volume":"4 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"129493772","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Bilateral Ischemic Lumbosacral Plexopathy after Proximal Aortic Surgery","authors":"Min Gi Kim, Seung Hak Lee","doi":"10.18214/jend.2022.00115","DOIUrl":"https://doi.org/10.18214/jend.2022.00115","url":null,"abstract":"In paraplegia after proximal aortic surgery, in addition to spinal cord ischemia, injuries to the plexuses and other peripheral nerves should be considered. Spinal cord ischemia—the most common etiology of paraplegia—can be caused by the occlusion of several radicular arteries resulting from aortic clamping. We report a case of bilateral ischemic lumbosacral plexopathy with a spared spinal cord occurring after proximal aortic surgery. A 76-year-old woman underwent aortic valve replacement with ascending aorta and total arch replacement, along with multiple hematoma evacuations. Postoperatively, she developed paraplegia with sensory deficits in the bilateral lower limbs. Spinal magnetic resonance imaging revealed no remarkable findings at any level of the spinal cord. In electrophysiological studies, the compound motor action potential and sensory nerve action potential of the bilateral lower extremities did not respond to stimuli; all examined muscles displayed abnormal spontaneous activities without motor unit action potentials. Based on these findings, a diagnosis of bilateral lumbosacral plexopathy was ultimately made.","PeriodicalId":440658,"journal":{"name":"Journal of Electrodiagnosis and Neuromuscular Diseases","volume":"9 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"133687545","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The Hmax/Mmax Ratio as a Diagnostic Tool in Assessing Spasticity in a Patient with Hereditary Spastic Paraplegia","authors":"Tae Yong Kim, K. Kim, Sung-Rae Cho","doi":"10.18214/jend.2022.00213","DOIUrl":"https://doi.org/10.18214/jend.2022.00213","url":null,"abstract":"Hereditary spastic paraplegia (HSP) refers to a group of inherited diseases caused by progressive degeneration of the corticospinal tracts. We report a case of an HSP patient with ankle spasticity treated with an intrathecal baclofen (ITB) pump, for whom the Hmax/Mmax ratio was used as a diagnostic tool for spasticity. A man in his early 30s who was born without any complications and developed normally in childhood was diagnosed with HSP when he was 29 years old. Equinus gait pattern and bilateral genu recurvatum improved after manual therapy and botulinum toxin injections in both gastrocnemius muscles; however, after a few months, his gait disturbance became more severe as a natural course of the disease. To treat ankle spasticity and clonus, he was considered a suitable candidate for an ITB therapy. However, as spasticity is a finding that is easy to recognize but difficult to evaluate, we conducted electrophysiological testing, including H-reflex and the ratio of H-reflex amplitude to compound muscle action potential amplitude. The Hmax/Mmax ratio was 75.2% on the right side and 65.2% on the left side before an ITB pump, and 37.6% on the right side and 47.0% on the left side after an ITB pump. This case illustrates the usefulness of testing electrophysiological parameters such as the Hmax/Mmax ratio to measure spasticity objectively in late-onset HSP patients. The Hmax/Mmax ratio is also a good tool for measuring the degree of improvement in spasticity after ITB or additional treatment in these patients.","PeriodicalId":440658,"journal":{"name":"Journal of Electrodiagnosis and Neuromuscular Diseases","volume":"7 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"132578832","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ho Jeong Shin, J. Lee, Sang Hun Kim, M. Shin, M. Jang
{"title":"Relationship between Chronic Obstructive Pulmonary Disease Severity and Surface Electromyography Parameters during Fatigue Caused by Knee Extensor Contractions","authors":"Ho Jeong Shin, J. Lee, Sang Hun Kim, M. Shin, M. Jang","doi":"10.18214/jend.2023.00024","DOIUrl":"https://doi.org/10.18214/jend.2023.00024","url":null,"abstract":"Objective: Skeletal muscle dysfunction, which is characterized by decreased muscle strength and endurance, contributes to dyspnea during exercise in patients with chronic obstructive pulmonary disease (COPD), regardless of pulmonary function. This study analyzed skeletal muscle function in patients with COPD through surface electromyography (sEMG) evaluations and signal processing and attempted to determine whether sEMG parameters for muscle fatigue reflect the course of the disease. Methods: In 24 patients with COPD, maximal voluntary isometric contraction and ramp contraction were performed during isometric knee extension, and the sEMG activity of the rectus femoris muscle was measured. The patients were divided into three groups according to their modified Medical Research Council (mMRC) grade and Global Initiative for Chronic Obstructive Lung Disease (GOLD) stage. Patients with mMRC grades 0 and 1 were classified into group A, grade 2 into group B, and grades 3 and 4 into group C. Patients with GOLD stage 1 were classified into group I, stage 2 into group II, and stages 3 and 4 into group III. sEMG parameters were compared between groups using one-way analysis of variance. Results: There were significant differences in the minimum median frequency and fatigue index between the mMRC groups. However, the sEMG parameters did not differ significantly among the GOLD groups. Conclusion: Muscle fatigue differs according to the severity of subjective dyspnea in COPD. sEMG evaluations and signal processing can play supplementary roles in evaluating muscle endurance and predicting functional capacity in COPD.","PeriodicalId":440658,"journal":{"name":"Journal of Electrodiagnosis and Neuromuscular Diseases","volume":"3 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"115816238","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Young-Gon Lee, Young-Su Ku, Han-Young Jung, M. Kim, K. Joa, C. Kim
{"title":"Paraneoplastic Neurologic Syndrome in Small Cell Lung Carcinoma","authors":"Young-Gon Lee, Young-Su Ku, Han-Young Jung, M. Kim, K. Joa, C. Kim","doi":"10.18214/jend.2023.00010","DOIUrl":"https://doi.org/10.18214/jend.2023.00010","url":null,"abstract":"A 54-year-old male smoker, previously in good health, was admitted to the hospital due to a tingling sensation in his upper and lower extremities. He reported difficulty walking due to a loss of balance and numbness, leading to an initial diagnosis of chronic inflammatory demyelinating polyneuropathy. Brain and spine magnetic resonance imaging, along with needle electromyography, yielded inconclusive findings. However, a nerve conduction study indicated a length-dependent pattern of sensory-dominant polyneuropathy. A cerebrospinal fluid study did not reveal any specific findings in terms of cell numbers, proteins, or immune tests. Following hospitalization, the patient reported progressive dizziness upon standing, leading to a preliminary diagnosis of orthostatic hypotension. However, a positive anti-Hu autoantibody test, along with chest computed tomography and positron emission tomography scans, revealed a mass in the left interlobar lymph node, suggestive of lung cancer. An endoscopic biopsy confirmed the presence of small cell lung cancer (SCLC). The patient underwent chemo-radiation treatment for the SCLC and immunoglobulin therapy for sensory ganglionopathy. As a result, a definitive diagnosis of paraneoplastic neurologic syndrome was made. Although such cases are rare, our observations suggest that symptoms of dysautonomia and sensory ganglionopathy may be associated with the production of anti-Hu antibodies.","PeriodicalId":440658,"journal":{"name":"Journal of Electrodiagnosis and Neuromuscular Diseases","volume":"22 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"123048746","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Diagnosis and Treatment of Cauda Equina Arachnoiditis, a Rare Manifestation of Tuberculosis Meningoencephalitis: A Case Report","authors":"Tae Yong Kim, D. Kim","doi":"10.18214/jend.2022.00199","DOIUrl":"https://doi.org/10.18214/jend.2022.00199","url":null,"abstract":"Tuberculous meningoencephalitis (TM) is an acute, progressive form of tuberculosis (TB). The epidemiology, clinical signs, and diagnosis of TB are well established, but several atypical forms of tuberculous spinal arachnoiditis can be easily misdiagnosed. We report a rare case of TM with cauda equina arachnoiditis diagnosed by magnetic resonance imaging and an electrodiagnostic study. A 26-year-old otherwise healthy male patient experienced fever, headache, gait disturbance, and bladder and bowel incontinence. Needle electromyography (EMG) recordings were suggestive of bilateral diffuse lumbar and lumbosacral polyradiculopathy, and the pudendal sensory-evoked potential and bulbocavernosus reflex latencies were prolonged. Lumbar arachnoiditis is a rare clinical condition that warrants a heightened index of suspicion. It has diverse etiologies and symptoms, and it can lead to potentially serious and irreversible disorders. This case illustrates the usefulness of nerve conduction, EMG, and pudendal sensory-evoked potential and bulbocavernosus reflex latency studies in the diagnosis of cauda equina syndrome induced by TB arachnoiditis.","PeriodicalId":440658,"journal":{"name":"Journal of Electrodiagnosis and Neuromuscular Diseases","volume":"49 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"130921641","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Sun Hong, Seoyeong Park, Yeji Lee, R. Lee, Sung Eun Hyun
{"title":"Prolonged Limb and Respiratory Muscle Weakness Associated with Japanese Encephalitis Virus Infection: A Case Report","authors":"Sun Hong, Seoyeong Park, Yeji Lee, R. Lee, Sung Eun Hyun","doi":"10.18214/jend.2022.00136","DOIUrl":"https://doi.org/10.18214/jend.2022.00136","url":null,"abstract":"Patients diagnosed with Japanese encephalitis (JE) may present with flaccid paralysis. JE has also been reported to be accompanied by anterior horn cell disease or motor axonal polyneuropathy. We report a case of a patient with JE with prolonged limb and respiratory muscle weakness who underwent electrodiagnostic studies, including a phrenic nerve conduction study, 10 months after the onset of paralysis. During that 10-month period, severe weakness of the upper and lower extremities showed no recovery, and the patient required long-term ventilator support through a tracheostomy. Nerve conduction studies and electromyography revealed chronic anterior horn cell disease with abundant denervation potentials involving the craniobulbar, cervical, thoracic, and lumbosacral segments. In addition to the nerves in the upper and lower extremities, the phrenic motor nerves showed abnormalities indicative of diaphragmatic weakness. Therefore, in patients with JE with chronic limb weakness and respiratory difficulty, thorough electrodiagnostic studies should be performed to diagnose the combination of anterior horn cell disease with encephalitis and to evaluate the condition’s severity and prognosis.","PeriodicalId":440658,"journal":{"name":"Journal of Electrodiagnosis and Neuromuscular Diseases","volume":"111 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"123443412","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Etiopathogenesis of Fibromyalgia","authors":"Seung-Geun Lee, Geun-Tae Kim","doi":"10.18214/jend.2022.00227","DOIUrl":"https://doi.org/10.18214/jend.2022.00227","url":null,"abstract":"Fibromyalgia is characterized by chronic widespread pain, and it is often accompanied by various symptoms such as fatigue, sleep disturbance, mood changes, cognitive dysfunction, and several somatic symptoms. The etiopathogenesis of fibromyalgia remains poorly understood, but it is thought to be caused by complex interactions among genetic predisposition, environmental factors, and biological factors. Emerging evidence suggests that central sensitization, which is characterized by impairment in the processes of pain perception, transmission, and modulation, plays an important role in fibromyalgia. Although various treatments have been used for fibromyalgia, patients still suffer from uncontrolled symptoms. Fibromyalgia still has many challenges to be solved. In this review, we discuss existing evidence on the etiopathogenesis of fibromyalgia.","PeriodicalId":440658,"journal":{"name":"Journal of Electrodiagnosis and Neuromuscular Diseases","volume":"311 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"121744029","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Vasculogenic Myoclonus of Peripheral Origin after Whiplash Injury: A Case Report","authors":"W. Choi, H. Kim","doi":"10.18214/jend.2022.00192","DOIUrl":"https://doi.org/10.18214/jend.2022.00192","url":null,"abstract":"","PeriodicalId":440658,"journal":{"name":"Journal of Electrodiagnosis and Neuromuscular Diseases","volume":"68 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-04-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"115393920","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Taewon Kim, Seung-Ah Lee, W. Choi, Seong-Woong Kang, Young-Chul Choi, H. Park
{"title":"Novel Pathogenic Variant in PIEZO2 in a Korean Patient with Distal Arthrogryposis","authors":"Taewon Kim, Seung-Ah Lee, W. Choi, Seong-Woong Kang, Young-Chul Choi, H. Park","doi":"10.18214/jend.2022.00171","DOIUrl":"https://doi.org/10.18214/jend.2022.00171","url":null,"abstract":"Alterations in PIEZO2 can result in distal arthrogryposis, which is characterized by non-progressive contracture in two or more areas of the body prior to birth. Here, we present a 29-year-old man born with multiple joint contractures and cleft palate. He showed short stature, low-set ears, macrotia, hearing impairment, micrognathia, a triangular face, blepharophimosis, deep-set eyes, high arched eyebrows, decreased facial expressions, retrognathia, arachnodactyly, absent phalan-geal crease, shortening of the first and fifth toes, short stature, pectus excavatum, epicanthus, bi-lateral ptosis, and ophthalmoplegia. He also complained of dyspnea and severe kyphoscoliosis. Pulmonary function tests showed a severe restrictive pattern. An electrodiagnostic study did not reveal any neurogenic or myogenic features. Next-generation sequencing revealed a novel de novo heterozygous pathogenic variant in PIEZO2 (c.7251G>T; p.Trp2417Cys). Our study is the first report of a pathogenic variant in PIEZO2 in a Korean patient with distal arthrogryposis.","PeriodicalId":440658,"journal":{"name":"Journal of Electrodiagnosis and Neuromuscular Diseases","volume":"87 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"126299404","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}