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Background@#and Purpose Among patients with double-seronegative myasthenia gravis (dSN-MG) who do not have detectable antibodies against acetylcholine receptor or musclespecific tyrosine kinase, autoantibodies against low-density lipoprotein receptor-related protein 4 (LRP4-Ab) have been detected recently. The purpose of this study was to develop an in-house cell-based assay (CBA) to detect LRP4-Ab and to apply it to samples from patients with MG. @*Methods@#The complementary DNA of LRP4 fused into a vector plasmid containing GFP was transfected into human embryonic kidney 293 (HEK293) cells. LRP4 expression in the transfected HEK293 cells was assessed using the reverse-transcription polymerase chain reaction (RT-PCR), Western blotting, and immunocytochemistry. The CBA included 252 sera collected from 202 patients with MG and 38 with other neuromuscular diseases, and 12 healthy controls. The transfected HEK293 cells were incubated using sera and antihuman immunoglobulin G antibodies conjugated with Alexa Fluor 594. The presence of LRP4-Ab was determined based on the fluorescence intensity and the localization in fluorescence microscopy. @*Results@#The expressions of the mRNA and protein of LRP4 in the transfected HEK293 cells were confirmed using RT-PCR and Western blotting, respectively. Immunocytochemistry indicated LPR4 expression on the cell membrane. Among 202 patients with MG including 53 with dSN-MG, LRP4-Ab were positive in 3 patients who were all double seronegative. LRP4-Ab were not detected in the patients with other neuromuscular diseases or the healthy controls. @*Conclusions@#A CBA for detecting LRP4-Ab associated with MG has been developed, and was used to find LRP4-Ab in the sera of patients with MG.
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Background@#and Purpose We performed a population-based study to determine the prevalence and incidence of chronic inflammatory demyelinating polyneuropathy (CIDP) in South Korea using data from the Korean Health Insurance Review and Assessment Service (HIRA) database. @*Methods@#Data recorded in the HIRA database between January 2016 and December 2020 were analyzed. The inclusion criteria in this study for patients with CIDP were a diagnostic code of G61.8 in the seventh and eighth revision of the Korean Standard Classification of Disease and a >3-month history of oral immunosuppressant use. The age-adjusted incidence rate and prevalence of CIDP in South Korea were also analyzed. @*Results@#CIDP was newly diagnosed in 953 patients during the study period. The mean age at diagnosis was 58.36 years, and the male-to-female ratio was 1.74. The age-adjusted incidence rates were 0.22, 0.21, 0.23, 0.30, and 0.25 per 100,000 person-years in 2016, 2017, 2018, 2019, and 2020, respectively. The age-adjusted prevalence was estimated at 1.16 per 100,000 persons in 2020. Age and the Elixhauser Comorbidity Index were associated with the in-hospital mortality of patients with CIDP. Infection and cardiovascular disease (CVD) were also significantly associated with the in-hospital mortality of those patients. Acute-onset CIDP was initially diagnosed in an estimated 101 out of 953 patients with CIDP. @*Conclusions@#The prevalence and incidence rates of CIDP in South Korea were comparable between this nationwide cohort study and previous studies. Common comorbidities such as CVD and diabetes should be appropriately monitored in patients with CIDP to prevent a poor prognosis and socioeconomic burden.
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Background@#and Purpose The description of pain is the most-important indicator leading to the adequate treatment of patients with neuropathic pain (NeP). The purpose of this study was to identify and characterize the unique features of Korean verbal descriptions in patients with peripheral NeP. @*Methods@#This study included 400 patients (167 males and 233 females) and their 1,387 paindescription responses. Patients with peripheral NeP freely described their symptoms in Korean. Collected verbal descriptions were grouped according to terminologies with similar meanings. Participants completed validated patient-reported outcome scales including the neuropathic pain symptom inventory (NPSI) and painDETECT questionnaire (PD-Q). The frequencies of each verbal pain descriptor were compared between the NPSI and PD-Q scores. @*Results@#‘Jeorim’ (tingling) was the most common among 17 types of organized verbal pain descriptors, and the ‘Sirim’ (cold) symptom had a significantly higher rate of use in the 2 highseverity groups when participants were classified by their total scores on the NPSI and PD-Q. @*Conclusions@#Korean verbal NeP descriptors were significantly diverse. The Jeorim (tingling) and Sirim (cold) descriptors can be utilized in evaluations of Korean patients with NeP.
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Parry-Romberg syndrome is a rare acquired disease of unknown etiology that is classically characterized by progressive atrophy of the skin, subcutaneous tissues, and underlying bone structures. Eyelid myokymia is a disorder of involuntary, fine, continuous, undulating muscle fiber contractions, which is seen as a rippling of the overlying periorbital area. We report a patient with Parry-Romberg syndrome presenting eyelid myokymia.
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Background@#A culturally validated Korean version of the PainDETECT Questionnaire (PD-Q) was used to identify neuropathic pain components (NeP) in patients suffering from chronic pain. The purpose of this study was to determine if the Korean PD-Q can be used to subgroup patients with peripheral NeP according to sensory symptom profiles. @*Methods@#This study included 400 Korean patients with peripheral neuropathic pain diagnosed as probable or definite NeP. The total scores and subscores for each item in PD-Q were transformed into a Z-score for standardization. Hierarchical cluster analysis was performed to identify clusters of subjects by PD-Q scores. @*Results@#The mean total PD-Q score of the study participants was 14.57 ± 6.46. A hierarchical cluster analysis identified 5 clusters with distinct pain characteristic profiles. Cluster 1 had relatively severe burning and tingling sensations. The mean total PD-Q score for cluster 2 was the lowest of the 5 clusters. Cluster 3 tended to be vulnerable to pain in response to cold/heat stimulation. Cluster 4 showed relatively severe pain induced by physical stimuli, such as light touch or slight pressure. Cluster 5 had high scores for all NeP symptoms. @*Conclusion@#This study demonstrates the ability of patients to cluster by symptoms using the Korean PD-Q. Subgrouping of peripheral neuropathic pain by sensory symptom profile may be useful in making effective drug treatment decisions.
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Subacute combined degeneration (SCD) of the spinal cord, which is a result of vitamin B12 deficiency, may cause irreversible neurological deficits. The lesion in the spinal cord is typically localized to the posterior and lateral columns, as the name implies. In this paper, we report on two patients with SCD, whose lesions involved the bilateral anterior column as well as the posterior and lateral columns. This report illustrates the importance of awareness of clinical and radiologic variable characteristics of SCD.
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Peripheral neuropathy and pain are common adverse effects of chemotherapy, which incidence are rising significantly commensurate with extension of survival period in cancer patients. Chemotherapy-induced peripheral neuropathy is caused by most commonly used chemotherapeutic agents including platinum compounds, taxenes, proteasome inhibitors, thalidomide, and vinca alkaloids. Management of neuropathy and pain caused by chemotherapy is still challenging due to there is no proven therapies and preventive methods. The pain and its impact are becoming a main deterioration factor in quality of life and economic burden in our society. We review the mechanism, clinical characteristics, updated evidence of possible management of neuropathy and pain caused by traditional chemotherapeutic agents for contributing to the application of clinicians in their actual medical environment.
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Diabetic neuropathy and chemotherapy-induced peripheral neuropathy are complex disorders affecting different nerve fiber group and remain as major source of morbidity and decreased quality of life of the patients. Given the recent advance in understanding the pathophysiological mechanism of these neuropathies and the development of new drugs, it is imperative to set proper scales to rate the severity of symptoms or signs. Moreover, well-designed, highly sensitive and valid scales could get scientific reliability and clinical utility if they are combined with objective neurophysiological measures. This review will discuss about the reliable and validated scales used in these common toxic and metabolic neuropathies.
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Background@#Pain is one of the most common chief complaints in neurological field. Authors try to inform the current situation of pain medicine in neurological practice and present an effective method for pain-related education during neurology residency and for practicing neurologists. @*Methods@#A survey was conducted from November 16 to November 27, 2020 for members of the Korean Neurological Association, and the results were analyzed. @*Results@#About two-thirds of neurologists replied that more than 25% of their patients were suffered from diverse pain including headache, spine pain or neuropathic pain. Despite many patients are visiting to neurologist for pain treatment, most neurologists are aware that they have not received sufficient practical pain education in the past and present. Therefore, they want more educational opportunities in pain medicine including interventional pain management and physical and pharmacologic therapies for more effective treatment. @*Conclusions@#More follow-up studies on pain treatment and education should be conducted. It is also essential that the members of the society continue to interest and participate in the change of the pain education program.
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Thorough evaluation and an accurate diagnosis of neuropathic pain are essential for effective treatment. The therapeutic approach and choice of medication for neuropathic pain are different from those for other kinds of nociceptive pain. Therefore, this study aimed to present the current evaluation and diagnostic methods for neuropathic pain.Current Concepts: Grading of the certainty of the presence of neuropathic pain according to the results of clinical history, neurological examination, and confirmatory tests improves the diagnosis of neuropathic pain. The Leeds Assessment of Neuropathic Symptoms and Signs, Neuropathic Pain Questionnaire, Douleur Neuropathique en 4 Questions, and PainDETECT are mainly used for neuropathic pain screening. During physical examination, sensory nerve function tests are more critical than other nervous system examination items, including the test of the sense of touch with a cotton swab and the sense of vibration with a tuning fork. In addition, pain sensation using pins and temperature sensation using cold metal are tested to check for nociceptive pathway abnormalities. Diagnostic tests include imaging tests, nerve conduction tests, and other neurophysiological tests, such as quantitative sensory function tests, autonomic nerve function tests, and blood tests.Discussion and Conclusion: To diagnose neuropathic pain, physicians should first determine whether patient symptoms match the characteristics of neuropathic pain. If there is a possibility of neuropathic pain, physicians should perform a neurological screening examination and a proper diagnostic test to identify the cause of pain.
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Diabetic neuropathy and chemotherapy-induced peripheral neuropathy are complex disorders affecting different nerve fiber group and remain as major source of morbidity and decreased quality of life of the patients. Given the recent advance in understanding the pathophysiological mechanism of these neuropathies and the development of new drugs, it is imperative to set proper scales to rate the severity of symptoms or signs. Moreover, well-designed, highly sensitive and valid scales could get scientific reliability and clinical utility if they are combined with objective neurophysiological measures. This review will discuss about the reliable and validated scales used in these common toxic and metabolic neuropathies.
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Background@#Pain is one of the most common chief complaints in neurological field. Authors try to inform the current situation of pain medicine in neurological practice and present an effective method for pain-related education during neurology residency and for practicing neurologists. @*Methods@#A survey was conducted from November 16 to November 27, 2020 for members of the Korean Neurological Association, and the results were analyzed. @*Results@#About two-thirds of neurologists replied that more than 25% of their patients were suffered from diverse pain including headache, spine pain or neuropathic pain. Despite many patients are visiting to neurologist for pain treatment, most neurologists are aware that they have not received sufficient practical pain education in the past and present. Therefore, they want more educational opportunities in pain medicine including interventional pain management and physical and pharmacologic therapies for more effective treatment. @*Conclusions@#More follow-up studies on pain treatment and education should be conducted. It is also essential that the members of the society continue to interest and participate in the change of the pain education program.
ABSTRACT
Thorough evaluation and an accurate diagnosis of neuropathic pain are essential for effective treatment. The therapeutic approach and choice of medication for neuropathic pain are different from those for other kinds of nociceptive pain. Therefore, this study aimed to present the current evaluation and diagnostic methods for neuropathic pain.Current Concepts: Grading of the certainty of the presence of neuropathic pain according to the results of clinical history, neurological examination, and confirmatory tests improves the diagnosis of neuropathic pain. The Leeds Assessment of Neuropathic Symptoms and Signs, Neuropathic Pain Questionnaire, Douleur Neuropathique en 4 Questions, and PainDETECT are mainly used for neuropathic pain screening. During physical examination, sensory nerve function tests are more critical than other nervous system examination items, including the test of the sense of touch with a cotton swab and the sense of vibration with a tuning fork. In addition, pain sensation using pins and temperature sensation using cold metal are tested to check for nociceptive pathway abnormalities. Diagnostic tests include imaging tests, nerve conduction tests, and other neurophysiological tests, such as quantitative sensory function tests, autonomic nerve function tests, and blood tests.Discussion and Conclusion: To diagnose neuropathic pain, physicians should first determine whether patient symptoms match the characteristics of neuropathic pain. If there is a possibility of neuropathic pain, physicians should perform a neurological screening examination and a proper diagnostic test to identify the cause of pain.
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3) of the pain in domains of tingling/prickling sensation (p=0.024), mechanical allodynia (p=0.027), sudden pain attacks (p=0.018), and thermal hyperalgesia (p=0.002) were significantly more frequent in NMOSD compared to MS patients. Among the patients experiencing pain with a neuropathic component, total pain-related interference (p=0.045) scores were significantly higher in NMOSD patients than in MS patients. In daily life, pain interfered with normal work (p=0.045) and relationships with other people (p=0.039) more often in NMOSD patients than in MS patients. Although pain medication was prescribed more frequently in NMOSD patients, the percentage of patients experiencing medication-related pain relief was lower in those patients.CONCLUSIONS: The severity of neuropathic pain and the pain-related interference in daily life were greater in NMOSD patients than in MS patients. Individualized analgesic management should be considered based on a comprehensive understanding of neuropathic pain in these patients.
Subject(s)
Humans , Hyperalgesia , Korea , Multiple Sclerosis , Neuralgia , Neuromyelitis Optica , Referral and Consultation , Sensation , Sex RatioABSTRACT
Neuropathy is the most prevalent microvascular complication of diabetes mellitus; it encompasses distal symmetric polyneuropathy, autonomic neuropathy, radiculoplexus neuropathy, mononeuropathy, and treatment-induced neuropathy. The prevalence rate of diabetic neuropathy in Korea was reported to be approximately 43%, which is similar to rates in other countries. However, the precise pathogenic mechanism underlying diabetic neuropathy is still obscure, and many clinical trials have failed to develop methods to prevent or reduce the progression of diabetic neuropathy. Nevertheless, early diagnosis and proper management of diabetic neuropathy are essential to alleviate disabling symptoms and to improve the quality of life of patients. This review discusses clinical manifestations and classification of diabetic neuropathies, bedside neurological examination, and electrophysiological tests.
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Neuromyelitis optica spectrum disorder (NMOSD) is generally known as selective involvement of central nervous system. However, in recent years, some evidences have been found that NMOSD invades other peripheral organs. Especially, skeletal muscle involvement of NMOSD has been documented scantily and further studies must be required. Here, we describe a patient who first had generalized fatigue, mild weakness, and myalgia with increased level of serum creatine kinase and was finally diagnosed with myopathy associated with NMOSD.
Subject(s)
Humans , Central Nervous System , Creatine Kinase , Fatigue , Muscle, Skeletal , Muscular Diseases , Myalgia , Myotonia , Myotonic Disorders , Neuromyelitis OpticaABSTRACT
The most prevalent microvascular complication of diabetes mellitus is neuropathy, which encompasses distal symmetric polyneuropathy, mononeuropathy, radiculoplexopathy, and autonomic neuropathy. Intensive glucose control prevents and effectively halts the progression of diabetic neuropathy in patients with type 1 diabetes mellitus. However, the effect of strict glucose control itself is at modest in those with type 2 diabetes. Although we have better understanding of the mechanism of diabetic neuropathy, many pharmacologic trials for the targeting underlying nerve damage have reported unsuccessful results. In this review, the effects and limitations of the current therapeutic options will be discussed.
Subject(s)
Humans , Diabetes Mellitus , Diabetes Mellitus, Type 1 , Diabetic Neuropathies , Glucose , Life Style , Mononeuropathies , PolyneuropathiesABSTRACT
Neuropathic cancer pain (NCP) is caused by nerve damage attributable to the cancer per se, and/or treatments including chemotherapy, radiotherapy, and surgery; the prevalence is reported to be as high as 40%. The etiologies of NCP include direct nerve invasion or nerve compression by the cancer, neural toxicity, chemotherapy, and radiotherapy. NCP is subdivided into plexopathy, radiculopathy, and peripheral neuropathies, among several other categories. The clinical characteristics of NCP differ from those of nociceptive pain in terms of both the hypersensitivity symptoms (burning, tingling, and an electrical sensation) and the hyposensitivity symptoms (numbness and muscle weakness). Recovery requires several months to years, even after recovery from injury. Management is complex; NCP does not usually respond to opioids, although treatments may feature both opioids and adjuvant drugs including antidepressants, anticonvulsants, and anti-arrhythmic agents, all of which improve the quality-of-life. This review addresses the pathophysiology, clinical characteristics and management of NCP, and factors rendering pain control difficult.
Subject(s)
Analgesics, Opioid , Anticonvulsants , Antidepressive Agents , Drug Therapy , Hypersensitivity , Neuralgia , Nociceptive Pain , Peripheral Nervous System Diseases , Prevalence , Radiculopathy , RadiotherapyABSTRACT
BACKGROUND AND PURPOSE: This retrospective cross-sectional study included 18 patients from unrelated families harboring mutations of the transthyretin gene (TTR), and analyzed their characteristics and geographical distribution in South Korea. METHODS: The included patients had a diagnosis of systemic amyloidosis, clinical symptoms, such as amyloid neuropathy or cardiomyopathy, and confirmation of a TTR gene mutation using genetic analysis recorded between April 1995 and November 2014. RESULTS: The mean age at disease onset was 49.6 years, and the mean disease duration from symptom onset to diagnosis was 3.67 years. Fifteen of the 18 patients were classified as mixed phenotype, 2 as the neurological phenotype, and only 1 patient as the cardiac phenotype. The most-common mutation pattern in South Korea was Asp38Ala, which was detected in eight patients. Thirteen patients reported their family hometowns, and five of the eight harboring the Asp38Ala mutation were from the Gyeongsang province in southeast Korea. The other eight patients exhibited a widespread geographical distribution. A particularly noteworthy finding was that the valine at position 30 (Val30Met) mutation, which was previously reported as the most-common TTR mutation worldwide and also the most common in the Japanese population, was not detected in the present South Korean patients. CONCLUSIONS: South Korean patients with hereditary TTR amyloidosis exhibited heterogeneous TTR genotypes and clinical phenotypes. The findings of this study suggest that the distribution of TTR amyloidosis in South Korea is due to de novo mutations and/or related to the other countries in East Asia.