Characteristics of Diverse Verbal Pain Descriptors in South Korean Patients With Peripheral Neuropathic Pain:‘Jeorim’ (Tingling) and ‘Sirim’ (Cold) as Key Neuropathic Pain Descriptors

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.

Subgrouping of Peripheral Neuropathic Pain Patients According to Sensory Symptom Profile Using the Korean Version of the PainDETECT Questionnaire

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.

The Role of Neurologists in Pain Management – Based on the Survey Conducted by the Korean Neurological Association in 2020

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.

Importance and challenges of neuropathic pain

Pain is a complex and subjective symptom frequently encountered in many clinical fields. Acute pain is usually physiological, but chronic pain is a pathological condition that seriously affects the patientsʼ normal daily activities and quality of life. In addition, chronic pain has profoundly detrimental effects on their family and social relationship as well as a national healthcare system.Current Concepts: Neuropathic pain is accompanied by diverse neurological disorders in the central and peripheral nervous systems. Neuropathic pain is also a type of chronic pain and has mechanisms unresolved completely yet. Despite recent advances in basic and clinical researches of neuropathic pain including an individualized therapy, the treatment has not been satisfactory. Another problem is the increasing prevalence of neuropathic pain because of the rapid increase of the elderly population as in the case of Korea.Discussion and Conclusion: The clinician should be aware of a clinical entity of neuropathic pain to make an accurate diagnosis and to treat the patients more effectively.

The Role of Neurologists in Pain Management – Based on the Survey Conducted by the Korean Neurological Association in 2020

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.

Importance and challenges of neuropathic pain

Pain is a complex and subjective symptom frequently encountered in many clinical fields. Acute pain is usually physiological, but chronic pain is a pathological condition that seriously affects the patientsʼ normal daily activities and quality of life. In addition, chronic pain has profoundly detrimental effects on their family and social relationship as well as a national healthcare system.Current Concepts: Neuropathic pain is accompanied by diverse neurological disorders in the central and peripheral nervous systems. Neuropathic pain is also a type of chronic pain and has mechanisms unresolved completely yet. Despite recent advances in basic and clinical researches of neuropathic pain including an individualized therapy, the treatment has not been satisfactory. Another problem is the increasing prevalence of neuropathic pain because of the rapid increase of the elderly population as in the case of Korea.Discussion and Conclusion: The clinician should be aware of a clinical entity of neuropathic pain to make an accurate diagnosis and to treat the patients more effectively.

Recurrent Myelopathy in a Patient with Klippel-Trenaunay Syndrome / 대한임상신경생리학회지

Klippel-Trenaunay syndrome (KTS) is a rare congenital malformation syndrome involving blood and lymph vessels, which is characterized by triad of cutaneous hemangioma, venous varicosities, and overgrowth of the affected limbs. Because vascular malformation in KTS can be located anywhere except the face and brain, the clinical presentation could be extremely variable. But there are only rare case reports that KTS is associated with spinal cord lesion. We report a case of recurrent myelopathy in a patient with KTS.

Myasthenia Gravis Associated with Non-Small Cell Lung Cancer and Idiopathic Thrombocytopenic Purpura

A 34-year-old male presented with fluctuating diplopia, ptosis and motor weakness, who had been suffering from idiopathic thrombocytopenic purpura (ITP) and non-small cell lung cancer (NSCLC). Clinical and laboratory findings confirmed the diagnosis of generalized myasthenia gravis (MG). MG was successfully managed with pharmacologic treatments including an immunosuppressive agent. To the best of our knowledge, this is the first case of MG combined with ITP and NSCLC.

Clinical and Electrophysiological Characteristics of Meralgia Paresthetica / 대한임상신경생리학회지

BACKGROUND: Meralgia paresthetica (MP) is a mononeuropathy affecting the lateral femoral cutaneous nerve. The disease is often diagnosed clinically, but electrophysiological tests play an important role. The aim of this study is to clarify clinical characteristics of MP as well as the role of sensory nerve conduction study (NCS) in the diagnosis of MP. METHODS: Sixty-five consecutive patients with clinical diagnosis of MP between March 2001 and June 2012 were retrospectively reviewed at a single tertiary center. General demographics, clinical characteristics and sensory NCS findings were investigated. Measurements of sensory NCS included the baseline-to-peak amplitude, side-to-side amplitude ratio and the conduction velocity. To compare between the normal and abnormal NCS groups, independent t-tests and chi-square test were performed. RESULTS: Sixty-five patients had male predominance (56.9%) with mean age of 48.4+/-13.4 years (range: 16-75). Seven patients (13.5%) had undergone operation or procedure before the symptom onset. The sensory nerve action potentials were obtainable in 52 (80%) of 65 clinically diagnosed MP patients. Sensory NCS revealed abnormalities in 38 patients (73.1%), and others (n=14, 26.9%) showed normal findings. Between the normal and abnormal NCS groups, there is no statistically significant difference on demographics or clinical features. CONCLUSIONS: We clarify the clinical features and sensory NCS findings of MP patients. Due to several limitations of sensory NCS, the diagnosis of MP could be accomplished both clinically and electrophysiologically.

Guillain-Barre Syndrome, Antiphospholipid Syndrome and Lupus Nephritis as Initial Manifestation of Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is an autoimmune disease with various manifestations, while its autoantibodies and immune reactions involve multiple organs. Neuropsychiatric involvement in SLE is known to be common, however, peripheral neuropathy is relatively rare. Guillain-Barre syndrome is clinically defined as an acute demyelinating peripheral neuropathy causing weakness and numbness in the legs and arms. We describe a case of Guillain-Barre syndrome with antiphospholipid syndrome and lupus nephritis. The patient was admitted with fever and diarrhea. He developed progressive weakness of the upper and lower extremities and dysarthria with characteristic nerve conduction patterns compatible with Guillain-Barre syndrome. He also had proteinuria and gangrene of the hand and toe with antiphospholipid antibody. He received intravenous immunoglobulin and plasmapheresis for progressive neuropathy, intravenous high dose steroid to control activity of SLE, and anticoagulation for antiphospholipid syndrome. Neuropsychiatric manifestation of SLE is related to lupus activity closely, so it is important to control lupus activity.

Development of Korean Neuropathic Pain Questionnaire for Neuropathic Pain Screening and Grading: A Pilot Study

BACKGROUND: The pain-screening questionnaire is a self-reported description of the intensity and nature of pain. This study aimed to develop the Korean Neuropathic Pain Questionnaire (KNPQ) and to assess its reliability and validity regarding the diagnosis of neuropathic pain. METHODS: Four screening tools and two rating scales were translated and modified to develop the preliminary KNPQ. Following a development phase and a pilot study, we generated the final 25-item version of the KNPQ. Each item was rated on a numerical scale of 0-10. The validation procedure was performed in 62 patients with neuropathic pain (21 with central pain and 41 with peripheral pain) and in 34 patients with nonneuropathic pain. The internal consistency between items was assessed to determine the reliability of the KNPQ, and its concurrent validity was determined by evaluating the relationship between the Visual Analogue Scale (VAS) and KNPQ scores. RESULTS: The KNPQ was not influenced by age, sex, or pain duration. The 25-item questionnaire demonstrated high internal consistency. The total score of the KNPQ was correlated with the global pain intensity on a VAS. These items were able to differentiate neuropathic pain from nonneuropathic pain with a sensitivity of 84% and a specificity of 44% (when using a cut-off point of 46). CONCLUSIONS: The newly developed KNPQ may be used for the initial screening of neuropathic pain patients. However, it cannot be used to differentiate central neuropathic pain from peripheral neuropathic pain.

Erratum: Comparison between Clinical Disabilities and Electrophysiological Values in Charcot-Marie-Tooth 1A Patients with PMP22 Duplication

The publisher wishes to apologize for incorrectly displaying the author (Seok Beom Gwon) name. We correct his name from Seok Beom Gwon to Seok Beom Kwon.

Comparison between Clinical Disabilities and Electrophysiological Values in Charcot-Marie-Tooth 1A Patients with PMP22 Duplication

BACKGROUND AND PURPOSE: Charcot-Marie-Tooth disease (CMT) type 1A (CMT1A) is the demyelinating form of CMT that is significantly associated with PMP22 duplication. Some studies have found that the disease-related disabilities of these patients are correlated with their compound muscle action potentials (CMAPs), while others have suggested that they are related to the nerve conduction velocities. In the present study, we investigated the correlations between the disease-related disabilities and the electrophysiological values in a large cohort of Korean CMT1A patients. METHODS: We analyzed 167 CMT1A patients of Korean origin with PMP22 duplication using clinical and electrophysiological assessments, including the CMT neuropathy score and the functional disability scale. RESULTS: Clinical motor disabilities were significantly correlated with the CMAPs but not the motor nerve conduction velocities (MNCVs). Moreover, the observed sensory impairments matched the corresponding reductions in the sensory nerve action potentials (SNAPs) but not with slowing of the sensory nerve conduction velocities (SNCVs). In addition, CMAPs were strongly correlated with the disease duration but not with the age at onset. The terminal latency index did not differ between CMT1A patients and healthy controls. CONCLUSIONS: In CMT1A patients, disease-related disabilities such as muscle wasting and sensory impairment were strongly correlated with CMAPs and SNAPs but not with the MNCVs or SNCVs. Therefore, we suggest that the clinical disabilities of CMT patients are determined by the extent of axonal dysfunction.

Autosomal Dominant Hereditary Spastic Paraplegia Relavant with a Novel Thr369Pro Mutation in SPAST Gene

Hereditary spastic paraplegia (HSP) is a group of genetically heterogeneous neurodegenerative disorders that are characterized by progressive spasticity and weakness of both lower extremities. Here we report a novel missense mutation [c.1105A>C (p.Thr369Pro)] of SPG4 in a Korean family with an autosomal dominant-inherited pure HSP. The mutation is located in exon 8, and results in a heterozygote of the c.1105A>C mutation. It is likely that the mutation of exon 8 leads to spastin dysfunction and causes the typical symptoms and signs of HSP.

Esophageal Cancer-Associated Paraneoplastic Necrotizing Myopathy

No abstract available.

Clinical Features, Disability and Socioeconomic Status of Patients With Muscular Dystrophy in Korea

BACKGROUND: Since 2001, the Ministry of Health and Welfare in Korea has designated muscular dystrophy (MD) to be a rare and intractable disease, and has ensured that patients with this condition obtain support from the National Health Insurance Corporation for their medical expenditure. However, the health-related and socioeconomic status of Korean patients with MD has yet to be established. METHODS: We selected 441 patients with MD who received medical services at 17 neuromuscular centers during 2005. The medical records of selected patients were analyzed, and the subtype of MD was classified by its clinical course and diagnostic tests. A total of 95 patients or their family members participated in this health-related and socioeconomic status survey. RESULTS: Medical record analysis showed similar clinical and diagnostic characteristic data to those published previously in other countries: male predominance, being young at onset, and muscular weakness of the extremities as a predominant symptom in most patients. The diagnostic tests for MD were based on laboratory and electrophysiological studies. The most frequent form of MD among our cohort was Duchenne/Becker muscular dystrophy (42%). Our survey revealed the effect of the patients' profound disability on their activities of daily living. One-half of the patients were dissatisfied with the medical expenditure support service that was made available to them, and most patients suffered from a financial burden. The most important medical services to be developed in the future are expansion of the public health service or development of a rehabilitation hospital. CONCLUSIONS: This is the first multicenter-based epidemiologic study on the health-related and socioeconomic status of patients with MD in Korea. The findings indicate that medical coverage and public health service are currently inadequate and hence should be expanded in the future.

Ipsilateral Cerebral and Contralateral Cerebellar Hyperperfusion in Patients with Unilateral Cerebral Infarction; SPM Analysis / 핵의학분자영상

PURPOSE: Cortical reorganization has an important role in the recovery of stroke. We analyzed the compensatory cerebral and cerebellar perfusion change in patients with unilateral cerebral infarction using statistical parametric mapping (SPM). MATERIALS AND METHODS: Fifty seven (99m)Tc-Ethylene Cystein Diethylester (ECD) cerebral perfusion SPECT images of 57 patients (male/female=38/19, mean age=56+/-17 years) with unilateral cerebral infarction were evaluated retrospectively. Patients were divided into subgroups according to the location (left, right) and the onset (acute, chronic) of infarction. Each subgroup was compared with normal controls (male/female=11/1, mean age=36+/-10 years) in a voxel-by-voxel manner (two sample t-test, p<0.001) using SPM. RESULTS: All 4 subgroups showed hyperperfusion in the ipsilateral cerebral cortex, but not in the contralateral cerebral cortex. Chronic left and right infarction groups revealed hyperperfusion in the ipsilateral primary sensorimotor cortex, meanwhile, acute subgroups did not. Contralateral cerebellar hyperperfusion was also demonstrated in the chronic left infarction group. CONCLUSION: Using (99m)Tc-ECD SPECT, we observed ipsilateral cerebral and contralateral cerebeller hyperperfusion in patients with cerebral infarction. However, whether these findings are related to the recovery of cerebral functions should be further evaluated.

Cerebrovascular Reactivity According to Migraine Subtypes: with or without Aura

BACKGROUND: In migraine studies, the cerebrovascular reactivity (CVR) using a transcranial Doppler (TCD) has been investigated to elucidate the nature and role of the vascular response. However, past studies have not comprised the posterior circulation including functionally important brainstem structures. The purpose of this study was to compare the simultaneous CVRs between the middle cerebral artery (MCA) and basilar artery (BA) in migraine patients with and without aura, by means of a power motion mode Doppler (PMD) with an anterior-posterior probes fixating device. METHODS: Thirty-six consecutive patients with migranes [15 migraine patients with aura (MA) and 21 migraine patients without aura (MWA)] were compared with 29 healthy volunteers. CVR [(Vmax-Vbase)x100/Vbase] was evaluated by the re-breathing technique. TCD was performed as two steps. First, the velocities and spectra of the MCAs through both temporal windows were simultaneously monitored. Second, those were simultaneously monitored between MCA and BA. RESULT: There were no significant differences in age, sex, baseline hemodynamic values (blood pressure, heart rate), and those of the baseline mean flow velocity and CVR of TCD between the migraine patients and the controls. However, the CVR of the BA significantly differed between the MA and the MWA (39.4+/-13.7 vs 64.6+/-25.4%; p=0.001), among MA, MWA, and controls (39.4+/-13.7, 64.6+/-25.4, 45.6+/-14.9%; p<0.001). CONCLUSIONS: CVR of BA was entirely different according to migraine subtypes: with or without aura. Our study suggests that MWA and MWOA seem to be distinct disorders in terms of different vascular responses of the BA during the interictal period.

Simultaneously Measured CO2 Reactivity in the Basilar and Middle Cerebral Artery: The Utilization of Power M-mode Doppler and Anterior-posterior Probes Fixating Device

BACKGROUND: Studies using a transcranial Doppler (TCD) to establish cerebral vasoreactivity (CVR) have mostly focused on the anterior circulation. The purpose of this study is not only to evaluate the feasibility of the power motion mode Doppler (PMD) with a probes fixating device, but also to simultaneously measure the CVR between the middle cerebral artery (MCA) and the basilar artery (BA) during hypercapnea simulated by the rebreathing technique. METHODS: Twenty eight healthy volunteers were enrolled. Baseline hemodynamic values (heart rate, blood pressure) were measured while volunteers sat in a comfortable position for 5 minutes. The TCD was performed in two steps. First, velocities and spectra of the MCAs were simultaneously monitored. Then, the velocities and spectra of the MCA and BA were simultaneously monitored by a headset that included an anterior-posterior probes fixating device. The equation for CVR was ([maximum mean velocity baseline mean velocity] x 100/baseline mean velocity). RESULTS: Baseline mean velocities were revealed as follows: (64.0+/-13.7, 65.0+/-11.9 cm/s in right and left MCA; p>0.05; 67.3+/-12.2, -45.3+/-7.6 cm/s in dominant MCA and BA). CVR did not differ between the dominant MCA and the BA (46.1+/-12.1, 46.0+/-15.1%; p>0.05), nor between the right and left MCAs (46.9+/-15.2, 46.4+/-14.8%; p>0.05). There was a positive linear correlation between the CVR of the dominant MCA and that of the BA (r=0.856; p<0.001). CONCLUSIONS: PMD with a probes fixating device for accurate insonation is a useful tool for evaluating the relative CVR between the MCA and BA. Our study suggests that CVR values of the BA are similar to those of the MCA.

Oral Administration of Memantine Prolongs Survival in a Transgenic Mouse Model of Amyotrophic Lateral Sclerosis

BACKGROUND AND PURPOSE: N-methyl-D-aspartate (NMDA)-mediated neurotoxicity and oxidative stress have been implicated in the etiology of amyotrophic lateral sclerosis (ALS). Memantine is a low-affinity, noncompetitive NMDA receptor antagonist that may protect against motor neuron degeneration. METHODS: Thirty transgenic mice expressing the G93A SOD1 mutation were randomly divided into control, low-dose memantine (30 mg/kg/day), and high-dose memantine (90 mg/kg/day) groups, with memantine supplied daily with drinking water beginning at 75 days of age. Body weight, survival, and behavioral performances including a rotarod test, paw grip endurance, and hindlimb extension reflex were assessed in the control and memantine-diet groups. RESULTS: Clinical symptoms were evident in the G93A transgenic mice by 11 weeks of age. Memantine was tolerated well. Compared to control, mice treated with memantine performed better in the rotarod test and hindlimb extension reflex. Moreover, low-dose memantine treatment significantly prolonged the survival of the transgenic mice relative to control mice (141 vs 134 days, p<0.05). CONCLUSIONS: These findings suggest that memantine, even when administered at the time of symptom onset, has beneficial effects on patients with ALS.