Clinical and Radiological Features of Korean Patients With Anti-HMGCR Myopathy

Background@#and Purpose To understand the characteristics of Korean patients with anti-3-hydroxy-3-methylglutaryl-coenxyme A reductase (HMGCR) myopathy, we measured antiHMGCR antibodies and analyzed the clinical, radiological, and pathological features of patients with anti-HMGCR myopathy. @*Methods@#We measured titers of anti-HMGCR antibodies in the sera of 99 patients with inflammatory myopathy, 36 patients with genetic myopathy, and 63 healthy subjects using an enzyme-linked immunosorbent assay. We tested 16 myositis-specific autoantibodies (MSAs) in all patients with anti-HMGCR myopathy. @*Results@#Positivity for the anti-HMGCR antibody was observed in 17 (4 males and 13 females) of 99 patients with inflammatory myopathy. The median age at symptom onset was 60 years.Ten (59%) of the patients with anti-HMGCR positivity had taken statins. The titer of antiHMGCR antibodies was significantly higher in the statin-naïve group (median=230 U/mL, interquartile range=170–443 U/mL) than in the statin-exposed group (median=178 U/mL, interquartile range=105–210 U/mL, p=0.045). The most common symptom was proximal muscle weakness in 15 patients (88%), followed by myalgia in 9 (53%), neck weakness in 4 (24%), dysphagia in 3 (18%), and skin lesions in 2 (12%). The median titer of anti-HMGCR antibody was 202 U/mL. We found eight different MSAs in nine (53%) patients. The median disease duration from symptom onset to diagnosis was significantly shorter in the MSA-positive group than in the MSA-negative group (p=0.027). @*Conclusions@#Our study was the first to measure anti-HMGCR antibodies in inflammatory myopathy. It has provided new findings, including the suggestion of the coexistence of other MSAs in Korean patients.

Eflapegrastim versus Pegfilgrastim for Chemotherapy-Induced Neutropenia in Korean and Asian Patients with Early Breast Cancer: Results from the Two Phase III ADVANCE and RECOVER Studies / Journal of the Korean Cancer Association, 대한암학회지

Purpose@#We investigated the consistent efficacy and safety of eflapegrastim, a novel long-acting granulocyte-colony stimulating factor (G-CSF), in Koreans and Asians compared with the pooled population of two global phase 3 trials. @*Materials and Methods@#Two phase 3 trials (ADVANCE and RECOVER) evaluated the efficacy and safety of fixed-dose eflapegrastim (13.2 mg/0.6 mL [3.6 mg G-CSF equivalent]) compared to pegfilgrastim (6 mg based on G-CSF) in breast cancer patients who received neoadjuvant or adjuvant docetaxel/cyclophosphamide. The primary objective was to demonstrate non-inferiority of eflapegrastim compared to pegfilgrastim in mean duration of severe neutropenia (DSN) in cycle 1, in Korean and Asian subpopulations. @*Results@#Among a total of 643 patients randomized to eflapegrastim (n=314) or pegfilgrastim (n=329), 54 Asians (29 to eflapegrastim and 25 to pegfilgrastim) including 28 Koreans (14 to both eflapegrastim and pegfilgrastim) were enrolled. The primary endpoint, DSN in cycle 1 in the eflapegrastim arm was non-inferior to the pegfilgrastim arm in Koreans and Asians. The DSN difference between the eflapegrastim and pegfilgrastim arms was consistent across populations: –0.120 days (95% confidence interval [CI], –0.227 to –0.016), –0.288 (95% CI, –0.714 to 0.143), and –0.267 (95% CI, –0.697 to 0.110) for pooled population, Koreans and Asians, respectively. There were few treatment-related adverse events that caused discontinuation of eflapegrastim (1.9%) or pegfilgrastim (1.5%) in total and no notable trends or differences across patient populations. @*Conclusion@#This study may suggest that eflapegrastim showed non-inferior efficacy and similar safety compared to pegfilgrastim in Koreans and Asians, consistently with those of pooled population.

A Familial Case with Phenotypic Differences in a CAV3 Pathogenic Variant / 대한신경근육질환학회지

We reported an age 32 male with progressive proximal muscle weakness. The serum creatine kinase was 1,908 IU/L. The muscle biopsy from biceps brachii muscle showed nonspecific myopathic changes. The whole exome sequencing identified a heterozygous variant (c.296A>C) in CAV3. It was previously reported as a likely pathogenic variant. It was also detected in the male’s mother and brother. However, his mother and brother had only hyperCKemia without muscle weakness. Our case showed phenotypic heterogeneity in a family, with the same variant in CAV3.

Validation of the Individualized Neuromuscular Quality of Life Questionnaire in Korean Patients With Genetic Neuromuscular Diseases

Background@#and Purpose The Individualized Neuromuscular Quality of Life questionnaire (INQoL) is a widely used measure of the quality of life in patients with neuromuscular diseases. The purpose of this study was to translate and validate the Korean version of INQoL in Korean patients with neuromuscular diseases. @*Methods@#We translated the original INQoL version into Korean while applying appropriate language adaptations. The internal consistency, known-group validity, and test-retest reliability were also assessed. Construct validity was measured using the modified Rankin Scale (mRS) score and the manual muscle testing (MMT)-sum score based on the Medical Research Council scale, and concurrent validity was measured using the 36-item Short Form Survey (SF-36) questionnaire. @*Results@#This study enrolled 193 patients. The coefficients for internal consistency (Cronbach’s α=0.805 to 0.987) and test-retest reliability (Spearman’s ρ=0.453 to 0.886) were adequately high for all subscales except in the ‘treatment effects’ dimension. INQoL subscales other than those for locking, droopy eyelids, double vision, and swallowing difficulties were significantly associated with their relevant SF-36 domains (Spearman’s ρ=-0.274 to -0.833). Functional status and muscle strength were most strongly associated with independence (Spearman’s ρ=0.753 and p<0.001 for mRS score, Spearman’s ρ=-0.741 and p<0.001 for MMT-sum score). @*Conclusions@#The Korean INQoL is a reliable and validated measurement tool for Korean patients with neuromuscular diseases.

Excision Can Be Delayed until Size Grows in Small Papillary Lesions of the Breast

Purpose@#The management of intraductal papilloma (IDP) without atypia remains controversial. This study evaluated the manifestations and incidence of malignancy during observation without surgery in patients diagnosed with IDP by core needle biopsy (CNB), to confirm whether close follow-up instead of surgical treatment is the preferred treatment option in selected patients. @*Methods@#We retrospectively reviewed the data of 589 patients diagnosed with IDP by CNB between January 2009 to December 2018. The data of the 102 IDP lesions from 90 women who did not undergo immediate excision were analyzed. Of these, 84 patients received imaging follow-up without excision, while 18 patients underwent delayed excision during follow-up. @*Results@#During the median follow-up period of 18.6 months, the mean change in tumor size and mean percent change in tumor size were −0.06 cm and −0.22%, respectively. Delayed excision was performed in 18 patients (17.6%). In the delayed excision group, three (16.7%) patients and one (5.6%) patient were diagnosed with atypical papilloma and intraductal papillary carcinoma, respectively, based on the final pathological findings. The upstaged group (atypia and malignancy; four patients) showed a 62.0% increase in the tumor size, which is higher than the benign group that showed a 10.4% increase in tumor size. However, the difference was not statistically significant (p=0.185). @*Conclusion@#Observation without excision is possible for small IDP without atypia, because of the minimal changes in tumor size and low incidence of malignancy after excision. However, to avoid a missed diagnosis of malignancy, excision should be considered if the lesion increases in size during follow-up.

Posterior condylar offset changes and its effect on clinical outcomes after posteriorsubstituting, fixed-bearing total knee arthroplasty: anterior versus posterior referencing

Background@#We sought to determine whether there was a difference in the posterior condylar offset (PCO), posterior condylar offset ratio (PCOR) and clinical outcomes following total knee arthroplasty (TKA) with anterior referencing (AR) or posterior referencing (PR) systems. We also assessed whether the PCO and PCOR changes, as well as patient factors were related to range of motion (ROM) in each referencing system. @*Methods@#This retrospective study included 130 consecutive patients (184 knees) with osteoarthritis who underwent primary posterior cruciate ligament (PCL)-substituting fixed-bearing TKA. The difference between preoperative and postoperative PCO and PCOR values were calculated. Clinical outcomes including ROM and Western Ontario and McMaster University (WOMAC) scores were evaluated. Furthermore, multiple linear regression analysis was performed to determine the factors related to postoperative ROM in each referencing system. @*Results@#The postoperative PCO was greater in the AR group (28.4 mm) than in the PR group (27.4 mm), whereas the PCO was more consistently preserved in the PR group. The mean postoperative ROM after TKA was greater in the AR group (129°) than in the PR group (122°), whereas improvement in WOMAC score did not differ between the two groups. Preoperative ROM was the only factor related to postoperative ROM in both groups. @*Conclusions@#There was no difference in postoperative PCO in AR and PR group and the PCO was not associated with postoperative ROM. PCO was more consistently preserved after surgery in the PR group. The postoperative PCO and PCOR changes did not affect the postoperative ROM. Furthermore, similar clinical outcomes were achieved in the AR and PR groups.

Considerations for patient selection: Prepectoral versus subpectoral implant-based breast reconstruction

BACKGROUND@#In recent years, breast implants have been frequently placed in the subcutaneous pocket, in the so-called prepectoral approach. We report our technique of prepectoral implant-based breast reconstruction (IBR), as well as its surgical and aesthetic outcomes, in comparison with subpectoral IBR. We also discuss relevant considerations and pitfalls in prepectoral IBR and suggest an algorithm for the selection of patients for IBR based on our experiences.@*METHODS@#We performed 79 immediate breast reconstructions with a breast implant and an acellular dermal matrix (ADM) sling, of which 47 were subpectoral IBRs and 32 were prepectoral IBRs. Two-stage IBR was performed in 36 cases (20 subpectoral, 16 prepectoral), and direct-to-implant IBR in 43 cases (27 prepectoral, 16 subpectoral). The ADM sling supplemented the inferolateral side of the breast prosthesis in the subpectoral group and covered the entire anterior surface of the breast prosthesis in the prepectoral group.@*RESULTS@#The postoperative pain score was much lower in the prepectoral group than in the subpectoral group (1.78 vs. 7.17). The incidence of seroma was higher in the prepectoral group (31.3% vs. 6.4%). Other postoperative complications, such as surgical site infection, flap necrosis, implant failure, and wound dehiscence, occurred at similar rates in both groups. Animation deformities developed in 8.5% of patients in the subpectoral group and rippling deformities were more common in the prepectoral group (21.9% vs. 12.8%).@*CONCLUSIONS@#The indications for prepectoral IBR include moderately-sized breasts with a thick well-vascularized mastectomy flap and concomitant bilateral breast reconstruction with prophylactic mastectomy.

Post-Traumatic Stress Disorder Symptoms and Quality of Life of Patients with Coronary Artery Disease / 중환자간호학회지

PURPOSE: The purpose of this study was to investigate the degree of post-traumatic stress disorder (PTSD) symptoms and the effect of PTSD on quality of life (QoL) among coronary artery disease (CAD) patients.METHODS: The participants were 135 CAD patients who visited outpatient clinics in a university hospital in B city of Korea. Their PTSD symptoms and QoL were measured by structured questionnaires and analyzed with multiple hierarchical regression analysis.RESULTS: Among the 135 participants, 20.7% were classified as moderate risk, and 3.7% as high risk for PTSD. PTSD symptoms were significantly higher in participants who were diagnosed with CAD within 6 months (t=26.02, p < .001). The physical component of health-related QoL was influenced by gender (β=-.25, p=.003), recurrence of CAD (β=.21, p=.008), and PTSD symptoms (β=-.33, p < .001). The mental component of health-related QoL was influenced by religion (β=-.17, p=.044), body mass index (β=.17, p=.033), and PTSD symptoms (β=-.37, p < .001).CONCLUSION: Patients who had had a CAD diagnosis for less than 6 months were found to be vulnerable to PTSD, and PTSD was found to have a negative impact on the physical and mental components of their QoL. It is necessary to develop an intervention program for the effective prevention and management of PTSD symptoms in patients with CAD.

Spontaneous Repetitive Constriction and Dilatation of a Unilateral Pupil in a Brain Death Patient

In brain death state, bilateral pupil light reflexes are disappeared, and pupils are fixed with dilated. However, spontaneous movements such as ocular microtremor or bilateral cyclical constriction-dilatation of pupils have been rarely reported in brain death patients. We present a brain death patient whose right pupil displayed spontaneously repetitive constriction and dilatation regardless of external stimuli such as light and pain. Early recognition of this phenomenon may prohibit the delay in the diagnosis of brain death and organ transplantation.

A Patient with Corpus Callosum Infarction Accompanying Infective Endocarditis

No abstract available.

A Patient with Cerebral Infarction and Renal Infarction Accompanying Complex Aortic Plaque of Descending Thoracic Aorta

No abstract available.

Ultrasound-Guided Greater Occipital Nerve Block for Primary Headache: Comparison of Two Techniques by Anatomical Injection Site

OBJECTIVE: Greater occipital nerve block (GONB) is a widely accepted treatment of primary headaches. Two ultrasound (US)-guided blockade techniques exist: 1) the classical distal nerve block technique performed medial to the occipital artery at the superior nuchal line, and 2) the new proximal nerve block technique performed at the obliquus capitis inferior muscle at the level of C2. Our study aim was to perform a head-to-head comparative study of these two US-guided techniques.METHOD: Forty-nine patients with primary headache treated in our university hospital were recruited. Patients were randomized into two groups of the classical nerve block and the new proximal nerve block techniques. The headache questionnaire was made to assess the intensity of the pain of headache attacks, number of days they experience headache, duration of headache, and amount of pain medication they consumed.RESULTS: In both groups, a decrease in the severity and frequency of the headache was observed. There was no measurable difference in outcome between the two groups.CONCLUSION: Our study showed that the classic and new proximal techniques are equally effective in decreasing the headache severity and frequency.

Effect of White Matter Hyperintensity on the Functional Outcome of Ischemic Stroke Patients after Inpatient Stroke Rehabilitation

The aim of the study is to investigate the association between cerebral white matter hyperintensity (WMH) and the functional improvement using the Korean version of Modified Barthel Index (K-MBI) score during inpatient stroke rehabilitation. One hundred sixty participants were divided into 2 groups based on the severity of WMH according to Fazekas scale: Mild WMH group was defined as patients with Fazekas scale 0 and 1, and severe WMH group was defined as Fazekas scale 2 and 3. Functional status was assessed using the K-MBI score, and functional gains were calculated from the K-MBI score. The absolute functional gain in mild WMH group was significantly higher compared to severe WMH group (p < 0.05). In addition, patients in mild WMH had higher absolute functional efficiency, rehabilitation effectiveness, and relative functional efficiency. In the generalized linear model analyses, there was an association between functional outcomes and severity of WMH. In this study, the severity and extent of WMH are significantly correlated with poor functional improvement in patients with ischemic stroke. The WMH could be considered as one of many factors that can influence functional recovery during rehabilitation of stroke.

Correlations Between Values of Articulation Tests and Language Tests for Children With Articulation Disorder in Korea

OBJECTIVE: To evaluate correlations between values of articulation tests and language tests for children with articulation disorder in Korea. METHODS: Data of outpatients with chief complaint of an articulation problem were retrospectively collected. Patients who underwent Urimal Test of Articulation and Phonation (U-TAP) with Assessment of Phonology and Articulation for Children (APAC), Preschool Receptive-Expressive Language Scale (PRES), or Receptive and Expressive Vocabulary Test (REVT) simultaneously were identified. Patients whose word-level percentages of correct consonants in U-TAP (UTAP_wC) were more than 2 standard deviations below the mean as diagnostic criteria for articulation disorder were selected. Those whose receptive language age (P_RLA), expressive language age (P_ELA), or combined language age (P_CLA) in PRES was delayed more than 24 months compared to their chronological age in months as diagnostic criteria for language disorder were excluded. RESULTS: Thirty-three children aged 3–6 years were enrolled retrospectively. PRES and U-TAP showed significant correlations for most of value relationships. PRES and APAC showed significant correlations for all value relationships except for receptive language age. All values of REVT were significantly correlated with all values from U-TAP, but not with any value from APAC. Articulation tests U-TAP and APAC showed significant correlations between percentages of correct consonants. Language tests PRES and REVT showed significant correlations for all value relationships. CONCLUSION: This study suggests that articulation abilities and language abilities might be correlated in children with articulation disorder.

Risk Factors for a False-Negative Result of Sentinel Node Biopsy in Patients with Clinically Node-Negative Breast Cancer / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: Although sentinel lymph node biopsy (SLNB) can accurately represent the axillary lymph node (ALN) status, the false-negative rate (FNR) of SLNB is the main concern in the patients who receive SLNB alone instead of ALN dissection (ALND). MATERIALS AND METHODS: We analyzed 1,886 patientswho underwent ALND after negative results of SLNB, retrospectively. A logistic regression analysis was used to identify risk factors associated with a false-negative (FN) result. Cox regression model was used to estimate the hazard ratio of factors affecting disease-free survival (DFS). RESULTS: Tumor located in the upper outer portion of the breast, lymphovascular invasion, suspicious node in imaging assessment and less than three sentinel lymph nodes (SLNs) were significant independent risk factors for FN in SLNB conferring an adjusted odds ratio of 2.10 (95% confidence interval [CI], 1.30 to 3.39), 2.69 (95% CI, 1.47 to 4.91), 2.59 (95% CI, 1.62 to 4.14), and 2.39 (95% CI, 1.45 to 3.95), respectively. The prognostic factors affecting DFS were tumor size larger than 2 cm (hazard ratio [HR], 1.86; 95% CI, 1.17 to 2.96) and FN of SLNB (HR, 2.51; 95% CI, 1.42 to 4.42) in SLN-negative group (FN and true-negative), but in ALN-positive group (FN and true-positive), FN of SLNB (HR, 0.64; 95% CI, 0.33 to 1.25) did not affect DFS. CONCLUSION: In patients with risk factors for a FN such as suspicious node in imaging assessment, upper outer breast cancer, less than three harvested nodes, we need attention to find another metastatic focus in non-SLNs during the operation. It may contribute to provide an exact prognosis and optimizing adjuvant treatments.

Age-Specific Cutoff Scores on a T1-Weighted Axial Medial Temporal-Lobe Atrophy Visual Rating Scale in Alzheimer's Disease Using Clinical Research Center for Dementia of South Korea Data

BACKGROUND AND PURPOSE: Visual assessment of medial temporal-lobe atrophy (MTA) has been quick, reliable, and easy to apply in routine clinical practice. However, one of the limitations in visual assessments of MTA is the lack of widely accepted age-adjusted norms and cutoff scores for MTA for a diagnosis of Alzheimer's disease (AD). This study aimed to determine the optimal cutoff score on a T1-weighted axial MTA Visual Rating Scale (VRS) for differentiating patients with AD from cognitively normal elderly people. METHODS: The 3,430 recruited subjects comprising 1,427 with no cognitive impairment (NC) and 2003 AD patients were divided into age ranges of 50–59, 60–69, 70–79, and 80–89 years. Of these, 446 participants (218 in the NC group and 228 in the AD group) were chosen by random sampling for inclusion in this study. Each decade age group included 57 individuals, with the exception of 47 subjects being included in the 80- to 89-year NC group. The scores on the T1-weighted axial MTA VRS were graded by two neurologists. The cutoff values were evaluated from the area under the receiver operating characteristic curve. RESULTS: The optimal axial MTA VRS cutoff score from discriminating AD from NC increased with age: it was ≥as ≥1, ≥2, and ≥3 in subjects aged 50–59, 60–69, 70–79, and 80–89 years, respectively (all p < 0.001). CONCLUSIONS: These results show that the optimal cutoff score on the axial MTA VRS for diagnosing of AD differed according to the decade age group. This information could be of practical usefulness in the clinical setting.

Ischemic Stroke after Use of Cannabis: a Case Report and Review of Literature

Cannabis or marijuana is the most commonly used recreational drug after alcohol in the world, and usage is generally recognized as having few serious adverse effects. However, usage is restricted in South Korea. The report of ischemic stroke associated with cannabis is rare in literature. We present a case of a 47-year-old female patient with no underlying disease presenting with acute ischemic stroke after smoking cannabis in South Korea. The result for synthetic cannabinoid metabolites (delta-9 tetrahydrocannabinol) screening was positive. Absence of other vascular risk factors and drug screening results suggest a causal role of cannabis in this ischemic stroke case. The patient eventually progressed to brain death. The underlying mechanism, clinical manifestation, and imaging findings of cannabis-related stroke will be reviewed.

Radiating Pain and Postural Balance with Pulsed Radiofrequency Treatment in Patients with Lumbosacral Radiculopathy

OBJECTIVE: To investigate the effect of pulsed radiofrequency (PRF) treatment on pain and balance in patients with lumbosacral radiculopathy.METHOD: This study included twenty-five patients who were diagnosed with chronic lumbosacral radiculopathy. They underwent PRF treatment in prone position under the fluoroscopic guidance. The effect of PRF was measured by the visual analog score (VAS). Patient ability to balance was evaluated by using the Tetra-ataxiometric posturography (Tetrax).RESULTS: After PRF all patients showed improvement in pain as measured by VAS (p < 0.001) and none of the patients reported any side effects. The posturographic balance, which was evaluated by Tetrax showed no significant decline after PRF treatment. The weight distribution index (WDI) with eyes open before treatment was 5.43 ± 2.88 and after treatment was 5.37 ± 2.65 (p=0.917). The mean stability index (SI) with eyes open before treatment was 16.52 ± 6.05 and after treatment was 16.61 ± 4.85 (p=0.906). The mean WDI with eyes closed before treatment was 5.66 ± 2.81 and after treatment was 5.16 ± 2.70 (p=0.470). Finally, the mean SI with eyes closed before was 25.88 ± 9.88 and after treatment was 25.99 ± 12.30 (p=0.962).CONCLUSION: The results suggest that PRF has an effect on pain in patients with chronic lumbosacral radiculopathy. The patients did not experience adverse effects, such as hypoesthesia, dysesthesia and decreased proprioception after PRF, and there were no significant declines in balance.

Neuromyopathy caused by long term colchicine therapy

Colchicine-induced neuromyopathy is an extremely rare complication, and can develop in the setting of acute overdose or chronic administration in therapeutic doses. A 72-year-old man presented with proximal muscle weakness and myalgia. He had angina pectoris and Behçet’s disease, leading to the treatment of colchicine (1.2 mg daily for about 6 years), cyclosporine, methylprednisolone, simvastatin, and aspirin. A biceps brachii muscle biopsy was performed and electron microscopic examination revealed scattered autophagic vacuoles. He was initially treated with steroid pulse therapy. However, muscle weakness did not improve. After the discontinuation of colchicine, muscle power and myalgia improved steadily. There should be heightened awareness of colchicine-induced neuromyopathy because that clinical suspicion is the most important diagnostic clue, and termination of colchicine is the only treatment.

Diagnostic accuracy of manual office blood pressure measurement in ambulatory hypertensive patients in Korea

BACKGROUND/AIMS@#Currently, office blood pressure (OBP) is the most widely used method of measuring blood pressure (BP) in daily clinical practice. However, data on the diagnostic accuracy of OBP in reference to ambulatory blood pressure (ABP) are scarce in Korea.@*METHODS@#In retrospective and prospective cohorts, manual OBP and ABP measurements were compared among ambulatory hypertensive patients. Hypertension was defined as systolic OBP ≥ 140 mmHg and/or diastolic OBP ≥ 90 mmHg, and systolic ABP ≥ 130 mmHg and/or diastolic ABP ≥ 80 mmHg.@*RESULTS@#In the retrospective cohort (n = 903), the mean OBP1 (before ABP measurement) was higher than ABP in both systolic (138 ± 17 mmHg vs. 123 ± 13 mmHg, p < 0.001) and diastolic (84 ± 12 mmHg vs. 78 ± 11 mmHg, p < 0.001) measurements. Interestingly, there was only a weak correlation between OBP and ABP (r² = 0.038, p < 0.001). The overall discordance rate of OBP compared to ABP, which is the reference method for measuring BP, was 43.9%. The prospective cohort (n = 57) showed similar results. In a subgroup analysis, male patients had higher false negative results (masked or under-treated hypertension) than did female patients (26.1% vs. 17.8%, p = 0.003), whereas female patients had a higher false positive rate (white-coat or over-treated hypertension) than did male patients (28.7% vs. 15.2%, p < 0.001).@*CONCLUSIONS@#The diagnostic accuracy of manual OBP is low in reference to ABP. Men and women have different patterns of discordance. These findings indicate that management of hypertensive patients with manual OBP measurements may be suboptimal and encourages the use of ABP in ambulatory hypertensive patients.