Targeting the CSF-1/CSF-1R Axis: Exploring the Potential of CSF1R Inhibitors in Neurodegenerative Diseases.

We report herein the potential of colony-stimulating factor-1 receptor (CSF1R) inhibitors as therapeutic agents in neuroinflammatory diseases, with a focus on Alzheimer's disease (AD). Employing a carefully modified scaffold, N-(4-heterocycloalkyl-2-cycloalkylphenyl)-5-methylisoxazole-3-carboxamide, we identify highly selective and potent CSF1R inhibitors─7dri and 7dsi. Molecular docking studies shed light on the binding modes of these key compounds within the CSF1R binding site. Remarkably, kinome-wide selectivity assessment underscores the impressive specificity of 7dri for CSF-1R. Notably, 7dri emerges as a potent CSF-1R inhibitor with favorable cellular activity and minimal cytotoxicity among the synthesized compounds. Demonstrating efficacy in inhibiting CSF1R phosphorylation in microglial cells and successfully mitigating neuroinflammation in an in vivo LPS-induced model, 7dri establishes itself as a promising antineuroinflammatory agent.

A Simple Laser-Induced Breakdown Spectroscopy Method for Quantification and Classification of Edible Sea Salts Assisted by Surface-Hydrophilicity-Enhanced Silicon Wafer Substrates.

Salt, one of the most commonly consumed food additives worldwide, is produced in many countries. The chemical composition of edible salts is essential information for quality assessment and origin distinction. In this work, a simple laser-induced breakdown spectroscopy instrument was assembled with a diode-pumped solid-state laser and a miniature spectrometer. Its performances in analyzing Mg and Ca in six popular edible sea salts consumed in South Korea and classification of the products were investigated. Each salt was dissolved in water and a tiny amount of the solution was dropped and dried on the hydrophilicity-enhanced silicon wafer substrate, providing homogeneous distribution of salt crystals. Strong Mg II and Ca II emissions were chosen for both quantification and classification. Calibration curves could be constructed with limits-of-detection of 87 mg/kg for Mg and 45 mg/kg for Ca. Also, the Mg II and Ca II emission peak intensities were used in a k-nearest neighbors model providing 98.6% classification accuracy. In both quantification and classification, intensity normalization using a Na I emission line as a reference signal was effective. A concept of interclass distance was introduced, and the increase in the classification accuracy due to the intensity normalization was rationalized based on it. Our methodology will be useful for analyzing major mineral nutrients in various food materials in liquid phase or soluble in water, including salts.

Highly Precise Laser-Induced Breakdown Spectroscopy Analysis of Major Mineral Nutrients in Edible Salts Using Miniaturized Salt Ponds and Alternating Laser-Ablation Data Sampling.

In this work, we applied a hydrophilicity-enhanced solid substrate and an alternating laser-ablation data sampling (ALADS) scheme to improve laser-induced breakdown spectroscopy (LIBS) measurement precision and demonstrated the performance in analyzing K, Mg, Ca, and S contained in commercially available edible salt products. Five edible salt products from Australia, Bolivia, France, and South Korea were dissolved in water and a tiny volume of each solution was dropped on the solid substrate, that is, a miniaturized salt pond. After being dried, the residual salt crystals distributed still inhomogeneously, but the homogeneity could be significantly improved in comparison with that from typical drop-and-dry methods. The ALADS scheme was applied to extract three precise measurements from 9798 single-shot LIBS spectra covering the entire salt pond. The measurements obtained by ALADS were found to agree well with one another regardless of the inhomogeneous distribution of salt crystals. As a result, the measurement precision was proved remarkably. Limits of detection for K, Mg, Ca, and S were estimated to be 0.64, 1.7, 14, and 530 mg/kg, respectively, which are enough to analyze those elements contained in salts typically at the level of 100 parts per million (ppm) to ∼3 wt% for the purpose of salt quality assessment.

Removal of an intraosseous hemangioma of the frontal bone through an anterior hairline incision: a case report.

An intraosseous hemangioma of the frontal bone is typically removed via a coronal incision. This procedure, while effective, can be lengthy and may result in complications such as a prominent scar and hair loss. An alternative approach involves a direct incision in the forehead, which leaves a less noticeable scar and allows a quicker recovery. However, in this specific case, the patient declined both coronal surgery and surgery through a direct forehead incision due to cosmetic concerns. Therefore, we proposed an anterior hairline incision. A 35-year-old woman presented with a firm, non-mobile, palpable mass on her right forehead. Preoperative non-contrast computed tomography revealed a heterogeneous osteolytic lesion. We performed an excisional biopsy through the anterior hairline. Postoperative non-contrast computed tomography was conducted 2 and 6 months after surgery. The wound was clean and free of complications, and there was no local recurrence. Partial resection can reduce scarring for patients who are concerned about cosmetic outcomes. However, the potential for recurrence remains a significant concern. We present this case of an anterior hairline incision for a hemangioma located in the forehead, evaluated using serial computed tomography for both preoperative and postoperative imaging.

Pharmacological Prevention of Ectopic Erythrophagocytosis by Cilostazol Mitigates Ferroptosis in NASH.

Hepatic iron overload (HIO) is a hallmark of nonalcoholic fatty liver disease (NAFLD) with a poor prognosis. Recently, the role of hepatic erythrophagocytosis in NAFLD is emerging as a cause of HIO. We undertook various assays using human NAFLD patient pathology samples and an in vivo nonalcoholic steatohepatitis (NASH) mouse model named STAMTM. To make the in vitro conditions comparable to those of the in vivo NASH model, red blood cells (RBCs) and platelets were suspended and subjected to metabolic and inflammatory stresses. An insert-coculture system, in which activated THP-1 cells and RBCs are separated from HepG2 cells by a porous membrane, was also employed. Through various analyses in this study, the effect of cilostazol was examined. The NAFLD activity score, including steatosis, ballooning degeneration, inflammation, and fibrosis, was increased in STAMTM mice. Importantly, hemolysis occurred in the serum of STAMTM mice. Although cilostazol did not improve lipid or glucose profiles, it ameliorated hepatic steatosis and inflammation in STAMTM mice. Platelets (PLTs) played an important role in increasing erythrophagocytosis in the NASH liver. Upregulated erythrophagocytosis drives cells into ferroptosis, resulting in liver cell death. Cilostazol inhibited the augmentation of PLT and RBC accumulation. Cilostazol prevented the PLT-induced increase in ectopic erythrophagocytosis in in vivo and in vitro NASH models. Cilostazol attenuated ferroptosis of hepatocytes and phagocytosis of RBCs by THP-1 cells. Augmentation of hepatic erythrophagocytosis by activated platelets in NASH exacerbates HIO. Cilostazol prevents ectopic erythrophagocytosis, mitigating HIO-mediated ferroptosis in NASH models.

Six-Transmembrane Epithelial Antigen of Prostate 4: An Indicator of Prognosis and Tumor Immunity in Hepatocellular Carcinoma.

Purpose: The six-transmembrane epithelial antigen of prostate 4 (STEAP4) has been linked to tumor progression via its involvement in inflammatory responses, oxidative stress, and metabolism. However, STEAP4 has rarely been studied in hepatocellular carcinoma (HCC). We explored STEAP4 expression associated with tumor prognosis to understand its role in tumor biology in HCC. Patients and Methods: STEAP4 mRNA and protein expressions were primarily analyzed using bioinformatics tools based on The Cancer Genome Atlas database to understand the expression pattern, molecular mechanism, prognostic impact, and association with immune cell infiltration. We further investigated the association between STEAP4 protein expression and clinicopathological parameters and their predictive value in HCC patients using immunohistochemical staining of tissue microarrays. Results: The expression of STEAP4 mRNA and protein in HCC tissues was significantly lower than in normal liver tissues. Reduced expression of STEAP4 was linked to advanced HCC stages, poor recurrence-free survival (RFS), and overall survival. Furthermore, reduced STEAP4 expression was a significant predictor of worse RFS in univariate and multivariate analyses in the immunohistochemical cohort. GO, KEGG, and GSEA analyses revealed that STEAP4 is related to numerous biological processes and pathways, including drug metabolism, DNA replication, RNA metabolism, and immune response. In terms of the immune system, the decreased level of STEAP4 was correlated with the immunosuppressive microenvironment. Conclusion: Our data indicated that reduced STEAP4 expression was significantly associated with tumor aggressiveness and poor prognosis, possibly because of its link to various biological processes and induction of HCC immune evasion. Therefore, STEAP4 expression may serve as a potential prognostic biomarker for cancer progression and immunity, as well as a therapeutic target in HCC.

A Standardized Pathology Report for Gastric Cancer: 2nd Edition.

The first edition of 'A Standardized Pathology Report for Gastric Cancer' was initiated by the Gastrointestinal Pathology Study Group of the Korean Society of Pathologists and published 17 years ago. Since then, significant advances have been made in the pathologic diagnosis, molecular genetics, and management of gastric cancer (GC). To reflect those changes, a committee for publishing a second edition of the report was formed within the Gastrointestinal Pathology Study Group of the Korean Society of Pathologists. This second edition consists of two parts: standard data elements and conditional data elements. The standard data elements contain the basic pathologic findings and items necessary to predict the prognosis of GC patients, and they are adequate for routine surgical pathology service. Other diagnostic and prognostic factors relevant to adjuvant therapy, including molecular biomarkers, are classified as conditional data elements to allow each pathologist to selectively choose items appropriate to the environment in their institution. We trust that the standardized pathology report will be helpful for GC diagnosis and facilitate large-scale multidisciplinary collaborative studies.

Feasibility of a Low-Power, Low-Resolution Laser-Induced Breakdown Spectroscopy Instrument for Analysis of Nickel Alloys: Quantification of the Major Alloying Elements and Classification.

A simple cost-effective laser-induced breakdown spectroscopy (LIBS) instrument was used for quantification of major elements in several nickel alloys and also sorting them. A compact low-power diode-pumped solid-state laser and a miniature low-resolution spectrometer were assembled for the LIBS instrument. Material properties of the nickel alloys depend mainly on the composition of the major elements, Ni, Cr, and Fe, ranging from a few to ∼60 wt%. The emission peaks at 547.7 nm, 520.4 nm, and 438.1 nm for Ni, Cr, and Fe, respectively, were chosen for this analysis. The analytical performance was found to be enough for the quantification of Ni, Cr, and Fe in the nickel alloys. Limits of detection and accuracy were estimated to be a few weight percent (wt%) and measurement precisions were less than 10% in terms of relative standard deviation. The calibration performance of this intensity-based method was compared with that of the "ratio method" which is used in conventional optical emission spectroscopy analyses. The comparison indicates that the intensity-based method is more appropriate with the low-performance LIBS instrument that detects emission peaks of only a few major elements. Also, multivariate modeling of the six different nickel alloy samples based on the emission peak intensities of Ni, Cr, and Fe was performed using k-nearest neighbors (KNN) and linear discriminant analysis (LDA). The KNN and ordinary LDA models showed 95.0% and 98.3% classification correctness for the separate test data set, respectively. To improve classification performance further, the two-step LDA model was trained. In this approach, the two closest sample classes responsible for the decrease in the classification correctness were separately modeled in the second step to exploit their difference effectively. The two-step LDA model showed 100% correctness in classifying the test objects. Our results indicate that such a low-performance LIBS instrument can be effectively utilized for quantitative analysis of the major elements in the nickel alloys and their rapid identification or sorting in combination with an appropriate multivariate modeling algorithm.

A standardized pathology report for gastric cancer: 2nd edition.

The first edition of 'A Standardized Pathology Report for Gastric Cancer' was initiated by the Gastrointestinal Pathology Study Group of the Korean Society of Pathologists and published 17 years ago. Since then, significant advances have been made in the pathologic diagnosis, molecular genetics, and management of gastric cancer (GC). To reflect those changes, a committee for publishing a second edition of the report was formed within the Gastrointestinal Pathology Study Group of the Korean Society of Pathologists. This second edition consists of two parts: standard data elements and conditional data elements. The standard data elements contain the basic pathologic findings and items necessary to predict the prognosis of GC patients, and they are adequate for routine surgical pathology service. Other diagnostic and prognostic factors relevant to adjuvant therapy, including molecular biomarkers, are classified as conditional data elements to allow each pathologist to selectively choose items appropriate to the environment in their institution. We trust that the standardized pathology report will be helpful for GC diagnosis and facilitate large-scale multidisciplinary collaborative studies.

Comparison Trial between I-SCAN-Optical Enhancement and Chromoendoscopy for Evaluating the Horizontal Margins of Gastric Epithelial Neoplasms.

Background/Aims: Endoscopic submucosal dissection is a widely used treatment for gastric epithelial neoplasms. Accurate delineation of the horizontal margins is necessary for the complete resection of gastric epithelial neoplasms. Recently, image-enhanced endoscopy has been used to evaluate horizontal margins of gastric epithelial neoplasms. The aim of this study was to investigate whether I-SCAN-optical enhancement (I-SCAN-OE) is superior to chromoendoscopy in evaluating the horizontal margin of gastric epithelial neoplasms. Methods: This was a multicenter, prospective, and randomized trial. The participants were divided into two groups: I-SCAN-OE and chromoendoscopy. For both groups, we first evaluated the horizontal margins of early gastric cancer or high-grade dysplasia using white-light imaging, and then evaluated, the horizontal margins using I-SCAN-OE or chromoendoscopy. We devised a unique scoring method based on the pathological results obtained after endoscopic submucosal dissection to accurately evaluate the horizontal margins of gastric epithelial neoplasms. The delineation scores of both groups were compared, as were the ratios of positive/negative horizontal margins. Results: In total, 124 patients were evaluated for gastric epithelial neoplasms, of whom 112 were enrolled in the study. A total of 112 patients participated in the study, and 56 were assigned to each group (1:1). There was no statistically significant difference in the delineation scores between the groups (chromoendoscopy, 7.80±1.94; I-SCAN-OE, 8.23±2.24; p=0.342). Conclusions: I-SCAN-OE did not show superiority over chromoendoscopy in delineating horizontal margins of gastric epithelial neoplasms.

Expression of HLA class I is associated with immune cell infiltration and patient outcome in breast cancer.

Downregulation of human leukocyte antigen (HLA) class I is one mechanism of escaping anti-tumor immunity by tumor cells. This study was conducted to compare HLA class I expression in ductal carcinoma in situ (DCIS) and invasive breast carcinoma (IBC) and to evaluate its association with immune cell infiltration of the tumors and clinical outcome of the patients. A total of 830 cases comprising 288 DCIS and 542 IBC were included in this study. Immunohistochemistry for HLA class I expression was performed using HLA-ABC in tissue microarrays and was analyzed in relation to clinicopathologic characteristics of tumors and infiltration of CD4+, CD8+, and FOXP3+ tumor-infiltrating lymphocyte (TIL) subsets and PD-L1+ immune cells. As a whole, there was no difference in HLA class I expression between DCIS and IBC when dichotomized into high or low expression. However, in the HR-negative group, a high level of HLA class I expression was more frequent in IBC than DCIS. On the contrary, in the HR-positive group, a complete loss of HLA class I expression was more frequently observed in IBC than DCIS. High HLA class I expression level was generally associated with aggressive clinicopathologic features of IBC and was associated with high CD4+, CD8+, and FOXP3+ TIL and PD-L1+ immune cell infiltration in both DCIS and IBC. In survival analyses, HLA class I expression was not associated with clinical outcome in DCIS and IBC as a whole; however, low HLA class I expression was associated with poor clinical outcome in HR-negative IBC, especially in triple-negative subtype. In conclusion, this study showed that HLA class I expression increased in association with increased immune cell infiltration during in situ to invasive transition of HR-negative breast cancer, and HLA class I down-regulation had a prognostic value in HR-negative breast cancer.

Cerebral Microangiopathy Mimicking a High-Grade Glioma in Old Age: A Case Report.

Cerebral microangiopathy (CM) has become a common disease related to improved neuroimaging modalities and an increased life expectancy. Intracerebral tumor-like mass lesions have rarely been reported in cases of cerebral amyloid angiopathy (CAA) in elderly patients. However, tumor-like mass lesions from CM without amyloid deposits have rarely been reported. These two angiopathies may have different pathogeneses and neuroimaging characteristics. Herein, we present the case of an 83-year-old man with CM mimicking a high-grade glioma. We described the possible pathogenesis and different neuroimaging features of CM compared to CAA.

Stability of acetylsalicylic acid in human blood collected using volumetric absorptive microsampling (VAMS) under various drying conditions.

Acetylsalicylic acid (ASA) is one of the most commonly used medications in global market, with a risk of intoxication in certain patients. However, monitoring blood drug concentration often requires frequent hospital visits; hence there is an unmet need to increase patient-centricity by conducting blood sampling at home. Volumetric absorptive microsampling (VAMS) is a device that allows collection of homogenous and accurate volume of blood without venipuncture, and can be utilized by patients who are not in hospital settings; but because ASA is prone to hydrolysis and stabilizing reagents cannot be added to VAMS samples, a way to improve sample stability must be developed. The objective of this study was to identify the cause of instability with ASA samples collected by VAMS, and to evaluate ways to improve sample stability. A liquid chromatography with tandem mass spectrometry (LC-MS/MS) was used for analysis of ASA concentration in whole blood. Samples collected with VAMS were kept under different drying conditions (desiccator, pressurized, nitrogen gas and household vacuum sealer) and were compared to the control samples collected by conventional venous sampling. The recovery of ASA was about 31% of the control when VAMS sample was dried at room temperature, whereas VAMS samples under humidity controlled conditions showed more than 85% of recovery. Our results suggest that adequate level of humidity control was critical to ensure sample stability of ASA, and this humidity control could also be achieved at home using household vacuum sealer, thus enabling patient-centric clinical trials to be conducted.

Two-Step Partial Least Squares-Discriminant Analysis Modeling for Accurate Classification of Edible Sea Salt Products Using Laser-Induced Breakdown Spectroscopy.

Laser-induced breakdown spectroscopy (LIBS) has been widely applied to material classification in various fields, and partial least squares-discriminant analysis (PLS-DA) is one of the frequently used classical multivariate statistics to construct classification models based on the LIBS spectra. However, classification accuracy of the PLS-DA model is sensitive to the number of classes and their similarities. Considering this characteristic of PLS-DA, we suggest a two-step PLS-DA modeling approach to improve the classification accuracy. This strategy was demonstrated for a six-class problem in which six commercial edible sea salts produced in Japan, South Korea, and France are classified using their LIBS spectra. At the first step, test spectra were sorted into four classes and one extended class, composed of the two other most confusing classes, and then the test spectra in the extended class were further classified into each of the two constituent classes which were modeled separately from the other four classes. This two-step classification has been found to remarkably improve the PLS-DA classification accuracy by maximizing the difference between the confusing classes in the second-step modeling.

Successful induction treatment of bullous pemphigoid using reslizumab: a case report.

BACKGROUND: Bullous pemphigoid (BP) is a potentially life-threatening autoimmune blistering disease which is characterized by autoantibodies against hemidesmosomal proteins of the skin and mucous membranes. In recent years, the role of eosinophil and immunoglobulin E autoantibodies have been further elucidated in BP, and have been considered potential therapeutic targets. CASE PRESENTATIONS: A 67-year-old male presented with erythematous bullous eruption. The skin eruption was located on whole body, and suggested BP. Peripheral blood eosinophil count and total immunoglobulin E markedly elevated in initial laboratory findings. Topical and systemic steroid (methylprednisolone 2 mg/kg/day) treatment was started, and his skin symptoms worsened repeatedly, whenever systemic steroid were reduced. On admission day 29, reslizumab (anti-interleukin-5) 3.5 mg/kg was administered intravenously to the patients. The bullous skin lesion began to improve rapidly, and methylprednisolone (8 mg/day) was reduced without any worsening of symptoms during two doses of reslizumab. CONCLUSIONS: We report a case of successful treatment response to reslizumab administration in a patient with BP. Further studies are needed to confirm the role of anti-interleukin-5 as a treatment for BP in the future.

Association of Galectin 9 Expression with Immune Cell Infiltration, Programmed Cell Death Ligand-1 Expression, and Patient's Clinical Outcome in Triple-Negative Breast Cancer.

Galectin-9 (Gal-9) is an immune checkpoint protein that facilitates T cell exhaustion and modulates the tumor-associated microenvironment, and could be a potential target for immune checkpoint inhibition. This study was conducted to assess Gal-9 expression in triple-negative breast cancer (TNBC) and evaluate its association with programmed cell death ligand 1 (PD-L1) expression and immune cell infiltration in tumors and the clinical outcome of patients. Overall, 109 patients with TNBC were included. Gal-9 expression was assessed its relationships with tumor clinicopathologic characteristics, tumor-infiltrating lymphocyte (TIL) levels, PD-L1+ immune cells, and tumor cells by tissue microarray and immunohistochemistry. Low Gal-9 expression was statistically correlated with higher tumor stage (p = 0.031) and presence of lymphovascular invasion (p = 0.008). High Gal-9 expression was associated with a high level of stromal TILs (sTIL; p = 0.011) and positive PD-L1 expression on tumor cells (p = 0.004). In survival analyses, low Gal-9 expression was associated with significantly poor OS (p = 0.013) in patients with TNBC with PD-L1 negativity in tumor cells. Our findings suggest that increased Gal-9 expression is associated with changes in the antitumor microenvironment, such as increased immune cell infiltration and antimetastatic changes. This study emphasizes the predictive value and promising clinical applications of Gal-9 in TNBC.

Quantum Artificial Neural Network Approach to Derive a Highly Predictive 3D-QSAR Model for Blood-Brain Barrier Passage.

A successful passage of the blood-brain barrier (BBB) is an essential prerequisite for the drug molecules designed to act on the central nervous system. The logarithm of blood-brain partitioning (LogBB) has served as an effective index of molecular BBB permeability. Using the three-dimensional (3D) distribution of the molecular electrostatic potential (ESP) as the numerical descriptor, a quantitative structure-activity relationship (QSAR) model termed AlphaQ was derived to predict the molecular LogBB values. To obtain the optimal atomic coordinates of the molecules under investigation, the pairwise 3D structural alignments were conducted in such a way to maximize the quantum mechanical cross correlation between the template and a target molecule. This alignment method has the advantage over the conventional atom-by-atom matching protocol in that the structurally diverse molecules can be analyzed as rigorously as the chemical derivatives with the same scaffold. The inaccuracy problem in the 3D structural alignment was alleviated in a large part by categorizing the molecules into the eight subsets according to the molecular weight. By applying the artificial neural network algorithm to associate the fully quantum mechanical ESP descriptors with the extensive experimental LogBB data, a highly predictive 3D-QSAR model was derived for each molecular subset with a squared correlation coefficient larger than 0.8. Due to the simplicity in model building and the high predictability, AlphaQ is anticipated to serve as an effective computational screening tool for molecular BBB permeability.

Transcriptomic Landscape of Lower Grade Glioma Based on Age-Related Non-Silent Somatic Mutations.

Glioma accounts for 80% of all malignant brain tumours and is the most common adult primary brain tumour. Age is an important factor affecting the development of cancer, as somatic mutations accumulate with age. Here, we aimed to analyse the significance of age-dependent non-silent somatic mutations in glioma prognosis. Histological tumour grade depends on age at diagnosis in patients with IDH1, TP53, ATRX, and EGFR mutations. Age of patients with wild-type IDH1 and EGFR increased with increase in tumour grade, while the age of patients with IDH1 or EGFR mutation remained constant. However, the age of patients with EGFR mutation was higher than that of patients with IDH1 mutation. The hierarchical clustering of patients was dominantly separated by IDH1 and EGFR mutations. Furthermore, patients with IDH1 mutation were dominantly separated by TP53 and ATRX double mutation and its double wild-type counterpart. The age of patients with ATRX and TP53 mutation was lower than that of patients with wild-type ATRX and TP53. Patients with the double mutation showed poorer prognosis than those with the double wild type genotype. Unlike IDH1 mutant, IDH1 wild-type showed upregulation of expression of epithelial mesenchymal transition associated genes.

Necrotizing myopathy presenting as congestive heart failure and life-threatening ventricular arrhythmias: a case report.

BACKGROUND: Necrotizing autoimmune myopathy is a rare subtype of idiopathic inflammatory myopathy; however, it can be associated with fatal cardiac manifestations. CASE SUMMARY: A 58-year-old female patient was referred for congestive heart failure with dysrhythmia. Electrocardiograms showed ventricular arrhythmias of various QRS complex morphologies and coupling intervals with beat-to-beat differences. Despite optimal medical therapy for heart failure, the patient was admitted for the progression of dyspnoea and generalized motor weakness. The burden of non-sustained ventricular tachycardia gradually increased, and ventricular fibrillation eventually occurred. In view of a differential diagnosis of an inflammatory myocardial diseases such as sarcoidosis, a cardiac biopsy was performed. However, pathologic examinations revealed only necrotic muscle fibres without granuloma. Further examinations revealed proximal dominant motor weakness, an elevated serum creatinine-phosphokinase level, myogenic potentials on needle electromyography, and biceps muscle biopsy findings that were compatible with necrotizing autoimmune myopathy. High-dose steroid therapy improved the patient's motor weakness, including her respiratory impairment, and successfully suppressed ventricular arrhythmias. DISCUSSION: This case suggests that intensive immunosuppressive therapy with high-dose steroid could be useful in the necrotizing autoimmune myopathy manifested as congestive heart failure and life-threatening ventricular arrhythmias.