We herein report a case of intractable epilepsy that occurred in a 7-year-old girl, which is consistent with radiological and clinicopathological hallmarks of Rasmussen's encephalitis. The patient showed characteristic primary unilateral involvement with secondary bilateral propagation. Microscopically, the cortical atrophy due to neuronal loss, intense GFAP-immunoreactive astrogliosis, neuronophagia, perivascular lymphocytic infiltration and microglial nodules was seen throughout the cortex and white matter. No viral inclusions were noted; no cytomegalovirus, herpes simplex virus or Epstein-Barr virus was found by in situ hybridization. Granular immunofluorescence for C4, C1q and IgG within the blood vessel walls was noted, and ultrastructurally, only nonspecific vascular injury was found. Rasmussen's encephalitis is a diagnosis of exclusion; it can be diagnosed by the combination of clinical manifestation, neuroimaging and characteristic pathologic features.
Atrophy ; Blood Vessels ; Child ; Cytomegalovirus ; Diagnosis ; Encephalitis* ; Epilepsy ; Female ; Fluorescent Antibody Technique ; Herpesvirus 4, Human ; Humans ; Immunoglobulin G ; Immunohistochemistry ; In Situ Hybridization ; Microscopy, Electron ; Neuroimaging ; Neurons ; Simplexvirus ; Vascular System Injuries

BACKGROUND AND PURPOSE: The occurrence of PWD in neurodegenerative disease is very rare, and this is the first report of it being related to early-onset AD. We describe a patient with early-onset Alzheimer's disease (AD) who presented with pure word deafness (PWD). CASE REPORT: The patient had experienced PWD for 2 years, followed by other cognitive deficits suggestive of parietotemporal dysfunction. Brain imaging including 18FDG-PET and [11C] PIB-PET supported the diagnosis of AD. CONCLUSIONS: Our case highlights the clinical variability that characterizes early-onset AD.
Alzheimer Disease ; Aphasia, Primary Progressive ; Deafness ; Humans ; Neurodegenerative Diseases ; Neuroimaging

Light chain deposition disease (LCDD) is a systemic disorder characterized by the deposition of monoclonal immunoglobulin light chains (LCs) in various organs. As LCs are overproduced by an abnormal clone of B cells, LCDD is usually described in the course of plasma cell dyscrasias or other lymphoproliferative disorders. However, it can occur in the absence of any detectable hematological disorder even during prolonged follow-up. We experienced a case of 62-year-woman who presented generalized edema, massive proteinuria and renal insufficiency. The histologic findings showed nodular glomerular mesangial expansion and prominent ribbon like immunohistochemical staining for kappa-light chain in the glomerular capillary wall. There was no evidence of multiple myeloma in bone marrow biopsy specimen. Because she refused the intensive chemothrapy, low dose of prednisolone and cyclophosphamide were prescribed for 6 months. Her renal function was maintained relatively well without renal replacement therapy for 7 months. We report a case of idiopathic LCDD not associated with multiple myeloma or other plasma cell dysclasia.
B-Lymphocytes ; Biopsy ; Bone Marrow ; Capillaries ; Clone Cells ; Cyclophosphamide ; Edema ; Follow-Up Studies ; Immunoglobulin Light Chains ; Lymphoproliferative Disorders ; Multiple Myeloma ; Nephrotic Syndrome ; Paraproteinemias ; Plasma Cells ; Prednisolone ; Proteinuria ; Renal Insufficiency ; Renal Replacement Therapy

OBJECTIVE: This study was conducted to evaluate the reliability of a questionnaire from an epidemiological study for nuclear power plants workers in Korea. METHODS: Among a total of 8,832 nuclear power plants workers who participated in the questionnaire survey, we selected 646 workers who repeatedly submitted the self-reported questionnaire. The reliability of the questionnaire for the categorical items was measured using kappa statistics and percentages of exact agreement, false disagreement and positive disagreement. Significance was evaluated for the continuous items by paired t-tests and intra-class correlation coefficients (ICCs). RESULTS: The questionnaire was observed to be highly reliable for the smoking history (kappa=0.85), which is known as an important confounding factor in assessing the cancer risk among radiation workers. Education level as a surrogate for the socio-economic status also appeared to be highly reliable with a kappa=0.89 and 95.09% of exact agreement. The history of alcohol drinking, medical exams and diseases showed more than moderately good agreement between the first and second responses (kappa>0.4). The self-reported values for physical measurements, the smoking period and smoking amount, and the period of alcohol drinking were highly correlated between the two responses (ICC>0.7). CONCLUSIONS: The questionnaire from an epidemiological study for nuclear power plants workers in Korea was found to be reliable for most items. To estimate the cancer or noncancer risk for nuclear power plants workers, we need to use the national database for incidence of diseases or death from diseases and the exposure history, and the medical records as well as the data collected from this study.
Alcohol Drinking ; Epidemiologic Studies ; Incidence ; Korea ; Medical Records ; Nuclear Power Plants ; Questionnaires ; Smoke ; Smoking

Background: Nonhuman primates (NHPs) are superior model for ocular research due to its morphological and physiological similarities with humans. Thus, the effect of four different anesthetic combinations [ketamine (10 mg/ kg), ketamine + xylazine (7 + 0.6 mg/kg), zoletil (4 mg/kg), and zoletil + xylazine (4 + 0.2 mg/kg)] on intraocular pressure (IOP) was determined in cynomolgus monkeys. Results: The administration of ketamine + xylazine or zoletil + xylazine resulted in lower IOP compared to ketamine or zoletil alone. Moreover, the IOP in male monkeys was higher than in females. The difference between the right and left eye was not found. Conclusions Anesthetics affected the IOP, and gender differences should be considered when measuring the IOP of nonhuman primates (NHPs).

Background: Nonhuman primates (NHPs) are superior model for ocular research due to its morphological and physiological similarities with humans. Thus, the effect of four different anesthetic combinations [ketamine (10 mg/ kg), ketamine + xylazine (7 + 0.6 mg/kg), zoletil (4 mg/kg), and zoletil + xylazine (4 + 0.2 mg/kg)] on intraocular pressure (IOP) was determined in cynomolgus monkeys. Results: The administration of ketamine + xylazine or zoletil + xylazine resulted in lower IOP compared to ketamine or zoletil alone. Moreover, the IOP in male monkeys was higher than in females. The difference between the right and left eye was not found. Conclusions Anesthetics affected the IOP, and gender differences should be considered when measuring the IOP of nonhuman primates (NHPs).

Objective: . To investigate the impact of the amendment of the Korean National Health Insurance (KNHI) reimbursement criteria for anti-tumor necrosis factor-α (TNF-α) agents based on from conventional clinical and laboratory measurements to disease activity score of 28 joints (DAS28) on treatment pattern, clinical response, and persistence rate in patients with rheumatoid arthritis (RA). Methods: . This multicenter retrospective cohort study evaluated 148 RA patients eligible for the initiation of anti- TNF-α agents as the first-line biologics by either the past (n=95) or current (n=53) KNHI reimbursement criteria. Persistence was defined as the duration between the initiation and discontinuation of anti-TNFα agents. Results: . In total, 106 (71.6%), 35 (23.6%), and 7 (4.7%) RA patients started treatment with adalimumab, etanercept, and infliximab, respectively. RA patients who received anti-TNF-α agents under the current reimbursement criteria had a significantly lower mean DAS28-erythrocyte sedimentation rate (ESR) (6.02 vs. 6.95, p＜0.001) and daily prednisolone-equivalent glucocorticoid dose (4.51 vs. 6.17 mg, p＜0.001) than those who received anti-TNF-α agents under the past reimbursement criteria. No significant differences in the 1-year remission rate defined by DAS28-ESR＜2.6 (17.9% vs. 30.2%, p=0.085) and the persistence rate (p=0.703) between the past and current reimbursement criteria was observed. Conclusion . Our data suggest that less active RA patients can receive reimbursement for anti-TNF-α agents under the current criteria, and the amendment of the KNHI reimbursement criteria may improve access to anti-TNF-α agents without affecting the treatment response and persistence rate.

Objective: To validate a simplified ordinal scoring method, referred to as modified length-based grading, for assessing coronary artery calcium (CAC) severity on non-electrocardiogram (ECG)-gated chest computed tomography (CT). Materials and Methods: This retrospective study enrolled 120 patients (mean age ± standard deviation [SD], 63.1 ± 14.5 years; male, 64) who underwent both non-ECG-gated chest CT and ECG-gated cardiac CT between January 2011 and December 2021. Six radiologists independently assessed CAC severity on chest CT using two scoring methods (visual assessment and modified length-based grading) and categorized the results as none, mild, moderate, or severe. The CAC category on cardiac CT assessed using the Agatston score was used as the reference standard. Agreement among the six observers for CAC category classification was assessed using Fleiss kappa statistics. Agreement between CAC categories on chest CT obtained using either method and the Agatston score categories on cardiac CT was assessed using Cohen’s kappa. The time taken to evaluate CAC grading was compared between the observers and two grading methods. Results: For differentiation of the four CAC categories, interobserver agreement was moderate for visual assessment (Fleiss kappa, 0.553 [95% confidence interval {CI}: 0.496–0.610]) and good for modified length-based grading (Fleiss kappa, 0.695 [95% CI: 0.636–0.754]). The modified length-based grading demonstrated better agreement with the reference standard categorization with cardiac CT than visual assessment (Cohen’s kappa, 0.565 [95% CI: 0.511–0.619 for visual assessment vs. 0.695 [95% CI: 0.638–0.752] for modified length-based grading). The overall time for evaluating CAC grading was slightly shorter in visual assessment (mean ± SD, 41.8 ± 38.9 s) than in modified length-based grading (43.5 ± 33.2 s) (P < 0.001). Conclusion The modified length-based grading worked well for evaluating CAC on non-ECG-gated chest CT with better interobserver agreement and agreement with cardiac CT than visual assessment.

Objective: CT plays a central role in determining the resectability of pancreatic cancer, which directs the use of neoadjuvant therapy. This study aimed to assess the diagnostic accuracy of CT in predicting circumferential resection margin (CRM) involvement in patients with resectable or borderline resectable pancreatic head cancer. Materials and Methods: Seventy-seven patients who were scheduled for upfront surgery for resectable or borderline resectable pancreatic head cancer were prospectively enrolled, and 75 patients (38 male and 37 female; mean age ± standard deviation, 68 ± 11 years) were finally analyzed. The CRM status was evaluated separately for the superior mesenteric artery (SMA) and posterior and superior mesenteric vein/portal vein (SMV/PV) margins. Three independent radiologists reviewed the preoperative CT images and evaluated the resection margin status. The reference standard for CRM status was pathologic examination of pancreaticoduodenectomy specimens in an axial plane perpendicular to the axis of the second portion of the duodenum. The diagnostic accuracy of CT was assessed for overall CRM involvement, defined as involvement of the SMA or posterior margins (per-patient analysis), and involvement of each of the three resection margins (per-margin analysis). The data were pooled using a crossed random effects model. Results: Forty patients had pathologically confirmed overall CRM involvement in pancreatic cancer, while CRM involvement was not seen in 35 patients. For overall CRM involvement, the pooled sensitivity and specificity were 15% (95% confidence interval: 7%–49%) and 99% (96%–100%), respectively. For each of the resection margins, the pooled sensitivity and specificity were 14% (9%–54%) and 99% (38%–100%) for the SMA margin, 12% (8%–46%) and 99% (97%–100%) for the posterior margin; and 37% (29%–53%) and 96% (31%–100%) for the SMV/PV margin, respectively. Conclusion CT showed very high specificity but low sensitivity in predicting pathological CRM involvement in pancreatic cancer.

BACKGROUND: Alveolar soft part sarcoma (ASPS) accounts for 0.5-1% of soft tissue sarcomas, and often metastasizes to the lung. Cases of pulmonary ASPS of unknown primary site have rarely been reported in literature. METHODS: Here, we report three cases of metastatic pulmonary ASPS and three cases of presumably primary ASPS using immunohistochemistry and clinicoradiologic findings. RESULTS: All of the cases occurred in young females. Two of the cases had metastasized from soft tissue ASPS of the lower extremities, and one case had metastasized from one of the patient? femur bones. Immunohistochemical stains were applied to four cases that had available paraffin blocks. The tumor cells of all cases on which immunohistochemical stains were done were positive for vimentin (4/4, 100%). None of the tumors were positive for myoglobin, desmin, smooth muscle actin, progesterone receptor, estrogen receptor, thyroid transcription factor-1, S-100 protein, pancytokeratin, and HMB-45 antibodies. CONCLUSION: The present study revealed that the rare pulmonary ASPS has nonspecific clinicoradiologic findings. In the immunohistochemical results, no differences existed between the presumably primary ASPS and the metastatic ASPS except for a higher Ki-67 labeling index in the latter (less than 0.1% vs. 30%). The higher index was not dissimilar to those of the extrapulmonary ASPS which showed a tumor with a low proliferation index, signifying a better prognosis and have a low potential to metastasize.
Actins ; Antibodies ; Coloring Agents ; Desmin ; Estrogens ; Female ; Femur ; Humans ; Immunohistochemistry ; Lower Extremity ; Lung* ; Muscle, Smooth ; Myoglobin ; Paraffin ; Prognosis ; Receptors, Progesterone ; S100 Proteins ; Sarcoma ; Sarcoma, Alveolar Soft Part* ; Thyroid Gland ; Vimentin ; Viperidae