Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) is a progressive degenerative white matter disorder caused by mutations in the tyrosine kinase domain of the CSF1R gene. ALSP is often misdiagnosed as other diseases due to its rarity and various clinical presentations such as Parkinsonism, pyramidal signs, cognitive impairment and/or psychiatric symptoms. We describe an autopsy case of ALSP with a CSF1R mutation. A 61-year-old woman presented insidious-onset gait difficulty for 12 years since her age of 49, and premature ovarian failure since her age of 35. At initial hospital visit, brain magnetic resonance imaging revealed hydrocephalus. Initially, Parkinson's syndrome was diagnosed, and she was prescribed L-dopa/carbidopa because of spasticity and rigidity of extremities, which had worsened. Subsequently, severe neuropsychiatric symptoms and cognitive impairment developed and radiologically, features of leukoencephalopathy or leukodystrophy were detected. She showed a down-hill course and died, 12 years after initial diagnosis. At autopsy, the brain showed severe symmetric atrophy of bilateral white matter, paper-thin corpus callosum, thin internal capsule, and marked hydrocephalus. Microscopically, diffuse loss of white matter, relatively preserved subcortical U-fibers, and many eosinophilic bulbous neuroaxonal spheroids were noted, but there was no calcification. Pigmented glia with brown cytoplasmic pigmentation were readily found in the white matter, which were positive for Periodic acid-Schiff, p62, and CD163 stains, but almost negative for CD68. Whole-exome and Sanger sequencing revealed a CSF1R mutation (c.2539G>A, p.Glu847Lys) which was reported in prior one ALSP case. This example demonstrates that ALSP could be associated with premature ovarian failure.
Atrophy ; Autopsy ; Axons ; Brain ; Cognition Disorders ; Coloring Agents ; Corpus Callosum ; Cytoplasm ; Diagnosis ; Eosinophils ; Extremities ; Female ; Gait ; Humans ; Hydrocephalus ; Internal Capsule ; Leukoencephalopathies ; Magnetic Resonance Imaging ; Middle Aged ; Muscle Spasticity ; Neuroglia ; Parkinsonian Disorders ; Pigmentation ; Primary Ovarian Insufficiency ; Protein-Tyrosine Kinases ; White Matter

BACKGROUND: Neuromuscular blocking agents (NMBAs) are a leading cause of perioperative anaphylaxis. However, the performance of systematic screening skin tests to detect reactions for NMBAs prior to general anesthesia is not recommended. We retrospectively examined intradermal tests (IDTs) for rocuronium and cisatracurium in patients with a history of allergy. METHODS: We reviewed the records of patients who underwent IDTs for NMBAs between January 1 and December 31, 2016. We analyzed the patients’ allergy histories and skin test results for NMBAs. RESULTS: The overall prevalence of positive IDTs was 5.8% (26/451), and there was no significant difference in prevalence among allergy types (P = 0.655). In logistic regression analysis, there was no allergy history that had a significant effect on positive IDT for NMBAs. CONCLUSIONS: We found no association between allergy history and positive skin test for NMBAs. Therefore, a systematic screening test for NMBAs or other anesthetic agents before anesthesia is not considered necessary even in patients with an allergy history.
Anaphylaxis ; Anesthesia ; Anesthesia, General ; Anesthetics ; Humans ; Hypersensitivity* ; Intradermal Tests ; Logistic Models ; Mass Screening ; Neuromuscular Blocking Agents ; Prevalence ; Retrospective Studies* ; Skin Tests* ; Skin*

Lower extremity deep vein thrombosis is a serious medical condition that can result in death or major disability due to pulmonary embolism or post-thrombotic syndrome. Appropriate diagnosis and treatment are required to improve symptoms and salvage the affected limb. Early thrombus clearance rapidly resolves symptoms related to venous obstruction, restores valve function and reduces the incidence of post-thrombotic syndrome. Recently, endovascular treatment has been established as a standard method for early thrombus removal. However, there are a variety of views regarding the indications and procedures among medical institutions and operators. Therefore, we intend to provide evidence-based guidelines for diagnosis and treatment of lower extremity deep vein thrombosis by multidisciplinary consensus. These guidelines are the result of a close collaboration between interventional radiologists and vascular surgeons. The goals of these guidelines are to improve treatment, to serve as a guide to the clinician, and consequently to contribute to public health care.
Consensus ; Cooperative Behavior ; Diagnosis* ; Extremities ; Incidence ; Lower Extremity* ; Methods ; Public Health ; Pulmonary Embolism ; Surgeons ; Thrombosis ; Venous Thrombosis*

OBJECTIVES: Subway drivers experience various types of work-related problems during their driving, and those experiences can act as risk factors for depressive symptoms. This study was conducted to investigate the association between work-related problems and subway driver's depressive symptoms. METHODS: We recruited all of the 961 current subway drivers of a subway company located in Seoul, South Korea and conducted a survey of their socio-demographic and vocational characteristics, hospital visits as an outpatient or inpatient, and work-related problem experiences during the last year. Work-related problems included an accident resulting in death or injury, a conflict with a customer, a sudden stop from an emergency bell, or a near accident. Depressive symptoms were measured with the Center for Epidemiologic Studies-Depression (CES-D) instrument. The survey was performed using a self-report questionnaire from April 16 to July 13, 2007. The data of 827 drivers (86.2%) were analyzed. RESULTS: Experience of a conflict with a passenger (p=0.011), a sudden stop from an emergency bell (p=0.001), or a near accident (p=0.001) increased the prevalence of depressive symptoms among subway drivers. A multiple logistic regression analysis revealed that a sudden stop from an emergency bell increased the risk of depressive symptoms significantly (OR=2.59, p=0.026). Near accidents were marginally associated with a higher risk for depressive symptoms (OR=1.62, p=0.062). CONCLUSION: The experience of a sudden stop from an emergency bell increased subway driver's depressive symptoms, and near accidents may increase the risk of depressive symptoms. Therefore, interventions for the drivers who had experienced these work-related problems are needed.
Depression ; Emergencies ; Humans ; Inpatients ; Logistic Models ; Outpatients ; Prevalence ; Railroads ; Republic of Korea ; Risk Factors ; Surveys and Questionnaires

BACKGROUND/AIMS: Although early recognition and treatment with effective antibiotics have lead to improvements in the prognosis of patients with spontaneous bacterial peritonitis (SBP), it remains to be a serious complication in cirrhotic patients. This study was designed to evaluate the clinical manifestations and prognosis of patients with liver cirrhosis and SBP in Korea. METHODS: This was a multicenter retrospective study examining 157 episodes of SBP in 145 patients with cirrhosis. SBP was diagnosed based on a polymorphonuclear cell count in ascitic fluid of >250 cells/mm3 in the absence of data compatible with secondary peritonitis. RESULTS: The mean age of the cohort was 56 years, and 121 (77%) of the 157 episodes of SBP occurred in men. Microorganisms were isolated in 66 episodes (42%): Gram-negative bacteria in 54 (81.8%), Gram-positive in 11 (16.7%), and Candida in 1. Isolated Gram-negative organisms were resistant to third-generation cephalosporin in 6 cases (17%), to ciprofloxacin in 11 (20.8%), and to penicillin in 33 (62.3%). The treatment failure and in-hospital mortality rates were 12.1% and 21%, respectively. A high Model of End-Stage Liver Disease (MELD) score, SBP caused by extended-spectrum beta-lactamase-producing organisms, and hepatocellular carcinoma were independent prognostic factors of high in-hospital mortality. CONCLUSIONS: SBP remains to be a serious complication with high in-hospital mortality, especially in patients with a high MELD score.
Anti-Bacterial Agents ; Ascitic Fluid ; Candida ; Carcinoma, Hepatocellular ; Cell Count ; Ciprofloxacin ; Cohort Studies ; Fibrosis ; Gram-Negative Bacteria ; Hospital Mortality ; Humans ; Liver ; Liver Cirrhosis ; Liver Diseases ; Male ; Penicillins ; Peritonitis ; Prognosis ; Retrospective Studies ; Treatment Failure

OBJECTIVES: This study was performed to review the clinical characteristics and operative results of brain abscess in order to define the therapeutic strategy for this disease. METHODS: We reviewed the medical records and radiology images of brain abscess patients treated in our hospital during the last 16 years. A total of 35 cases included 23 males and 12 females, with the mean age of 48 years old. We excluded cases of postoperative, post traumatic, and fungal abscess. All patient underwent at least one surgical treatment such as stereotactic aspiration or craniotomy with excision. RESULTS: Twenty seven (77.1%) patients presented with symptoms of increased intracranial pressure. The frontal lobe was the most common anatomical place, and streptococcal species were the most frequently encountered pathogens. The chronic pulmonary diseases and chronic otitis media are common underlying condition. Eighteen patients underwent stereotactic aspiration and 17 patients had excision of their abscess as an initial treatment. Seven patients had a repeated surgery, 6 of them had been treated with aspiration initially. At discharge, 60.0% patients showed a favorable outcome. CONCLUSION: The stereotactic drainage would be more suitable for the brain abscess located in deep and eloquent area. A large, solitary, and well-encapsulated lesion of superficial location could be best treated with complete excision, and this procedure was more definite because it is associated with less repeated surgery and showed more favorable outcome compared to aspiration surgery.
Abscess ; Brain Abscess* ; Brain* ; Craniotomy ; Drainage ; Female ; Frontal Lobe ; Humans ; Intracranial Pressure ; Lung Diseases ; Male ; Medical Records ; Middle Aged ; Otitis Media

OBJECTIVE: We investigate risk factors of cerebral microbleeds(MBs) and their relation to concomitant magnetic resonance (MR) findings in intracerebral hemorrhages(ICHs) patients. METHODS: We studied 100 consecutive patients with primary ICH over a 1-year period. These patients underwent brain MR images using 3.0-T scanners within the first week of the hemorrhage. MBs and old hematomas were located and counted by using T2*-weighted gradient-echo MR imaging. We also counted lacunes and graded white matter and periventricular hyperintensity on T1- and T2-weighted spin-echo sequences. The association between MBs and vascular risk factors and MR abnormalities were analyzed. RESULTS: MBs were seen in 77 of ICH patients, and their number ranged from 1 to 65 lesions (mean 11, median 6). The locations of MBs were subcortex-cortex (40.6%), basal ganglia (26.7%), thalamus (14.1%), brain stem (12.5%), and cerebellum (9.1%). Analysis of clinical data revealed that age, hypertension, history of stroke, and duration of hypertension were frequently associated with MBs. The incidence of lacunes, old hematomas, and advanced leukoaraiosis was significantly higher in the MBs group, compared with the patients without MBs. CONCLUSION: MBs are frequently observed in ICH patients with advancing age, chronic hypertension, and previous hemorrhagic stroke, and are also closely related with morphological signs of occlusive type microangiopathy, such as lacunar infarct and severe leukoaraiosis.
Basal Ganglia ; Brain ; Brain Stem ; Cerebellum ; Cerebral Hemorrhage* ; Hematoma ; Hemorrhage ; Humans ; Hypertension ; Incidence ; Leukoaraiosis ; Magnetic Resonance Imaging ; Risk Factors ; Stroke ; Stroke, Lacunar ; Thalamus

PURPOSE: We examined the relationship and concordance between end-tidal carbon dioxide (Petco2) and arterial CO2 (Paco2) in acute asthmatic patients presenting to emergency department. METHODS: This was prospective observational cohort study of acutely ill adult asthmatic patients observed March 2005 to February 2006. Data Collected were age, sex, vital sign, treatment, simultaneous Paco2 and Petco2 value and peak expiratory flow rate (PEFR). Concordance between Paco2 and Petco2 was represented by Bland-Altman plot, using pre-specified limits of agreement of +/-6 mmHg difference and described by interclass correlation coefficient. RESULTS: The study population consisted 51 adult asthma patients admitted during the study period. There was 92 Paco2 and Petco2 pairs. The Pearson correlation coefficient was 0.773 (p=0.000). The mean GAP (The difference Paco2 and Petco2) is 6.04mmHg. PEFR and GAP were negative correlation (R=-0.370). We obtained a Receiver operating characteristic (ROC) curve from PEFR and GAP, using a PEFR cutoff value of 30.5%. The interclass correlation coefficient between Paco2 and Petco2 was 0.652 for patients with PEFR above 30.5%, 0.362 for patients with PEFR below 30.5%, and 0.575 for patients with undetectable PEFR. CONCLUSION: Good correlation exists between Paco2 and Petco2, butr concordance is poor (among all patients the interclass correlation coefficient was 0.508). We attribute this to ventilation-perfusion mismatch. We concluded that in severely asthmatic patients with low PEFR or mechanical ventilation, end-tidal carbon dioxide is not concordant with arterial carbon dioxide.
Adult ; Asthma* ; Capnography ; Carbon Dioxide* ; Carbon* ; Cohort Studies ; Emergency Service, Hospital ; Humans ; Peak Expiratory Flow Rate ; Prospective Studies ; Respiration, Artificial ; ROC Curve ; Vital Signs

OBJECTIVE: The object of this study is to present the treatment experience of the 6cases of scalp arteriovenous malformations(AVMs) focus on treatment strategy. METHODS: Six patients with scalp AVM were treated during past 12years. We analysis the clinical characteristics of the lesions, treatment methods and management outcomes. RESULTS: The lesions were located on temporal in 2 patients, parietal in 2 patients, frontal and occipital area in each one. Four of six patients had a trauma history on scalp. The presenting symptoms were progressive enlarged pulsating mass with or without bruit. Four of the six lesions had the large fistula in the lesion. Two patients were treated with surgical resection alone, three patients with proximal feeding artery balloon(s) occlusion followed by surgical resection, and one patient with coil embolization through trans-venous route alone. We obtained good results in all patients. CONCLUSION: Most of scalp AVM can be completely cured by judicious selection and a combination of treatment modalities, i.e., surgery only, or embolization only, or embolization plus surgical therapy. Although embolization became a primary therapy for this sort of scalp AVM recently, the selection of treatment modality should be chose based on the size, angioarchitecture, and clinical presentations of the lesion.
Arteries ; Arteriovenous Malformations* ; Embolization, Therapeutic ; Fistula ; Humans ; Rabeprazole ; Scalp*

Melioidosis is an infection of the Gram-negative bacterium Burkholderia pseudomallei. While it is known as an important cause of sepsis or chronic abscessforming disease in Southeast Asia and northern Australia, no case has yet been reported in Korea. A 50-yr-old man visited our hospital for intermittent fever associated with dry cough and sputum. Roentgenographic examination showed migrating pulmonary infiltration. Symptoms and chest radiograph and computed tomography (CT) image findings did not improve despite use of fluoroquinolone antibiotics. Gram-negative bacteria were isolated on bronchoscopic washing culture and were identified as B. pseudomallei on DNA sequencing of 16S ribosomal RNA with 100% homology. Treatment for melioidosis was commenced with high dose ceftazidime, and the patient's fever, cough, and sputum were improved and the lesion on chest radiograph and CT almost disappeared.
Anti-Bacterial Agents/pharmacology ; Burkholderia pseudomallei/metabolism ; Ceftazidime/pharmacology ; Diagnosis, Differential ; Fluoroquinolones/pharmacology ; Humans ; Korea ; Lung/*microbiology ; Lung Diseases/diagnosis/*microbiology ; Male ; Melioidosis/diagnosis/*pathology ; Middle Aged ; RNA, Ribosomal, 16S/chemistry ; Sepsis ; Sequence Analysis, DNA ; Temperature ; Time Factors ; Tomography, X-Ray Computed