Development and Validation of the Korean Version of the Edinburgh Cognitive and Behavioral Amyotrophic Lateral Sclerosis Screen (ECAS-K)

Background@#and Purpose Cognitive and behavioral changes are common in amyotrophic lateral sclerosis (ALS), with about 15% of patients presenting with overt frontotemporal dementia and 30%–50% with varying degrees of impairments. We aimed to develop and validate the Korean version of the Edinburgh Cognitive and Behavioral ALS Screen (ECAS-K), a brief multidomain assessment tool developed for ALS patients with physical disability. @*Methods@#We developed the ECAS-K according to the translation guidelines, and administered it to 38 patients with ALS and 26 age- and education-level-matched controls. We also administered the Montreal Cognitive Assessment (MoCA) and Frontal Assessment Battery (FAB) to investigate convergent validity, and the Center for Neurologic Study-Liability Scale to assess the association between pseudobulbar affect and cognitive/behavioral changes. @*Results@#Internal consistency among the ECAS-K test items was found to be high, with a Cronbach’s alpha of 0.87. Significant differences were found between patients with ALS and the controls in language, fluency, and memory functions (p<0.05). Abnormal performance based on the ECAS total score was noted in 39.4% of patients, and 66.6% presented behavioral changes in at least one domain. Significant correlations were observed between the scores of the ECAS-K and those of other cognitive screening tools (MoCA and FAB, with correlation coefficients of 0.69 and 0.55, respectively; p<0.01). @*Conclusions@#We developed and validated the ECAS-K which could be used as an effective tool to screen the cognitive and behavioral impairments in Korean patients with ALS.

Diagnostic Approach to the Suspected Cases of Hereditary Spastic Paraplegia / 대한신경근육질환학회지

Hereditary spastic paraplegia (HSP) is a heterogeneous group of monogenic neurodegenerative disorders characterized by progressive spasticity of the lower limbs. The clinical features and imaging abnormalities vary greatly according to the affected genes. HSP is classified clinically as pure and complex forms, depending upon the presence or absence of additional neurological defects other than spastic lower limbs. Despite the recent advances in next-generation sequencing technology and its wide availability, a genetic diagnosis of HSP is still not made in more than half of all suspected cases of HSP. In this review, we summarized the various phenotypes of relatively common HSP in clinical practice according to the inheritance pattern, highlighting their clinical, radiological, and neurophysiological features. We further discussed the practical approach to patients with suspected HSP in the current era of next-generation sequencing.

Associations between HLA-A, -B, and -C alleles and iodinated contrast media– induced hypersensitivity in Koreans

A common cause of drug hypersensitivity reactions is iodinated contrast media (ICM). ICM-induced hypersensitivity had been considered to be a non-immunological reaction, but evidence for an immunological mechanism has increased recently. Thus, we evaluated whether HLA-A, -B, and -C alleles were associated with ICM-induced hypersensitivity. In total, 126 patients who underwent contrast-enhanced computed tomography studies through outpatient clinics at a tertiary referral hospital between 2008 and 2012 were assessed. Sixty-one patients experienced ICM-induced hypersensitivity and the remainder, 65, were ICM-tolerant patients (control). ICM-induced hypersensitivity patients showed 51 with immediate, 7 with non-immediate, 3 with both or mixed type. HLA-A, -B, and -C genotyping was performed using a PCR sequence-based typing method. Four kinds of ICM were used: iopromide, iohexol, iobitridol, and iodixanol. The most used ICM among the hypersensitivity patients was iopromide. Significant difference in the frequency of HLA-B*58:01 (odds ratios [OR], 3.90; p = 0.0200, 95% confidence interval [CI], 1.16–13.07) was observed between ICM-induced immediate hypersensitivity and control. There were statistically significant differences in the frequencies of the HLA-B*38:02 (OR, 10.24; p = 0.0145; 95% CI, 1.09–96.14) and HLA-B*58:01 (OR, 3.98; p = 0.0348; 95% CI, 1.03–15.39) between iopromide-induced immediate hypersensitivity and control. The mechanism of ICM-induced hypersensitivity remains unknown, but this study showed associations, although weak, with HLA-B*58:01 alleles for ICM-induced immediate hypersensitivity and HLA-B*38:02 and HLA-B*58:01 for iopromideinduced immediate hypersensitivity as risk predictors. Further studies are needed to validate the associations in larger samples and to identify the functional mechanism behind these results.

CT Follow-Up of Postoperative Bronchopleural Fistula:Risk Factors for Progression to Chronic Complicated Infection

Purpose@#We evaluated the risk factors for progression to chronic complicated bronchopleural fistula (BPF) after pulmonary resection using follow-up CT. @*Materials and Methods@#We retrospectively reviewed 45 cases with BPF that had undergone pulmonary resection during 2010-2018. We compared the clinical and radiological characteristics of those with complicated BPF (n = 24) and those without complicated (sterilized) BPF (n = 21). The clinical and radiological risk factors for progression to chronic complicated BPF were examined by logistic regression analysis. @*Results@#The thickness of the pleural cavity wall (p = 0.022), the size of the pleural cavity (p = 0.029), and the size increase of BPF on follow-up (p = 0.012) were significantly different between the two groups. The risk factors for progression to chronic complicated BPF were age > 70 years (odds ratio, 6.43; 95% confidence interval, 1.2–33.7), the thickness of the cavity wall > 5 mm (odds ratio, 52.5; 95% confidence interval, 5.1–545.4), and an increase in the size of the pleural cavity on follow-up CT (odds ratio, 12.5; 95% confidence interval, 2.1–73.5), only in the univariate analysis. @*Conclusion@#The risk factors for progression to chronic complicated BPF can be evaluated using follow-up CT.

Diagnostic Approach to the Suspected Cases of Hereditary Spastic Paraplegia / 대한신경근육질환학회지

Hereditary spastic paraplegia (HSP) is a heterogeneous group of monogenic neurodegenerative disorders characterized by progressive spasticity of the lower limbs. The clinical features and imaging abnormalities vary greatly according to the affected genes. HSP is classified clinically as pure and complex forms, depending upon the presence or absence of additional neurological defects other than spastic lower limbs. Despite the recent advances in next-generation sequencing technology and its wide availability, a genetic diagnosis of HSP is still not made in more than half of all suspected cases of HSP. In this review, we summarized the various phenotypes of relatively common HSP in clinical practice according to the inheritance pattern, highlighting their clinical, radiological, and neurophysiological features. We further discussed the practical approach to patients with suspected HSP in the current era of next-generation sequencing.

Medical students’ thought process while solving problems in 3 different types of clinical assessments in Korea: clinical performance examination, multimedia case-based assessment, and modified essay question / 보건의료교육평가

Purpose@#This study aimed to explore students’ cognitive patterns while solving clinical problems in 3 different types of assessments—clinical performance examination (CPX), multimedia case-based assessment (CBA), and modified essay question (MEQ)—and thereby to understand how different types of assessments stimulate different patterns of thinking. @*Methods@#A total of 6 test-performance cases from 2 fourth-year medical students were used in this cross-case study. Data were collected through one-on-one interviews using a stimulated recall protocol where students were shown videos of themselves taking each assessment and asked to elaborate on what they were thinking. The unit of analysis was the smallest phrases or sentences in the participants’ narratives that represented meaningful cognitive occurrences. The narrative data were reorganized chronologically and then analyzed according to the hypothetico-deductive reasoning framework for clinical reasoning. @*Results@#Both participants demonstrated similar proportional frequencies of clinical reasoning patterns on the same clinical assessments. The results also revealed that the three different assessment types may stimulate different patterns of clinical reasoning. For example, the CPX strongly promoted the participants’ reasoning related to inquiry strategy, while the MEQ strongly promoted hypothesis generation. Similarly, data analysis and synthesis by the participants were more strongly stimulated by the CBA than by the other assessment types. @*Conclusion@#This study found that different assessment designs stimulated different patterns of thinking during problem-solving. This finding can contribute to the search for ways to improve current clinical assessments. Importantly, the research method used in this study can be utilized as an alternative way to examine the validity of clinical assessments.

The Relationship between Anti-Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease and the Rubella Virus

No abstract available.

Developing the Korean Association of Medical Colleges graduate outcomes of basic medical education based on “the role of Korean doctor, 2014” / 한국의학교육

The Korean Association of Medical Colleges (KAMC) developed graduate outcomes based on “The role of Korean doctor, 2014” to serve as guidelines regarding outcome-based education in Korea. The working group in this study analyzed 65 competencies proposed in “The role of Korean doctor, 2014” according to the developmental principle that certain outcomes should be demonstrated at the point of entry into the graduate medical education. We established 34 competencies as “preliminary graduate outcomes” (PGOs). The advisory committee consisted of 11 professors, who reviewed the validity of PGOs. Ultimately, a total of 19 “revised graduate outcomes” (RGOs) were selected. We modified the RGOs based on opinions from medical schools and a public hearing. In November 2017, the KAMC announced the “graduate outcomes for basic medical education,” which serves as a guide for basic medical education for the 40 medical schools throughout Korea. Medical schools can expand the graduate outcomes according to their educational goals and modify them according to their own context. We believe that graduate outcomes can be a starting point for connecting basic medical education to graduate medical education.

Proximal Median Neuropathy Caused by Axillary Brachial Plexus Block

An axillary brachial plexus block (BPB) is commonly used in local anesthesia, especially for hand surgery. Infraclavicular brachial plexopathy is a potential complication of axillary BPB. A 44-year-old man with an injury to his left third fingertip presented with weakness of the left thumb and index finger flexion after orthopedic surgery under axillary BPB. This was a rare case of proximal median neuropathy caused by axillary BPB. The diagnosis was confirmed by a detailed neurological examination and electrodiagnostic studies.

A Case of Solitary Brain Metastasis from Uterine Mullerian Adenosarcoma with Sarcomatous Overgrowth

Uterine adenosarcoma (AS) are rare tumors and have more favorable outcomes than the aggressive uterine carcinosarcomas. Uterine adenosarcoma with sarcomatous overgrowth (ASSO) is a variant form of AS and exhibits aggressive growth of tumor and the prognosis is relatively poor compared with typical AS. Usually patterns of metastasis have been known to behave like endometrial carcinoma and spread through the lymphatics. Brain metastasis from uterine AS is extremely rare. Herein, we report a case of successfully surgically removed solitary brain metastasis without any extracranial recurrence from uterine ASSO after 4 years of primary treatment.

Multiple Symmetric Lipomatosis Presenting with Bilateral Brachial Plexopathy

No abstract available.

Multidrug-Resistant Tuberculosis Presenting as Miliary Tuberculosis without Immune Suppression: A Case Diagnosed Rapidly with the Genotypic Line Probe Assay Method / 결핵및호흡기질환

Miliary tuberculosis (TB) is a rare extrapulmonary form of TB, and there have been only two reports of miliary TB associated with infection with multidrug-resistant (MDR)-TB pathogen in an immunocompetent host. A 32-year-old woman was referred to our hospital because of abnormal findings on chest X-ray. The patient was diagnosed with MDR-TB by a line probe assay and was administered proper antituberculous drugs. After eight weeks, a solid-media drug sensitivity test revealed that the pathogen was resistant to ethambutol and streptomycin in addition to isoniazid and rifampicin. The patient was then treated with effective antituberculous drugs without delay after diagnosis of MDR-TB. To the best of our knowledge, this is the first case of miliary TB caused by MDR-TB pathogen in Korea.

Evaluation of Cardiovascular Anomalies in Patients with Asymptomatic Turner Syndrome Using Multidetector Computed Tomography

Turner syndrome is well known to be associated with significant cardiovascular abnormalities. This paper studied the incidence of cardiovascular abnormalities in asymptomatic adolescent patients with Turner syndrome using multidetector computed tomography (MDCT) instead of echocardiography. Twenty subjects diagnosed with Turner syndrome who had no cardiac symptoms were included. Blood pressure and electrocardiography (ECG) was checked. Cardiovascular abnormalities were checked by MDCT. According to the ECG results, 11 had a prolonged QTc interval, 5 had a posterior fascicular block, 3 had a ventricular conduction disorder. MDCT revealed vascular abnormalities in 13 patients (65%). Three patients had an aberrant right subclavian artery, 2 had dilatation of left subclavian artery, and others had an aortic root dilatation, aortic diverticulum, and abnormal left vertebral artery. As for venous abnormalities, 3 patients had partial anomalous pulmonary venous return and 2 had a persistent left superior vena cava. This study found cardiovascular abnormalities in 65% of asymptomatic Turner syndrome patients using MDCT. Even though, there are no cardiac symptoms in Turner syndrome patients, a complete evaluation of the heart with echocardiography or MDCT at transition period to adults must be performed.

Concurrent Diagnosis of Pulmonary Metastasis of Malignant Mixed Mullerian Tumor and Small Cell Lung Cancer / 결핵및호흡기질환

A patient who has multiple lung masses with a history of malignancy in organs other than the lung is more likely to be diagnosed with metastatic rather than primary lung cancer. Rarely, metastatic cancer can coexist with primary. We experienced a case of concurrent diagnosis of primary small cell lung cancer and pulmonary metastasis of uterine malignant mixed Mullerian tumor (MMMT). The patient was a 52-year-old female with femur fracture and multiple lung masses with a history of an operation for uterine MMMT. The small cell lung cancer was diagnosed by bronchoscopic biopsy. The central lung mass decreased after chemotherapy for small cell lung cancer but multiple peripheral masses increased. A percutaneous biopsy for one of peripheral masses revealed metastatic uterine MMMT. We suggest that we have to consider the possible presence of concomitant malignancies of different origins in one organ especially with patients who had a history of malignancy in another organ.

Concurrent Diagnosis of Pulmonary Metastasis of Malignant Mixed Mullerian Tumor and Small Cell Lung Cancer / 결핵및호흡기질환

A patient who has multiple lung masses with a history of malignancy in organs other than the lung is more likely to be diagnosed with metastatic rather than primary lung cancer. Rarely, metastatic cancer can coexist with primary. We experienced a case of concurrent diagnosis of primary small cell lung cancer and pulmonary metastasis of uterine malignant mixed Mullerian tumor (MMMT). The patient was a 52-year-old female with femur fracture and multiple lung masses with a history of an operation for uterine MMMT. The small cell lung cancer was diagnosed by bronchoscopic biopsy. The central lung mass decreased after chemotherapy for small cell lung cancer but multiple peripheral masses increased. A percutaneous biopsy for one of peripheral masses revealed metastatic uterine MMMT. We suggest that we have to consider the possible presence of concomitant malignancies of different origins in one organ especially with patients who had a history of malignancy in another organ.

Retrospective Analysis of Adverse Reactions to Iodinated Contrast Media in Korean

BACKGROUND: This study was performed to evaluate the prevalence and risk factors on the adverse reactions caused by iodinated contrast media (CM) for computed tomography (CT) examination in a university hospital. METHODS: Clinical and demographic data among outpatients with CM use were collected at 0000 University Busanpaik Hospital in Busan, Korea between 2008 and 2010. Adverse reaction rate was calculated by the number of adverse reaction among total outpatients with CM use, which was stratified by seasons and sex. The association of risk factors on adverse reaction was investigated using logistic regression model. RESULTS: The total outpatients and events of administered CMs were 27,587 and 48,616, respectively. The administered CMs were iopromide, iohexol, iobitridol, and iodixanol. Adverse reactions occurred in 300 outpatients among the total outpatients (1.1 %). The number of outpatients administered CM more than twice were 8,348. Among them, outpatients who experienced adverse reaction(s) more than once and twice were 124 (1.5 %) and 26 (0.3 %), respectively. Adverse reaction rate was significantly different by sex(p=0.01). The other risk factors were cancer history (OR 2.57, 95 % CI 2.00-3.31) and previous CM administration (OR 1.89, 95 % CI 1.47-2.44). Urticaria was the most frequent symptoms. CONCLUSION: Total adverse reaction rate was 1.1 % with most common symptom in skin system. Related risk factors were female, cancer history, and previous CM administration. These results were similar to previous studies and will be contribute to clinical practice and future research especially in Koreans.

Retrospective Analysis of Adverse Reactions to Iodinated Contrast Media in Korean

BACKGROUND: This study was performed to evaluate the prevalence and risk factors on the adverse reactions caused by iodinated contrast media (CM) for computed tomography (CT) examination in a university hospital. METHODS: Clinical and demographic data among outpatients with CM use were collected at 0000 University Busanpaik Hospital in Busan, Korea between 2008 and 2010. Adverse reaction rate was calculated by the number of adverse reaction among total outpatients with CM use, which was stratified by seasons and sex. The association of risk factors on adverse reaction was investigated using logistic regression model. RESULTS: The total outpatients and events of administered CMs were 27,587 and 48,616, respectively. The administered CMs were iopromide, iohexol, iobitridol, and iodixanol. Adverse reactions occurred in 300 outpatients among the total outpatients (1.1 %). The number of outpatients administered CM more than twice were 8,348. Among them, outpatients who experienced adverse reaction(s) more than once and twice were 124 (1.5 %) and 26 (0.3 %), respectively. Adverse reaction rate was significantly different by sex(p=0.01). The other risk factors were cancer history (OR 2.57, 95 % CI 2.00-3.31) and previous CM administration (OR 1.89, 95 % CI 1.47-2.44). Urticaria was the most frequent symptoms. CONCLUSION: Total adverse reaction rate was 1.1 % with most common symptom in skin system. Related risk factors were female, cancer history, and previous CM administration. These results were similar to previous studies and will be contribute to clinical practice and future research especially in Koreans.

A Case of a Human Tail Without Associated Anomalies / 대한소아신경학회지

Human tails or caudal cutaneous appendages are rare congenital anomalies with lesions usually arising from the midline of the lumbosacrococcygeal area. There have been only around 70 cases reported in literature. A 4-month-old male infant born as the 1st child of a dizygotic twin was presented with a penis-shaped mass of 4-cm in the coccygeal area. The mass was entirely covered by skin without any skin defect and was connected to the midline skin of the posterior coccygeal area by a stalk. Magnetic resonance images showed fat density of the mass without any demonstrable communication with the spinal canal nor any underlying central nervous system anomalies. The patient underwent a simple surgical excision of the mass without residual effects and complications. Histopathologic examination of the mass revealed lobules of mature adipose tissue, fibromuscular soft tissue, small vessels and nerves with normal hair follicles on the skin.

Transvenous Embolization of Cavernous Sinus Dural Arteriovenous Fistula Using the Direct Superior Ophthalmic Vein Approach: A Case Report / 신경중재치료의학

Transvenous coil embolization has been successfully applied for the treatment of cavernous sinus dural arteriovenous fistula (CSDAVF). Unfortunately, the technique cannot be applied in cases of poor or absent inferior petrosal sinus or facial venous access route to the fistula. Recently, we experienced a successful embolization using direct superior ophthalmic vein approach in cases of CSDAVF which were no opacification of inferior petrosal sinus or facial vein.

NK-T cell lymphoma manifesting as acute respiratory distress syndrome / 대한내과학회지

Primary pulmonary lymphoma is a rare disease, and non-B cell lymphomas (T-cell and natural killer cell lymphomas) involving the lung parenchyma are uncommonly reported. The most common radiological feature of pulmonary parenchymal lymphoma is a single mass or nodule. A 49-year-old woman with dyspnea was referred with suspicion of severe pneumonia. A chest radiograph showed diffuse nodular infiltration in both lungs. Acute respiratory failure was severe and rapidly progressive, so she was managed with a mechanical ventilator under the impression of acute respiratory distress syndrome (ARDS). A bronchoalveolar lavage and lung biopsy by video-assisted thoracic surgery revealed NK-T cell lymphoma. We report a case of extranodal NK-T cell lymphoma presenting as ARDS.