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Updates in postural tachycardia syndrome
문장섭 대한임상신경생리학회 2021 Annals of Clinical Neurophysiology Vol.23 No.1
Postural tachycardia syndrome (POTS) is the most common form of orthostatic intolerance in young people. However, it is still considered an underrecognized disorder and so deserves more attention from clinicians. This review covers the diagnostic challenges, correlations between the symptoms, evidence of autoimmune involvement in the pathogenesis, and treatment strategies in POTS.
뇌미세혈관병증 3가지 표식과 열공뇌경색 초기 신경학적 악화와의 관련성
문장섭,김나영,강지훈,양미화,장명숙,한문구,배희준 대한신경과학회 2012 대한신경과학회지 Vol.30 No.4
Background: Neurological deterioration following acute lacunar infarction is not uncommon. Its association with poor clinical outcome is well-known, but little is known about what causes it. This study aimed to elucidate whether 3 stigmas of cerebral microangiopathy, a pathogenesis of lacunar infarction, are associated with neurological deterioration in patients with acute lacunar infarction. Methods: Patients with acute lacunar infarction who were admitted within 24 hours of onset were identified using a prospective stroke registry. Patients who presented neurological deterioration within 7 days of hospitalization (progressive lacune group) were matched to 4 controls (non-progressive lacune group) for ‘onset to arrival time’. Three stigmas of cerebral microangiopathy (leukoaraiosis, cerebral microbleeds, and silent lacunes) were measured using initial brain MRI, and their associations with neurological deterioration were analyzed. Results: During 45 months, a total of 23 patients were identified and matched to 80 controls. Simple comparison of 2groups showed that those 3 stigmas of cerebral microangiopathy were not significantly associated with neurological deterioration. Hyperlipidemia (p=0.18), history of transient ischemic attack or stroke (p=0.01), initial NIH stroke scale (p=0.07), white blood cell counts (p=0.16), and lesion volume (p=0.03) were possibly different (p’s<0.2) between 2 groups. Multivariable logistic regression analysis did not reveal any significant association of those 3 stigmas with neurological deterioration, too (all p values>0.5). Conclusions: This study did not find a relationship between cerebral microangiopathy and neurological deterioration following acute lacunar infarction. The possibility of inadequate power should be noted.
Case of Rickettsia typhi-induced Brain Abscess Mimicking Brain Tumor
장윤혁,문장섭,Jin-Sun Jun,Tae-joon Kim,Kyung-Il Park,Soon-Tae Lee,Keun-Hwa Jung,이상건,주건 질병관리본부 2018 Osong Public Health and Research Persptectives Vol.9 No.3
Murine typhus is one of the most prevalent rickettsial infections in the world, caused by the bacterial genus Rickettsia. Though the disease manifests a relatively benign clinical course with fever, rash, and headache being the 3 classic symptoms, neurological complications may arise in patients that could become permanent. In this case study, a patient with a brain abscess caused by R typhi infection is described. Based upon the recent reemergence of arthropod-borne disease, the findings in this case are significant; R typhi can cause a brain abscess that mimics a brain tumor, which delays the diagnosis and appropriate management of the disease. Murine typhus should always be considered when performing the differential diagnosis of brain abscesses in South Korea.
Applications of CRISPR/Cas9 for Gene Editing in Hereditary Movement Disorders
임우석,문장섭,김만호 대한파킨슨병및이상운동질환학회 2016 Journal Of Movement Disorders Vol.9 No.3
Gene therapy is a potential therapeutic strategy for treating hereditary movement disorders, including hereditary ataxia, dystonia, Huntington’s disease, and Parkinson’s disease. Genome editing is a type of genetic engineering in which DNA is inserted, deleted or replaced in the genome using modified nucleases. Recently, clustered regularly interspaced short palindromic repeat/CRISPR associated protein 9 (CRISPR/Cas9) has been used as an essential tool in biotechnology. Cas9 is an RNA-guided DNA endonuclease enzyme that was originally associated with the adaptive immune system of Streptococcus pyogenes and is now being utilized as a genome editing tool to induce double strand breaks in DNA. CRISPR/Cas9 has advantages in terms of clinical applicability over other genome editing technologies such as zinc-finger nucleases and transcription activator-like effector nucleases because of easy in vivo delivery. Here, we review and discuss the applicability of CRISPR/Cas9 to preclinical studies or gene therapy in hereditary movement disorders
Prognostic Value of Initial Standard EEG and MRI in Patients with Herpes Simplex Encephalitis
김영수,정근화,이순태,강봉수,염정숙,문장섭,신정원,이상건,주건 대한신경과학회 2016 Journal of Clinical Neurology Vol.12 No.2
Background and Purpose Herpes simplex encephalitis (HSE) is the most common type of sporadic encephalitis worldwide, and it remains fatal even when optimal antiviral therapy is applied. Tere is only a weak consensus on the clinical outcomes and prognostic factors in patients with HSE. Tis study examined whether the radiological and electrophysiological fndings have a prognostic value in patients with HSE. Methods We retrospectively analyzed patients who were diagnosed with HSE by applying the polymerase chain reaction to cerebrospinal fuid and who received intravenous acyclovir at our hospital from 2000 to 2014. We evaluated the clinical outcomes at 6 months afer onset and their correlations with initial and clinical findings, including the volume of lesions on MRI, the severity of EEG fndings, and the presence of epileptic seizures at the initial presentation. Results Twenty-nine patients were enrolled (18 men and 11 women). Univariate analysis revealed that the presence of severe EEG abnormality and epileptic seizures at the initial presentation were signifcant correlated with a poor clinical outcome at 6 months (p=0.005 and p=0.009, respectively). In multivariate analysis, the presence of severe EEG abnormality was the only independent predictor of a poor outcome at 6 months (p=0.006). Conclusions In cases of HSE, the initial EEG severity and seizure presentation may be useful predictive factors for the outcome at 6 months afer acyclovir treatment.
Anti-N-Methyl-D-Aspartate Receptor Encephalitis in Korea: Clinical Features, Treatment, and Outcome
임정아,이순태,정근화,김소연,신정원,문장섭,변정익,김태준,신용원,이건주,김영수,박경일,이상건,주건 대한신경과학회 2014 Journal of Clinical Neurology Vol.10 No.2
Background and Purpose Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitisis the most common type of autoimmune synaptic encephalitis and it often responds to treatment. We analyzed the clinical characteristics of anti-NMDAR encephalitis in Korea. Methods Serum and/or cerebrospinal fluid (CSF) of adult patients (aged ≥18 years) with encephalitis of undetermined cause were screened for anti-NMDAR antibodies using a cell-basedindirect immunofluorescence assay. The patients came from 41 university hospitals. Results Of the 721 patients screened, 40 were identified with anti-NMDAR antibodies andclinical details of 32 patients were obtained (median age, 41.5 years; 15 females). Twenty-twopatients (68.8%) presented with psychiatric symptoms, 16 (50%) with seizures, 13 (40.6%)with movement disorders, 15 (46.9%) with dysautonomia, 11 (34.4%) with memory disturbance, and 11 (34.4%) with speech disturbance. Magnetic resonance imaging, electroencephalography, and CSF examinations yielded nonspecific findings. Tumor information was onlyavailable for 22 patients: 5 patients had tumors, and 2 of these patients had ovarian teratomas. Twenty-two patients received immunotherapy and/or surgery, and therapeutic responses wereanalyzed in 21 patients, of which 14 (66.7%) achieved favorable functional outcomes (score onthe modified Rankin Scale of 0–2). Conclusions This study investigated the clinical characteristics of adult anti-NMDAR encephalitis in Korea. Currently, elderly patients who do not have tumors are commonly diagnosed with this condition. Understanding the detailed clinical characteristics of this disease willimprove the early detection of anti-NMDAR encephalitis in patients both young and old.
First Cases of Spinocerebellar Ataxia 42 in Two Korean Families
손효신,Jihoon G. Yoon,Man Jin Kim,문장섭,김한준 대한파킨슨병및이상운동질환학회 2023 Journal Of Movement Disorders Vol.16 No.1
we identified the first Korean cases of SCA42 in two unrelated families. This report highlights the importance of NGS testing in Korean patients with ataxia who remain undiagnosed after testing for tandem repeat disorders. As the prevalence of SCA42 in each ethnicity or country is unknown, further studies are warranted to unravel the genetic characteristics of this disease.
Sung Kwon Kim,Ji Eun Park,Kyung Hwan Kim,Jin Mo Cho,문장섭,Wan-Soo Yoon,김세훈,김영일,김영준,Ho Sung Kim,도윤식,박재성,윤홍인,서영범,Kyoung-Su Sung,송진호,Chan Woo Wee,Se-Hoon Lee,임도훈,임정호,장종희,한명훈,홍제범,Kihwan Hwang,Chul-Kee Park 대한뇌종양학회 2020 Brain Tumor Research and Treatment Vol.8 No.1
Background: The Guideline Working Group of the Korean Society for Neuro-Oncology (KSNO) conducted the nationwide questionnaire survey for diverse queries facing to treat patients with brain tumor. As part III of the survey, the aim of this study is to evaluate the national patterns of clinical practice for patients with brain metastasis and primary central nervous system lymphoma (PCNSL). Methods: A web-based survey was sent to all members of the KSNO by email. The survey included 7 questions of brain metastasis and 5 questions of PCNSL, focused on the management strategies in specific situations. All questions were developed by consensus of the Guideline Working Group. Results: In the survey about brain metastasis, respondents preferred surgical resection with adjuvant treatment for patients with a surgically accessible single brain metastatic lesion less than 3 cm in size without extracranial systemic lesions. However, most respondents considered radiosurgery for surgically inaccessible lesions. As the preferred treatment of multiple brain metastases according to the number of brain lesions, respondents tended to choose radiotherapy with increasing number of lesions Radiosurgery was mostly chosen for the brain metastases of less than or equal to 4. In the survey about PCNSL, a half of respondents choose high-dose methotrexate-based polychemotherapy as the first-line induction therapy for PCNSL. The consolidation and salvage therapy showed a little variation among respondents. For PCNSL patients with cerebrospinal fluid dissemination, intrathecal chemotherapy was most preferred. Conclusion: The survey demonstrates the prevailing clinical practice patterns for patients with brain metastasis and PCNSL among members of the KSNO. This information provides a point of reference for establishing a practical guideline in the management of brain metastasis and PCNSL.