• Annals of Clinical Neurophysiology
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• 제16권2호
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• pp.70-73
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• 2014

Nonconvulsive status epilepticus usually presents with altered mentation without distinct manifestations of seizures. It may be related with various medical disorders. Hashimoto's encephalopathy is characterized by various neurological manifestations accompanied by high titers of anti-thyroid antibodies. Here, we report a patient with nonconvulsive status epilepticus caused by Hashimoto's encephalopathy who showed a dramatic response to steroids.

• Journal of Korean Dental Science
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• 제11권2호
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• pp.82-85
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• 2018

Leukemia is a hematological malignant disease with various clinical symptoms. Due to the fatal nature of the disease, early detection is important. Oral manifestations include ulcers and gingival enlargement with bleeding. Moreover, myeloid sarcoma or opportunistic infections may also occur. This report introduces a 31-year-old male presenting with generalized gingival enlargement with bleeding and another 81-year-old female with neoplasm on the left retromolar area. Both were diagnosed as acute monocytic leukemia. These cases implicate that gingival enlargement or mucosal lesion in the oral cavity may represent underlying systemic diseases. Related to this, it has to be reminded that making timely diagnosis and referral according to the clinical findings is crucial.

• Journal of Yeungnam Medical Science
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• 제36권1호
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• pp.8-15
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• 2019

Connective tissue diseases (CTDs) can affect all compartments of the lungs, including airways, alveoli, interstitium, vessels, and pleura. CTD-associated lung diseases (CTD-LDs) may present as diffuse lung disease or as focal lesions, and there is significant heterogeneity between the individual CTDs in their clinical and pathological manifestations. CTD-LDs may presage the clinical diagnosis a primary CTD, or it may develop in the context of an established CTD diagnosis. CTD-LDs reveal acute, chronic or mixed pattern of lung and pleural manifestations. Histopathological findings of diverse morphological changes can be present in CTD-LDs airway lesions (chronic bronchitis/bronchiolitis, follicular bronchiolitis, etc.), interstitial lung diseases (nonspecific interstitial pneumonia/fibrosis, usual interstitial pneumonia, lymphocytic interstitial pneumonia, diffuse alveolar damage, and organizing pneumonia), pleural changes (acute fibrinous or chronic fibrous pleuritis), and vascular changes (vasculitis, capillaritis, pulmonary hemorrhage, etc.). CTD patients can be exposed to various infectious diseases when taking immunosuppressive drugs. Histopathological patterns of CTD-LDs are generally nonspecific, and other diseases that can cause similar lesions in the lungs must be considered before the diagnosis of CTD-LDs. A multidisciplinary team involving pathologists, clinicians, and radiologists can adequately make a proper diagnosis of CTD-LDs.

• 대한유전성대사질환학회지
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• 제23권2호
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• pp.21-30
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• 2023

Inherited metabolic disorders (IMD) are a group of disorders caused by defects in specific biochemical pathways. Up to 85% of IMD display predominantly neurological manifestations by affecting neurodevelopment or causing neurodegeneration. These neurometabolic disorders present with a variety of neurological and non-neurological manifestations. Early diagnosis of IMD is important because some disorders can be treated or improved with specific treatment if detected early. For prompt diagnosis and treatment, it is important to suspect IMD by being familiar with the clinical characteristics, biochemical abnormalities, and characteristic neuroimaging patterns that appear in IMD. Genetic testing, including next-generation sequencing, is also important in diagnosing IMD. During the follow-up of patients with IMD, it is necessary to conduct regular physical and neurological examinations in addition to disease-specific management.

• Annals of Clinical Neurophysiology
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• 제25권2호
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• pp.78-83
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• 2023

Nociplastic pain refers to pain arising from altered nociception without evidence of tissue or somatosensory damage. It encompasses various clinical conditions with shared neurophysiological mechanisms involving different organ systems. Nociplastic pain can occur independently or alongside chronic pain conditions with a nociceptive or neuropathic origin. This review introduces the concept of nociplastic pain, its clinical manifestations and the underlying pathophysiology. Taking a biopsychosocial approach can lead to a better understanding of nociplastic pain and improved treatment outcomes for affected individuals.

• BMB Reports
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• 제39권3호
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• pp.229-239
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• 2006

Sj$\"{o}$gren's syndrome (SS) is an autoimmune disorder that affects the salivary glands, leading to xerostomia, and the lacrimal glands, resulting in xerophthalmia. Secondary SS is associated with other autoimmune disorders such as systemic rheumatic diseases and systemic lupus erythematosis (SLE), which can affect multiple organs, including the epidermis. Recent studies have demonstrated that green tea polyphenols (GTPs) possess both anti-inflammatory and anti-apoptotic properties in normal human cells. Epidemiological evidence has indicated that, in comparison to the United States, the incidence of SS, clinical xerostomia and lupus is considerably lower in China and Japan, the two leading green tea-consuming countries. Thus, GTPs might be responsible, in part, for geographical differences in the incidence of xerostomia by reducing the initiation or severity of SS and lupus. Consistent with this, molecular, cellular and animal studies indicate that GTPs could provide protective effects against autoimmune reactions in salivary glands and skin. Therefore, salivary tissues and epidermal keratinocytes could be primary targets for novel therapies using GTPs. This review article evaluates the currently available research data on GTPs, focusing on their potential application in the treatment of the oral manifestations of SS and skin manifestations of SLE.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제24권2호
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• pp.197-206
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• 2021

Purpose: Celiac disease (CD) is a common autoimmune disease with extra-intestinal manifestations, including neurological disorders. There are few reports to assess various factors in increasing the chances of developing neurological disorders in CD, so we designed this study. Methods: All patients with CD at any age who had been referred to the Celiac Clinic were evaluated for neurological problems. CD was defined as IgA anti-transglutaminase antibodies (anti-tTG) of 18 IU/mL or higher in serology and Marsh type I or more severe in histopathological evaluation. Logistic regression analysis was used to evaluate the impact of various independent variables on the neurological manifestations. Results: A total of 540 patients enrolled in this study. A 360 (66.7%) of patients were children. A 64.8% and 35.2% were female and male, respectively. Overall, 34.1% of patients had neurological manifestation, including headache, neuropathy, epilepsy, and ataxia. The odds of developing neurological manifestations in children were significantly lower than in adults (odds ratio [OR], 0.66; 95% confidence interval [CI], 0.45-0.96; p=0.03) and in patients with gastrointestinal (GI) symptoms significantly higher than in the group without GI manifestations (OR, 1.77; 95% CI, 1.18-2.63; p=0.005). Other variables, including Marsh classification (OR, 0.44; 95% CI, 0.18-1.11; p=0.08) and anti-tTG levels (OR, 1.00; 95% CI, 0.999-1.001; p=0.59) did not significantly increase the chances of developing neurological disorders. Conclusion: Our study showed that increasing age and the presence of GI symptoms, but not serological and histological findings, could increase the chances of developing neurological diseases in CD patients.

• Clinical and Experimental Pediatrics
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• 제55권2호
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• pp.48-53
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• 2012

Purpose: Gaucher disease is caused by a ${\beta}$-glucocerebrosidase (GBA) deficiency. The aim of this study is to investigate the clinical and genetic characteristics according to subtypes of Gaucher disease in the Korean population. Methods: Clinical findings at diagnosis, $GBA$ mutations, and clinical courses were reviewed in 20 patients diagnosed with Gaucher disease. Results: Eleven patients were diagnosed with non-neuronopathic type, 2 with acute neuronopathic type, and 7 with chronic neuronopathic type. Most patients presented with hepatosplenomegaly, thrombocytopenia, and short stature. In the neuronopathic group, variable neurological features, such as seizure, tremor, gaze palsy, and hypotonia, were noted at age $8.7{\pm}4.3$ years. B cell lymphoma, protein-losing enteropathy, and hydrops fetalis were the atypical manifestations. Biomarkers, including chitotriosidase, acid phosphatase, and angiotensin-converting enzyme, increased at the initial evaluation and subsequently decreased with enzyme replacement treatment (ERT). The clinical findings, including hepatosplenomegaly, thrombocytopenia, and skeletal findings, improved following ERT, except for the neurological manifestations. L444P was the most common mutation in our cohort. One novel mutation, R277C, was found. Conclusion: Although the clinical outcome for Gaucher disease improved remarkably following ERT, the outcome differed according to subtype. Considering the high proportion of the neuronopathic form in the Korean population, new therapeutic strategies targeting the central nervous system are needed, with the development of a new scoring system and biomarkers representing clinical courses in a more comprehensive manner.

• 대한유전성대사질환학회지
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• 제15권3호
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• pp.147-154
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• 2015

목적: 우리나라 케톤분해이상질환군의 연령별분포, 임상양상 등을 알아보고자 본 연구를 시행하였다. 방법: 2007년 1월부터 2015년 9월 사이에 소변유기산분석을 시행하였던 2,794명의 환자 중에서 반복부하 유기산분석으로 진단된 67명의 케톤분해이상질환군환자들의 임상양상을 분석하였다. 결과: 케톤분해이상질환군 환자의 분포는 신생아기 1명, 영아기 30명, 소아기 31명, 청소년기 5명으로 나타났고, 가장 많은 주증상은 경련발작이었으며 발달지연을 동반한 경련발작, 발달지연 등이 뒤를 이었다. 결론: 우리나라의 발병빈도는 다른 나라에 비하여 높은 것으로 판단되며, 소아청소년과 의사들의 관심이 필요하다고 판단된다.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제23권5호
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• pp.435-446
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• 2001

Natural Killer/T-cell(NK/TC) lymphoma is a rare disease of oral and maxillofacial region with an aggressive clinical course, showed unusual clinical manifestations. Prognosis is generally poor and the disease is invariably fatal after systemic dissemination. A case of nasal NK/TC non-Hodgkin's lymphoma in the left maxilla that showed unusual clinical manifestations and a fulminant course of disease, are described with literature reviews. A 81-year-old female patient presented with rapidly growing ulceration and general malaise after extraction of right upper second premolar NK/TC lymphoma was subsequently detected by biopsy. rapid and wide invasion from maxilla to mandible developed within 10 days and multiple metastasis to whole body was after a few weeks. Although the time relationship is not clear, local invasion and multiple metastasis could be dissemination from localized disease of NK/TC. As seen in my case, the course can be excessively aggressive and fulminant even though it first appeared as a localized ulceractive lesion. She is expired 2 months after biopsy. Positivity of immunohistochemical stain (CD56, LCA, UCHL-1, CD3), which is a specific characteristic of NK/TC, may serve as a factor showing a poor prognosis of a malignant lymphoma