• 제목/요약/키워드: neurological disease

검색결과 576건 처리시간 0.024초

고셔병에서 샤프론 치료 (Chaperone Therapy in Gaucher Disease)

  • 이범;허선희;전종근;김유미;김자혜;최인희;김재민;김구환;유한욱
    • 대한유전성대사질환학회지
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    • 제13권1호
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    • pp.37-42
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    • 2013
  • Gaucher disease is a lysosomal storage disease caused by deficiency of glucocerebrosidase (GBA). This condition is characterized by accumulation of glucocerebrosidase in liver, spleen, lung, skeletal system, and central nervous system. Gaucher disease is the prototype of disease in which efficacy of enzyme replacement therapy has been established. However, because recombinant enzyme is not able to enter the central nervous system, its efficacy is limited to the non-neurological manifestations of Gaucher disease. Importantly, approximately a half of Korean patients with Gaucher disease suffer from neurological manifestations. In addition, Korean Gaucher disease patients exhibit distinct mutation spectrum from those in other populations. Common mutations in Korean patients with Gaucher disease are also associated with neurological phenotype. Therefore, therapeutic strategies tailored to Korean patients were necessary. Interestingly, a chemical chaperone, ambroxol, has been known to increase residual enzymatic activities of the select mutant GBAs encoded by mutations prevalent in Korean patients. One promising aspect of this drug is that it can cross blood-brain barrier, and enhance the enzyme activity in the brain. In vitro study suggested this chemical chaperone as one of new therapeutic agents in Gaucher disease, and a well-designed human trial is required to confirm its efficacy.

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파킨슨병 환자의 통증에 대한 한의치료의 효과 : 후향적 연구 (Effects of Korean Medicine on Pain in Patients with Parkinson's Disease: A Retrospective Study)

  • 정혜선;김하리;김서영;임태빈;진철;권승원;조승연;정우상;문상관;박정미;고창남;박성욱
    • 대한한방내과학회지
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    • 제41권6호
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    • pp.947-958
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    • 2020
  • Objectives: To investigate efficacy of Korean medicine for patients with Parkinson's Disease (PD) with pain. Methods: We performed a retrospective review of the medical records for patients diagnosed with PD between 2012 and 2019 at Gangdong Kyung Hee University Korean Medicine Hospital in South Korea. Results: Twenty-two patients with King's Parkinson's Disease Pain Scale(KPPS) scores at least twice were analyzed for evaluating the efficacy of Korean medicine for pain treatment in PD. The mean total scores before and after Korean medicine treatment were 15.23±1 .01 and 9.2±8.7, respectively, and the mean difference between the before/after total scores was 6.0±5.8 (P<0.001). Specifically, the score of radicular pain was significantly decreased (P=0.048). Conclusions: These findings suggest that Korean medicine could be beneficial for reducing pain associated with PD. Clinical efficacy should be confirmed by further studies, such as large-sample cohort studies and randomized controlled trials to clarify the pathological pain relief mechanism and the analgesic effect of Korean medicine.

A Comparison Study of Magnetic Resonance Imaging Findings and Neurological Signs in Canine Brain Diseases

  • Kim, Min-Ju;Song, Joong-Hyun;Hwang, Tae-Sung;Lee, Hee-Chun;Yu, Do-Hyeon;Kang, Byeong-Teck;Jung, Dong-In
    • 한국임상수의학회지
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    • 제35권5호
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    • pp.178-183
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    • 2018
  • The object of this study was to compare magnetic resonance imaging (MRI) findings and neurological signs in canine brain diseases. Brain diseases can cause severe neurological deficits and may be life-threatening. The antemortem diagnosis of the brain diseases is difficult for the clinician, since definitive diagnosis is based upon histopathological confirmation. Brain diseases are often associated with specific clinical signs, signalment, progression, and location. Accurate lesion localization through neurological examination and MRI findings is helpful for developing a differential diagnosis. A retrospective study was performed to compare the neurological examination of dogs with suspected brain disease to the MRI findings. Based on this study, neurological examination is a reliable way to localize most brain lesions. Postural reaction deficits do not provide sufficient information to localize lesions. Additionally, not all brain lesions present clinical signs and inflammatory lesions may cause no detectable abnormalities on MRI. Therefore, in clinical practice, a combination of neurological examination and MRI findings recommended for accurate brain lesion localization.

뇌자기자극을 이용한 운동신경계의 신경생리학적 평가 (Neurophysiological Evaluation of the Motor System Using Transcranial Magnetic Stimulation)

  • 신혜원;손영호
    • Annals of Clinical Neurophysiology
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    • 제13권1호
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    • pp.1-12
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    • 2011
  • Transcranial magnetic stimulation (TMS) is a non-invasive tool used to study aspects of human brain physiology, including motor function and the pathophysiology of various brain disorders. A brief electric current passed through a magnetic coil produces a high-intensity magnetic field, which can excite or inhibit the cerebral cortex. Although various brain regions can be evaluated by TMS, most studies have focused on the motor cortex where motor evoked potentials (MEPs) are produced. Single-pulse and paired-pulse TMS can be used to measure the excitability of the motor cortex via various parameters, while repetitive TMS induces cortical plasticity via long-term potentiation or long-term depression-like mechanisms. Therefore, TMS is useful in the evaluation of physiological mechanisms of various neurological diseases, including movement disorders and epilepsy. In addition, it has diagnostic utility in spinal cord diseases, amyotrophic lateral sclerosis and demyelinating diseases. The therapeutic effects of repetitive TMS on stroke, Parkinson disease and focal hand dystonia are limited since the duration and clinical benefits seem to be temporary. New TMS techniques, which may improve clinical utility, are being developed to enhance clinical utilities in various neurological diseases.

소아 위식도 역류 질환의 외과적 치료 (Surgical Management of Gastroesophageal Reflux Disease)

  • 김성철;김대연;김경모;김인구
    • Advances in pediatric surgery
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    • 제6권2호
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    • pp.100-105
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    • 2000
  • Eleven children with gastroesophageal reflux disease underwent fundoplication. Eight had neurological impairment, two had hiatal hernias and one had a history of esophageal repair for esophageal atresia. The most common and significant symptom was vomiting(81.8 %), followed by recurrent respiratory infections(72.7 %) and failure to thrive(72.7 %). The most common diagnostic tool was 24 hour esophageal pH study, which showed pH less than 4 for more than 10 % of the total recorded time in 6 of 9 patients. Nissen fundoplication was performed in 10 patients. Thal fundoplication was carried out in one patient with esophageal atresia. Stamm gastrostomy was added for nutritional and/or swallowing problem in all 8 patients with neurological impairment. The median follow up period was 13 months. There was one late death of unrelated cause and one recurrence. The quality of life after antireflux surgery was greatly improved. Antireflux surgery should be done if indicated, and a simultaneous gastrostomy considered in a patient with neurological impairment.

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Computational electroencephalography analysis for characterizing brain networks

  • Sunwoo, Jun-Sang;Cha, Kwang Su;Jung, Ki-Young
    • Annals of Clinical Neurophysiology
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    • 제22권2호
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    • pp.82-91
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    • 2020
  • Electroencephalography (EEG) produces time-series data of neural oscillations in the brain, and is one of the most commonly used methods for investigating both normal brain functions and brain disorders. Quantitative EEG analysis enables identification of frequencies and brain activity that are activated or impaired. With studies on the structural and functional networks of the brain, the concept of the brain as a complex network has been fundamental to understand normal brain functions and the pathophysiology of various neurological disorders. Functional connectivity is a measure of neural synchrony in the brain network that refers to the statistical interdependency between neural oscillations over time. In this review, we first discuss the basic methods of EEG analysis, including preprocessing, spectral analysis, and functional-connectivity and graph-theory measures. We then review previous EEG studies of brain network characterization in several neurological disorders, including epilepsy, Alzheimer's disease, dementia with Lewy bodies, and idiopathic rapid eye movement sleep behavior disorder. Identifying the EEG-based network characteristics might improve the understanding of disease processes and aid the development of novel therapeutic approaches for various neurological disorders.

The Option of Motion Preservation in Cervical Spondylosis: Cervical Disc Arthroplasty Update

  • Chang, Chih-Chang;Huang, Wen-Cheng;Wu, Jau-Ching;Mummaneni, Praveen V.
    • Neurospine
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    • 제15권4호
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    • pp.296-305
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    • 2018
  • Cervical disc arthroplasty (CDA), or total disc replacement, has emerged as an option in the past two decades for the management of 1- and 2-level cervical disc herniation and spondylosis causing radiculopathy, myelopathy, or both. Multiple prospective randomized controlled trials have demonstrated CDA to be as safe and effective as anterior cervical discectomy and fusion, which has been the standard of care for decades. Moreover, CDA successfully preserved segmental mobility in the majority of surgical levels for 5-10 years. Although CDA has been suggested to have long-term efficacy for the reduction of adjacent segment disease in some studies, more data are needed on this topic. Surgery for CDA is more demanding for decompression, because indirect decompression by placement of a tall bone graft is not possible in CDA. The artificial discs should be properly sized, centered, and installed to allow movement of the vertebrae, and are commonly 6 mm high or less in most patients. The key to successful CDA surgery includes strict patient selection, generous decompression of the neural elements, accurate sizing of the device, and appropriately centered implant placement.

Gut-Brain Connection: Microbiome, Gut Barrier, and Environmental Sensors

  • Min-Gyu Gwak;Sun-Young Chang
    • IMMUNE NETWORK
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    • 제21권3호
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    • pp.20.1-20.18
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    • 2021
  • The gut is an important organ with digestive and immune regulatory function which consistently harbors microbiome ecosystem. The gut microbiome cooperates with the host to regulate the development and function of the immune, metabolic, and nervous systems. It can influence disease processes in the gut as well as extra-intestinal organs, including the brain. The gut closely connects with the central nervous system through dynamic bidirectional communication along the gut-brain axis. The connection between gut environment and brain may affect host mood and behaviors. Disruptions in microbial communities have been implicated in several neurological disorders. A link between the gut microbiota and the brain has long been described, but recent studies have started to reveal the underlying mechanism of the impact of the gut microbiota and gut barrier integrity on the brain and behavior. Here, we summarized the gut barrier environment and the 4 main gut-brain axis pathways. We focused on the important function of gut barrier on neurological diseases such as stress responses and ischemic stroke. Finally, we described the impact of representative environmental sensors generated by gut bacteria on acute neurological disease via the gut-brain axis.

베이비붐세대가 65세 노인인구로 전환 시의 만성질환 환자수와 진료비 예측 (Estimate over the Number of Chronic Disease Patients and Medical Care Expenditure at the Time of Transition of Baby Boomer into 65 Years Old Aging Population)

  • 이선영;김영훈;김한성
    • 보건행정학회지
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    • 제23권4호
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    • pp.376-386
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    • 2013
  • Purpose: The purpose of study is to estimate the number of chronic disease patients and medical care expenditure at the time baby-boomers belong to 65 years old aging population, and compare with current 65 year-old aging population. Methods: Analysis method used an estimating formula devised by the researcher and estimated the number of chronic disease patients and medical care expenditure of each generation. Results: When comparing the estimated number of chronic diseases patients of each generation, 40.6% of the first generation, 76.4% of the second generation, 95.2% of third generation are expected to get chronic disease. When comparing each generation's total medical care expenditure, based on the estimated number of chronic diseases patients of each generation, the second generation( 1,206,251,224 thousand won) showed higher than other generation. This study compared the number of chronic disease patients and medical care expenditure between the second generation of the elderly and current elder generation. As a result, the second generation patients was higher than the fourth generation in high blood pressure, diabetes, psychological and behavioral disorder, and neurological diseases whereas the fourth generation is only high the number of patients in heart disease. As for total medical care expenditure, the second generation paid more in high blood pressure, psychological and behavioral disorder while the fourth generation in neurological disease and heart disease. Conclusion: It is desired that considering the number of chronic disease patients and medical care expenditure of baby boomers accounting for 14.6% of total population, in-depth follow-up study is carried out that inquires into what are issues with a current chronic disease management project, what business is needed in order to manage these issues, and how to fund to cover increasing medical care expenditure.

원발성, 다발성 뇌종양 - 증례보고 - (Multiple Primary Brain Tumors - Case Report -)

  • 여인욱;서재희;권양
    • Journal of Korean Neurosurgical Society
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    • 제30권7호
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    • pp.925-927
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    • 2001
  • Multiple intracranial tumors of different cell types are rare. We report a case of double primary intracranial tumors of different histologic types : oligodendroglioma, and pituitary adenoma. Von Recklinghausen's disease or other etiologic factors supposedly associated with multiple brain tumors were not recognized in this 36-year-old male patient.

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