사르코이드증에 동반된 다발신경병증

안석원,김동욱,홍윤호,이광우 대한임상신경생리학회 2003 Annals of Clinical Neurophysiology Vol.5 No.1

Sarcoidosis is multisystemic granulomatous disease of unknown etiology ogenesis, and most frequently presented with bilateral hilar lymphadenopathy, pulmonary infiltration, skin and eye lesion. However, neurological involvement including peripheral neruopathy is relatively rare. We report a patient who had sensorimotor polyneuropathy without other systemic or organ involvements frequently reported in sarcoidosis. Laboratory investigation suggestive of sarcoidosis lead to sural nerve biopsy for confirmation, which demonstrated noncaseating granulomatous changes. Sarcoidosis should be included in the differential diagnosis in subacute polyneuropathy even if there is no usual symptoms or signs suggestive of the systemic disease.

Adult Sandhoff Disease Presenting as Motor Neuron Disease Phenotype

안석원,김수현,김수연,이광우,김성민,성정준 대한임상신경생리학회 2009 Annals of Clinical Neurophysiology Vol.11 No.2

We report a 23-year-old woman with adult Sandhoff disease, who presented with motor neuron disease phenotype. The patient had experienced progressive motor weakness in four extremities since 1 year prior to admission. Electrophysiological study revealed wide-spread denervation potentials, and the assay of total hexosaminidase involving A and B activities showed decreased levels of these activities, which was consistent with Sandoff disease. This is the first Korean case of adult Sanhoff disease presented as a motor neuron disease phenotype.

근위축성측삭경화증의 SOD1-G93A 유전자 이식 마우스모델에서 라파마이신의 신경보호효과

안석원,전계선,박광열,홍윤호,이광우,성정준 대한임상신경생리학회 2013 Annals of Clinical Neurophysiology Vol.15 No.2

Background: The autophagy is the major route for lysosomal degradation of misfolded protein aggregates and oxidative cell components. We hypothesized that rapamycin (autophagy enhancer) would prolong the survival of motor neuron and suppress the disease progression in amyotrophic lateral sclerosis (ALS). Methods: A total of 24 transgenic mice harboring the human G93A mutated SOD1 gene were used. The clinical status involving rotarod test and survival, and biochemical study of ALS mice model were evaluated. Results: The onset of symptoms was significantly delayed in the rapamycin administration group compared with the control group. However, after the clinical symptom developed,the rapamycin exacerbated the disease progression and shortened the survival of ALS mice model, and apoptosis signals were up-regulated compared with control group. Conclusions: Even though further detailed studies on the relevancy between autophagy and ALS will be needed, our results revealed that the rapamycin administration was not effective for being novel promising therapeutic strategy in ALS transgenic mice and exacerbated the apoptosis. Background: The autophagy is the major route for lysosomal degradation of misfolded protein aggregates and oxidative cell components. We hypothesized that rapamycin (autophagy enhancer) would prolong the survival of motor neuron and suppress the disease progression in amyotrophic lateral sclerosis (ALS). Methods: A total of 24 transgenic mice harboring the human G93A mutated SOD1 gene were used. The clinical status involving rotarod test and survival, and biochemical study of ALS mice model were evaluated. Results: The onset of symptoms was significantly delayed in the rapamycin administration group compared with the control group. However, after the clinical symptom developed, the rapamycin exacerbated the disease progression and shortened the survival of ALS mice model, and apoptosis signals were up-regulated compared with control group. Conclusions: Even though further detailed studies on the relevancy between autophagy and ALS will be needed, our results revealed that the rapamycin administration was not effective for being novel promising therapeutic strategy in ALS transgenic mice and exacerbated the apoptosis.

Motor Unit Number Estimation in Evaluating Disease Progression in Patients with Amyotrophic Lateral Sclerosis

안석원,김수현,오동훈,김성민,박경석,홍윤호,권오상,성정준,이광우 대한의학회 2010 Journal of Korean medical science Vol.25 No.9

We investigated the availability of motor unit number estimation (MUNE) as a quantitative method to assess the severity and clinical progression of amyotrophic lateral sclerosis (ALS). The 143 ALS patients were evaluated by statistical MUNE and the revised amyotrophic lateral sclerosis functional rating scale (ALSFRS-R). By using mean values of MUNE according to disease duration, regression equation between mean MUNE and disease duration was presented as a formula. The individual MUNE ratio was calculated by dividing individual MUNE value by mean MUNE value. All patients were classified into 2 groups (MUNE ratio <1 vs. MUNE ratio ≥1) according to the MUNE ratio. Comparison between the 2 groups revealed that the patients in MUNE ratio <1 group or MUNE ratio ≥1 group were respectively assigned to rapid progression or slow progression. We recommended informative mean values of MUNE and best regression equation in ALS patients according to disease duration. These values allow us to evaluate the severity and rapidity of progression in ALS.

Isolated Spinal Cord Neurosarcoidosis Diagnosed by Cord Biopsy and Thalidomide Trial

안석원,김경태,윤영철,권오상,김영백 대한의학회 2011 Journal of Korean medical science Vol.26 No.1

We report a case of 54-yr-old woman who presented with 4-extremities weakness and sensory changes, followed by cervical spinal cord lesion in magnetic resonance imaging. Based on the suspicion of spinal tumor, spinal cord biopsy was performed, and the histology revealed multinucleated giant cells, lymphocytes and aggregated histiocytes within granulomatous inflammation, consistent with non-caseating granuloma seen in sarcoidosis. The patient was treated with corticosteroid, immunosuppressant and thalidomide for years. Our case indicates that diagnosis of spinal cord sarcoidosis is challenging and may require histological examination, and high-dose corticosteroid and immunosuppressant will be a good choice in the treatment of spinal cord sarcoidosis, and the thalidomide has to be debated in the spinal cord sarcoidosis.

막사구체신염과 포도막염이 동반된 길랑-바레증후군 1예

안석원,오동훈,전범석 대한신경과학회 2005 대한신경과학회지 Vol.23 No.5

Relapsing Polychondritis Presented with Encephalitis Followed by Brain Atrophy

안석원,박무석,정해봉,권오상,윤병남,김희성,최상태 한국뇌신경과학회 2017 Experimental Neurobiology Vol.26 No.1

Relapsing polychondritis (RP) is a rare autoimmune disease that is characterized by inflammatory reaction of unknown etiology and destruction of cartilaginous structures. Characteristic symptoms of this disease include cartilage inflammation of the ear, nose, larynx, trachea, bronchi, joints, eyes, heart and skin. Concomitance with neurologic symptom is very rare in RP, and the detailed underlying mechanism of neurological involvement associated with RP is not fully understood. We herein described an unusual recurrent case of inflammatory brain lesions associated with RP, with attention to clinical manifestations, autoimmune disease involvement, and therapeutic effects.

Concurrence of Multifocal Motor Neuropathy and Hashimoto’s Thyroiditis

안석원,김수현,박병수,차정인,김성민,성정준,이광우 대한신경과학회 2011 Journal of Clinical Neurology Vol.7 No.3

Background Multifocal motor neuropathy (MMN) is an immune-mediated disorder that is characterized by slowly progressive and asymmetrical weakness, but its pathophysiological mechanism is uncertain. The hypothesis that MMN is an immunological disease has been supported by the proven therapeutic effects of intravenous immunoglobulin and the detection of antiganglioside antibodies in MMN patients. The coexistence of MMN with other immune diseases has been rarely reported. Case Report A 37-year-old woman visited our hospital complaining of weakness in both hands. The clinical manifestations coincided well with MMN: predominantly distal upper-limb weakness, asymmetric involvement, a progressive course, absence of sensory symptoms, absence of pyramidal signs, and sparing of the cranial muscles. The electrophysiological findings also supported a diagnosis of MMN, with motor nerve conduction block in the median, ulnar,and radial nerves, without sensory nerve involvement. The patient was simultaneously diagnosed as having Hashimoto’s thyroiditis, which is a well-known immune-mediated disease. Conclusions The concurrence of MMN and Hashimoto’s thyroiditis in our patient is significant for understanding the immunological characteristics of the two diseases.

Nerve conduction studies: basic principal and clinical usefulness

안석원,Byung-Nam Yoon,Jee-Eun Kim,석진명,김광국,임영민,Ki-Han Kwon,박기덕,Bum-ChunSuh 대한임상신경생리학회 2018 Annals of Clinical Neurophysiology Vol.20 No.2

Nerve conduction study (NCS) is an electrophysiological tool to assess the overall function of cranial and peripheral nervous system, therefore NCS has been diagnostically helpful in the identification and characterization of disorders involving nerve roots, peripheral nerves, muscle and neuromuscular junction, and are frequently accompanied by a needle Electromyography. Furthermore, NCS could provide valuable quantitative and qualitative results into neuromuscular function. Usually, motor, sensory, or mixed nerve studies can be performed with using NCS, stimulating the nerves with the recording electrodes placed over a distal muscle, a cutaneous sensory nerve, or the entire mixed nerve, respectively. And these findings of motor, sensory, and mixed nerve studies often show different and distinct patterns of specific abnormalities indicating the neuromuscular disorders. The purpose of this special article is to review the neurophysiologic usefulness of NCS, to outline the technical factors associated with the performance of NCS, and to demonstrate characteristic NCS changes in the setting of various neuromuscular conditions.

LCA 를 이용한 친환경 세제의 개발

안석원,이재란 한국전과정평가학회 1999 한국전과정평가학회지 Vol.1 No.1

친환경 세제 개발을 위하여 전과정 평가 기법을 분말형 세탁세제에 적용하였다. 전과정 평가 수행 절차는 목표 및 범위 설정, 목록 분석, 특성화, 정규화, 가중치 적용으로 행해졌으며 CML 방법을 적용하였다. 이에 따라 분말형 세탁세제에 대한 전과정 목록이 도출되었으며 이를 산성화, 오존층 파괴, 부영양화, 지구온난화, 수계독성의 영향범주로 나누어서 영향평가를 실시하였다. 특히, 수계독성에 대한 특성화 지수는 세제 원료들에 대한 수계독성자료를 바탕으로 도출하였다. 영향평가의 결과로써, 영향범주별로는 수계 독성의 환경 영향이 크게 나타났고, 공정별로는 원재료 생산 단계가 환경 영향이 큰 것으로 나타났다. 또한, 세제 각 원료 성분들에 대한 환경 지수 산출을 통하여 환경 점수를 부여하였다. 본 연구의 결과로 연구 개발 단계에서부터 세탁세제의 환경성, 경제성, 세정 성능적 측면을 동시에 고려하여 환경영향을 최소화할 수 있는 제품의 설계 방법을 개발하였으며, 이를 적용하여 수계 독성을 많이 개선한 친환경세제를 개발하였다. For development of environmentally friendly detergent, we have applied LCA method to a laundry detergent. The procedure of LCA was followed to goal & scope definition, classification, characterization, normalization, weighting by using CML method. Therefore, we have achieved the life cycle inventory of laundry detergent, and have done with impact assessment about acidification, ozone layer depletion, eutrophicaiton, greenhouse effect and ecotoxicity. Especially, the ecotoxicological classification factor for aquatic ecosystem was derived from basis of detergent ingredient toxicity data. As a result of impact assessment, we have found that the most environmentally loaded impact category was ecotoxicity and the most environmentally loaded process was raw material production stage. In addition, we derived environmental indicator from each detergent ingredients. Finally, we have developed the method of design for environment that is minimized the environmental impact considering environmental, economical, and washing performance properties in coincidence. By using this, we have developed the environmentally friendly detergent that has improved on the ecotoxicity.