H-반사 촉진의 소실: 길랑-바레 증후군의 초기 이상과 중추신경 침범의 증거로써의 의미

성정준,이윤정,홍윤호,정영민,이광우 대한임상신경생리학회 2002 Annals of Clinical Neurophysiology Vol.4 No.2

Background : Guillain-Barre syndrome(GBS) is a neurologically emergent condition, leading to respiratory insufficiency without an early and appropriate treatment. Thus, the treatment of GBS requires early diagnosis but it is difficult due to the low sensitivity of laboratory tools in the initial stage. Hoffman reflex (H-reflex) and its facilitation by Jendrassik maneuver (JM) are sensitive tools evaluating the central circuit of motor system on the spinal cord level. The aim of this study is to test whether the change of H-reflex and F-wave under the JM is able to detect the early stage of GBS and whether GBS involves the central nervous system (CNS). Material and Methods : All 7 GBS patients who showed normal or nearly normal nerve conduction study were included. The facilitation of H-reflex and changes of F-wave were calculated by measuring the percent difference f H-reflex or F-wave amplitude under JM compared to basal H-reflex of F-wave amplitude. The changes of F-wave and H-reflex in the GBS patients were compared with them of 8 healthy controls. Results : The F-wave amplitudes of both healthy controls and GBS patients did not changed under the influence of JM (102.4±24.9 %, 108.7±29.0% respectively). However, the facilitation of H-wave by JM in the GBS patients was absent (98.8±5.8%), even though the H-reflex amplitude I the healthy controls increased under the influence of JM (124.8±12.1%). Conclusions : The loss of H-reflex facilitation in GBS implies that this phenomenon might be the most early change detected in the electrophysiological study and support the early diagnosis, and that GBS might include lesion in the spinal motor CNS.

유기인산염 아세틸콜린에스터레이스 억제제(Diisopropylfluorophosphate)가 니코틴성 아세틸콜린 수용체의 기능에 미치는 효과

성정준,박경석,이광우 대한신경과학회 2000 대한신경과학회지 Vol.18 No.6

돌연변이 Cu,Zn-superoxide dismutase 유전자를 발현하는 운동신경세포에 대한 Homocysteine의 선택적 세포독성 기전

성정준,김현정,김성민,민주홍,홍윤호,박경석,김만호,이석호,호원경,이광우 대한신경과학회 2006 대한신경과학회지 Vol.24 No.1

Background: Mutations in Cu, Zn-superoxide dismutase (SOD1) cause about 20% of familial amyotrophic lateral sclerosis (FALS) cases. The mechanism of late-onset disease manifestation despite the innate mutation has no clear explanation. The relationship between homocysteine (HC) and amyotrophic lateral sclerosis (ALS) has not been investigated fully, in spite of the similarity in their pathogenesis. Methods: We investigated the effect of HC on the motor neuronal cell-line (VSC4.1) transfected with SOD1 of either wild-type or mutant forms (G93A and A4V) using various methods including the MTT assay for the cytotoxic assay, the immunocytochemical staining using anti-SOD1 for the aggregation of SOD1, the western blotting using anti-nitrotyrosine and anti-DNPH for the oxidative protein damage, and the measurement of the intracellular Ca2+ concentration using Fura2-AM. Results: In the MTT assay, the HC induced significant cytotoxicity in the mutants, as compared with wild-type. This HC-induced cytotoxicity was inhibited by the trolox and the bathocuproinedisulfonate (BC). HC increased the carbonylation and nitrosylation of the mutant proteins. HC also increased significant SOD1-aggregation in mutants. This HC-induced SOD1-aggregation in mutants was inhibited by trolox, N-nitro-L-arginine methyl ester, BC, and z-VAD-FMK. HC did not change the intracellular concentration of Ca2+ in the mutants compared with the wild-type. Conclusions: The authors showed that the vulnerability of the SOD1 mutant motor neuronal cells to HC involves the copper-mediated oxygen radical toxicity, and that HC may be a lifelong precipitating factor in some forms of FALS, suggesting a possible treatment modality with vitamin supplements.

빙원위의 건강한 한국인들

성정준 대한의학협회 1995 대한의학협회지 Vol.38 No.4

MuSK 항체 양성을 보이는 혈청음성 중증근무력증 환자의 임상적 특징

이지영,성정준,오동훈,민주홍,김현정,박종하,최영철,김성훈,A. Vincent,이광우 대한신경과학회 2005 대한신경과학회지 Vol.23 No.6

Background: A variable proportion of seronegative myasthenia gravis (SNMG) patients have antibodies to the muscle-specific tyrosine kinase (MuSK). Although several reports from Western countries suggest differences in the clinical features of MuSK antibody-positive and -negative SNMG patients, there have been no reports about these patients in Korea. Methods: We performed the first survey of MuSK antibodies in Korea, measuring MuSK antibodies by commercial preparations (RSR Ltd) in the serum of SNMG patients who registered at the Seoul National University Hospital from October 2003 to January 2004, and identified clinical features and treatment responses prospectively until October 2004 using double blind method. Results: Twenty-three (15 generalized and eight ocular MG, 15 men and eight women) SNMG patients with the ages from 1 to 60 years, (mean 36.24±16.82 years), were included. None of 8 ocular SNMG had MuSK antibody, whereas MuSK antibody was present in four (26.7%) of 15 generalized SNMG. All four MuSK positive patients were females, with pharyngeal and respiratory muscle weakness, and required immunosuppressive treatment in addition to acetylcholine esterase inhibitors. However, the overall disease severity and the age of onset did not show significant differences between MuSK antibody-positive and -negative SNMG patients and the responses to treatment were equally favorable. Conclusions: Our study showed the lower rate of MuSK antibodies in SNMG than the previous reports. However it seems to require large multicenter survey to confirm the possibilities of geographical or ethnical differences in the future.

The Anti-Inflammatory Effects of Oral-Formulated Tacrolimus in Mice with Experimental Autoimmune Encephalomyelitis

김명진,성정준,김승현,김정민,전계선,문석균,안석원 대한의학회 2017 Journal of Korean medical science Vol.32 No.9

Multiple sclerosis (MS) is a T-lymphocyte-mediated autoimmune disease that is characterized by inflammation in the central nervous system (CNS). Although many disease-modifying therapies (DMTs) are presumed effective in patients with MS, studies on the efficacy and safety of DMTs for preventing MS relapse are limited. Therefore, we tested the immunosuppressive anti-inflammatory effects of oral-formulated tacrolimus (FK506) on MS in a mouse model of experimental autoimmune encephalomyelitis (EAE). The mice were randomly divided into 3 experimental groups: an untreated EAE group, a low-dose tacrolimus-treated EAE group, and a high-dose tacrolimus-treated EAE group. After autoimmunization of the EAE mice with myelin oligodendrocyte glycoprotein, symptom severity scores, immunohistochemistry of the myelination of the spinal cord, and western blotting were used to evaluate the EAE mice. After the autoimmunization, the symptom scores of each EAE group significantly differed at times. The group treated with the larger tacrolimus dose had the lowest symptom scores. The tacrolimus-treated EAE groups exhibited less demyelination and inflammation and weak immunoreactivity for all of the immunization biomarkers. Our results revealed that oral-formulated tacrolimus inhibited the autoimmunization in MS pathogenesis by inactivating inflammatory cells.

두개내 동맥 병변을 보인 혈관형 베체트씨병 2예

박은미,성정준,이정주,강동화,노재규 대한신경과학회 1999 대한신경과학회지 Vol.17 No.1

전문의 고시에서 표준화 환자를 이용한 진료 수행 시험의 경험: 3개 연도 신경과 전문의 시험 결과 분석을 바탕으로

김범준,성정준,박훈기,서대원,정진상,윤병우 한국의학교육학회 2011 Korean journal of medical education Vol.23 No.2

Purpose: Evaluation of clinical skills and attitude including development of dynamic patient-doctor relationship is important in board examination (BE). Korean Neurological Association (KNA) has introduced clinical performance examination (CPX) utilizing standardized patients (SP) to BE in 2007. In this study, the authors describe the 3-year experience of CPX in BE through 2009. Methods: To implement CPX session in BE, KNA developed CPX workshop for BE attendees and members of grading committee. CPX sessions in BE consisted of two model scenarios mimicking neurological patients in clinical practice. The total score and itemized scores of CPX sessions were compared with other areas of BE, and scores from each year were also compared. Results: Scores from CPX sessions were significantly correlated with BE step II. Among the itemized scores of CPX sessions, clinical items including history taking and physical examination were significantly correlated with scores from other areas of BE. However,scores from global assessment from SP were strongly associated with patient-doctor relationship, history taking, and patient education. Conclusion: Our experiences suggest that CPX utilizing SP is a useful tool to assess the clinical skills in BE. In order to produce clinically well qualified neurologists, more efforts should be made to develop cases and to improve assessment tools for CPX.

Hereditary Spastic Paraplegia with a Novel SPAST Mutation Misdiagnosed with Subacute Combined Degeneration

양지원,한지영,성문우,성정준,박성섭,이광우 한국뇌신경과학회 2013 Experimental Neurobiology Vol.22 No.2

Autosomal dominant hereditary spastic paraplegia (AD-HSP) is due to mutations in the “spastin” gene (SPAST gene) encoding the AAA protein. The main clinical features of “pure” HSP are progressive lower-limb spasticity with corticospinal tracts and dorsal column degeneration without peripheral neuropathy. Here we report the case of HSP with novel SPAST gene mutation that misdiagnosed with subacute combined degeneration initially. A 58-year-old man with gait disturbance came to our hospital. He was unable to regulate his steps by himself. The impaired gait began 3 years after he had undergone subtotal gastrectomy and chemotherapy for 6 months. Thereafter, he started feeling tingling sensations in the hands and feet and acquired gait difficulties. He denied having a family history of abnormal gait or developmental problem. We diagnosed him with subacute combined degeneration on the evidence of history of gastrectomy, lower normal limit of vitamin B12 (363 pg/ml), apparent absence of vibration sensations and paresthesia in the feet. He was intramuscularly administered cyanocobalamin regularly. However, there was no improvement in his condition. We reconsidered his symptoms and signs, decided to examine the SPAST gene, which is the most common mutation in HSP. The SPAST gene, c.870+1delG, heterozygote, splicing mutation is detected from the gene sample. There was no previous information of this polymorphism or mutation at this locus. We examined his two children, and the same mutation was founded in his son. We report a patient of novel SPAST gene mutation with AD-HSP which is misdiagnosed with SCD.

Oral Solubilized Ursodeoxycholic Acid Therapy in Amyotrophic Lateral Sclerosis: A Randomized Cross-Over Trial

민주홍,홍윤호,성정준,김성민,이정복,이광우 대한의학회 2012 Journal of Korean medical science Vol.27 No.2

To evaluate the efficacy and safety of ursodeoxycholic acid (UDCA) with oral solubilized formula in amyotrophic lateral sclerosis (ALS) patients, patients with probable or definite ALS were randomized to receive oral solubilized UDCA (3.5 g/140 mL/day) or placebo for 3 months after a run-in period of 1 month and switched to receive the other treatment for 3 months after a wash-out period of 1 month. The primary outcome was the rate of progression, assessed by the Appel ALS rating scale (AALSRS), and the secondary outcomes were the revised ALS functional rating scale (ALSFRS-R) and forced vital capacity (FVC). Fifty-three patients completed either the first or second period of study with only 16 of 63 enrolled patients given both treatments sequentially. The slope of AALSRS was 1.17 points/month lower while the patients were treated with UDCA than with placebo (95% CI for difference 0.08-2.26, P = 0.037), whereas the slopes of ALSFRS-R and FVC did not show significant differences between treatments. Gastrointestinal adverse events were more common with UDCA (P < 0.05). Oral solubilized UDCA seems to be tolerable in ALS patients, but we could not make firm conclusion regarding its efficacy, particularly due to the high attrition rate in this cross-over trial.