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신현욱(Hyun Uk Shin),김종국(Jong Kuk Kim),윤별아(Byeol A Yoon),류원열(Won Yeol Ryu) 대한안과학회 2016 대한안과학회지 Vol.57 No.12
Purpose: In this study, a case of toxic encephalopathy and optic neuropathy due to methyl bromide poisoning is reported. Case summary: A 31-year-old male presented with dysarthria, gait disturbance and bilateral visual impairment. He was treated with intravenous methylprednisolone for bilateral optic neuritis 1 year prior. He previously worked in a fumigation warehouse and was exposed to methyl bromide in the past 3 years. His corrected visual acuity was 20/30 in both eyes. The patient had reduced color vision and enlarged central scotoma in both eyes. His mentality was alert but exhibited slow response, ataxia and dysarthria. Brain magnetic resonance imaging (MRI) revealed high signals in the brainstem, cerebellum and midbrain. His serum and urine methyl bromide concentrations were significantly elevated. The patient was treated with intravenous methylprednisolone 1.0 g/day for 5 days. MRI showed resolution of the multiple brain lesions observed previously. Ten days after steroid therapy, his visual acuity was 20/20 in both eyes and his neurologic manifestations were completely recovered at 2 months after treatment. Conclusions: Taking a detailed occupational history is necessary in patients with optic neuropathy. The probability of toxic optic neuropathy should be considered when patients are exposed to toxic materials. 목적: 메틸브로마이드의 중독으로 유발된 독성 뇌병증을 동반한 시신경병증 1예를 진단하고 치료하였기에 이를 보고하고자 한다. 증례요약: 31세 남자가 구음 및 보행 장애를 동반한 양안 시력 저하를 주소로 내원하였다. 1년 전 양안 시신경염 의심하에 스테로이드치료를 받은 과거력이 있었다. 직업력상 최근 7년간 메틸브로마이드를 사용하는 소독 작업 창고에서 운반 일을 하였다. 내원 시 교정시력은 양안 20/30, 색각 이상과 중심 암점이 관찰되었다. 의식 상태는 명료하나 반응이 느렸고, 구음 장애 및 운동 실조를 보였다. 뇌 자기공명영상에서 교뇌와 소뇌에 양측성 신호변화가 관찰되었다. 정맥 스테로이드 치료를 5일간 시행하였고, 10일째 추적 검사한 뇌 자기공명영상에서 초기에 보이던 신호변화는 거의 소실되었다. 치료 시작 후 10일째 시력은 양안 20/20으로 회복되었고, 신경학적 이상 소견은 2개월 후 완전 회복되었다. 결론: 시신경병증 환자에서도 직업력에 대한 자세한 조사가 필요하며, 독성 물질에 대한 지속적인 노출력이 있을 경우 중독에 의한 시신경병증 가능성에 대하여 고려해야 할 것으로 생각된다.
Acute bulbar palsy as a variant of Guillain-Barré syndrome
Kim, Jong Kuk,Kim, Byung-Jo,Shin, Ha Young,Shin, Kyong Jin,Nam, Tai-Seung,Oh, Jeeyoung,Suh, Bum Chun,Yoon, Byeol-A,Park, Hwan Tae,Huh, So-Young,Oh, Seong-Il,Bae, Jong Seok Ovid Technologies (Wolters Kluwer) - American Acad 2016 Neurology Vol.86 No.8
<P>Objective: To categorize a syndrome manifesting as prominent acute bulbar palsy (ABP) without limb motor weakness as a variant form of Guillain-Barre syndrome (GBS) and differentiate it from Miller Fisher syndrome (MFS) and pharyngeal-cervical-brachial (PCB) variants. Methods: We analyzed cases of ABP without limb motor weakness based on a dataset containing clinical information and the results of antiganglioside antibodies assays for acute immune-mediated neuropathies. Results: Eleven cases with an age at onset ranging from 18 to 65 years (mean 33.8 years) were identified as ABP-plus syndrome. All of the enrolled cases manifested with ABP as the predominant symptom, and with no limb weakness. The following features accompanied ABP in order of decreasing frequency: ophthalmoplegia (n = 9, 82%), ataxia (n = 9, 82%), and facial palsy (n = 6, 55%). An enzyme-linked immunosorbent assay study disclosed that immunoglobulin G (IgG) anti-GT1a antibodies were the most frequent (n = 11), followed by IgG anti-GQ1b antibodies (n = 6). Conclusions: We propose that ABP-plus syndrome without neck or limb weakness is a variant of GBS that is distinct from the MFS and PCB variants. The presence of IgG anti-GT1a antibodies can explain the relationships between the distinct clinical characteristics and the underlying pathomechanisms.</P>
Misra, Jagannath,Kim, Don-Kyu,Jung, Yoon Seok,Kim, Han Byeol,Kim, Yong-Hoon,Yoo, Eun-Kyung,Kim, Byung Gyu,Kim, Sunghoon,Lee, In-Kyu,Harris, Robert A.,Kim, Jeong-Sun,Lee, Chul-Ho,Cho, Jin Won,Choi, Hue American Diabetes Association 2016 Diabetes Vol.65 No.10
<P>Estrogen-related receptor gamma (ERR gamma) is a major positive regulator of hepatic gluconeogenesis. Its transcriptional activity is suppressed by phosphorylation signaled by insulin in the fed state, but whether posttranslational modification alters its gluconeogenic activity in the fasted state is not known. Metabolically active hepatocytes direct a small amount of glucose into the hexosamine biosynthetic pathway, leading to protein O-GlcNAcylation. In this study, we demonstrate that ERR gamma is O-GlcNAcylated by O-GlcNAc transferase in the fasted state. This stabilizes the protein by inhibiting proteasome-mediated protein degradation, increasing ERR gamma recruitment to gluconeogenic gene promoters. Mass spectrometry identifies two serine residues (S317, S319) present in the ERR gamma ligand-binding domain that are O-GlcNAcylated. Mutation of these residues destabilizes ERR gamma protein and blocks the ability of ERR gamma to induce gluconeogenesis in vivo. The impact of this pathway on gluconeogenesis in vivo was confirmed by the observation that decreasing the amount of O-GlcNAcylated ERR gamma by overexpressing the deglycosylating enzyme O-GlcNAcase decreases ERR gamma-dependent glucose production in fasted mice. We conclude that O-GlcNAcylation of ERR gamma serves as a major signal to promote hepatic gluconeogenesis.</P>
Miller Fisher syndrome mimicking Wernicke encephalopathy during pregnancy
Seo, Jung Hwa,Kang, Mi-Ri,Yoon, Byeol-A,Ji, Ki-Hwan,Oh, Seong-il The Korean Society of Clinical Neurophysiology 2019 Annals of Clinical Neurophysiology Vol.21 No.1
Miller Fisher syndrome (MFS) is characterized by ataxia, areflexia, and ophthalmoparesis. Here we present a case of MFS mimicking Wernicke encephalopathy (WE) during pregnancy. A 31-year-old woman at 8 weeks of gestation presented with diplopia and ataxia after experiencing nausea and vomiting for several weeks. We initiated thiamine based on a suspicion of WE, which produced no clear effects. However, her symptoms began to improve following intravenous immunoglobulin treatment, and other findings finally lead to a diagnosis of MFS. Because ataxia and ophthalmoparesis can be misdiagnosed as WE during pregnancy, clinicians should consider MFS in the differential diagnosis.
Grb2-associated binder-1 is required for extrafusal and intrafusal muscle fiber development
Park, So Y.,Jang, So Y.,Shin, Yoon K.,Yoon, Byeol A.,Lee, Hye J.,Park, Hwan T. Lippincott Williams & Wilkins 2017 NeuroReport Vol.28 No.10
<P>The neuregulin-1 (NRG1) signaling pathway plays an important role in the development of the peripheral neuromuscular system, including in muscle spindle and postnatal myelination. We previously showed that NRG1 on the axonal membrane regulates peripheral nerve myelination through Grb2-associated binder 1 (Gab1),a scaffolding mediator of receptor tyrosine kinase signaling. Here, we determined the role of Gab1 in the development of muscles and the muscle spindle using muscle-specific conditional Gab1 knockout mice. The mutant mice showed general retardation in muscular growth and hypotrophy of extrafusal muscle fibers. In addition, the muscle-specific Gab1 knockout mutant exhibited significant underdevelopment of muscle spindles, which are normally regulated by NRG1, and abnormal proprioceptive behavior. Furthermore, the selective knockdown of Gab1 in C2C12 muscle cells reduced NRG1-induced expression of Egr3, a critical transcription factor for muscle spindle development. However, Gab2 knockout mice did not show any defects in the development of muscles or muscle spindles. Our findings suggest that Gab1 is an essential signaling molecule in mediating axonal NRG1 signaling for the development of both extrafusal and intrafusal muscle fibers. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.</P>
Clinical Heterogeneity of Anti-GM2-Ganglioside-Antibody Syndrome
Kim, Jong Kuk,Kim, Yoo Hwan,Yoon, Byeol-A,Cho, Joong-Yang,Oh, Sun-Young,Shin, Ha Young,Kim, Ji Soo,Park, Kee Hong,Kim, Sun Young,Suh, Bum Chun,Seok, Hung Youl,Yoo, Jin Hyuk,Bae, Jong Seok Korean Neurological Association 2018 Journal of Clinical Neurology Vol.14 No.3
<P><B>Background and Purpose</B></P><P>Antiganglioside antibodies are known to play a pathogenic role in Guillain-Barré syndrome (GBS). Either an immunoglobulin (Ig)G- or IgM-type anti-GM2 antibody is detected in rare cases in GBS patients. However, the specific pathogenic role of these antibodies in GBS has not been reported previously. This study aimed to define and characterize the clinical spectrum of GBS with anti-GM2 positivity.</P><P><B>Methods</B></P><P>We reviewed the database of the Dong-A University Neuroimmunology Team, which has collected sera of GBS and its variants from more than 40 general and university-based hospitals in Korea. Detailed information about the involved patients was often obtained and then corrected by the charge doctor applying additional questionnaires.</P><P><B>Results</B></P><P>Four patients with acute monophasic peripheral neuropathy or cranial neuropathy with isolated IgM-type anti-GM2-antibody positivity were recruited. In addition, IgG-type anti-GM2 antibody was solely detected in the sera of another four patients. The IgM-positive group comprised heterogeneous syndromes: two cases of acute motor axonal neuropathy, one of acute inflammatory demyelinating polyneuropathy, and one of isolated facial diplegia. In contrast, all of the cases enrolled in the IgG-positive group manifested with dizziness with or without oculomotor palsy due to cranial neuropathy syndrome.</P><P><B>Conclusions</B></P><P>This study has identified that anti-GM2 antibody can be found in various subtypes of GBS and its variants in rare cases. Compared to the clinical heterogeneity of the IgM-positive group, the IgG-positive group can be characterized by cranial-dominant GBS variants presenting mainly with oculomotor and vestibular dysfunctions.</P>