Pub Date : 2023-06-30DOI: 10.59578/jmsni.2023.14.1.9
Je-Young Shin, Suk-Won Ahn
Multiple sclerosis (MS) is an inflammatory disease of the central nervous system (CNS) with a chronic and often progressive disease course. The current disease-modifying treatments (DMTs) limit disease progression primarily by controlling immune cell activity in the peripheral blood or inhibiting their migration from the periphery into the CNS. However, approved therapies are less effective at slowing disability accumulation in patients with MS, and new therapies are needed to target CNS immunopathology, which is a key driver of disability progression in MS. Bruton’s tyrosine kinase (BTK) is an intracellular signalling molecule involved in the regulation of maturation, survival, migration and activation of B cells and microglia, therefore BTK inhibitors target both adaptive and innate mechanisms that contribute to the immunopathology of MS on both sides of the blood-brain barrier. This article reviews the preclinical researches and therapeutic roles of Bruton's tyrosine kinase inhibitors as promising DMTs to target cells of the adaptive and innate immune system outside and within the CNS in the MS.
{"title":"Bruton’s Tyrosine Kinase Inhibitors for Multiple Sclerosis","authors":"Je-Young Shin, Suk-Won Ahn","doi":"10.59578/jmsni.2023.14.1.9","DOIUrl":"https://doi.org/10.59578/jmsni.2023.14.1.9","url":null,"abstract":"Multiple sclerosis (MS) is an inflammatory disease of the central nervous system (CNS) with a chronic and often progressive disease course. The current disease-modifying treatments (DMTs) limit disease progression primarily by controlling immune cell activity in the peripheral blood or inhibiting their migration from the periphery into the CNS. However, approved therapies are less effective at slowing disability accumulation in patients with MS, and new therapies are needed to target CNS immunopathology, which is a key driver of disability progression in MS. Bruton’s tyrosine kinase (BTK) is an intracellular signalling molecule involved in the regulation of maturation, survival, migration and activation of B cells and microglia, therefore BTK inhibitors target both adaptive and innate mechanisms that contribute to the immunopathology of MS on both sides of the blood-brain barrier. This article reviews the preclinical researches and therapeutic roles of Bruton's tyrosine kinase inhibitors as promising DMTs to target cells of the adaptive and innate immune system outside and within the CNS in the MS.","PeriodicalId":324885,"journal":{"name":"Journal of Multiple Sclerosis and Neuroimmunology","volume":"129 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"124236160","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-06-30DOI: 10.59578/jmsni.2023.14.1.67
Eun Sug Park, Hyun Young Kim, Young Seo Kim, H. S. Kwon
Copyright c 2023 by Korean Society of Neuroimmunology 67 Systemic lupus erythematosus (SLE) is a complex systemic autoimmune disorder characterized by a wide range of immunological abnormalities. One of its notable features is the potential to affect the central nervous system (CNS), leading to diverse neurological manifestations including headaches, acute state of confusion, seizure disorder, cerebrovascular disease, and meningoencephalitis. CNS involvement is highly prevalent in SLE patients, ranging from 12% to 75%, and is associated with a poor prognosis. Treatment options such as steroids, azathioprine, rituximab, and intravenous immunoglobulins (IVIGs) have been considered in managing these cases. However, early diagnosis of SLE-related CNS involvement remains challenging due to the rapid progression of symptoms and the need to exclude various alternative diagnoses. In this article, we present a fatal case of an SLE patient with meningoencephalitis, initially resembling an ischemic stroke. Despite administering antibiotics, antiviral agents, IVIGs, steroids, rituximab, and plasma exchange, no clinical improvement was observed.
{"title":"Fatal Meningoencephalitis Initially Misdiagnosed as Cerebral Infarction in a Patient with Systemic Lupus Erythematosus","authors":"Eun Sug Park, Hyun Young Kim, Young Seo Kim, H. S. Kwon","doi":"10.59578/jmsni.2023.14.1.67","DOIUrl":"https://doi.org/10.59578/jmsni.2023.14.1.67","url":null,"abstract":"Copyright c 2023 by Korean Society of Neuroimmunology 67 Systemic lupus erythematosus (SLE) is a complex systemic autoimmune disorder characterized by a wide range of immunological abnormalities. One of its notable features is the potential to affect the central nervous system (CNS), leading to diverse neurological manifestations including headaches, acute state of confusion, seizure disorder, cerebrovascular disease, and meningoencephalitis. CNS involvement is highly prevalent in SLE patients, ranging from 12% to 75%, and is associated with a poor prognosis. Treatment options such as steroids, azathioprine, rituximab, and intravenous immunoglobulins (IVIGs) have been considered in managing these cases. However, early diagnosis of SLE-related CNS involvement remains challenging due to the rapid progression of symptoms and the need to exclude various alternative diagnoses. In this article, we present a fatal case of an SLE patient with meningoencephalitis, initially resembling an ischemic stroke. Despite administering antibiotics, antiviral agents, IVIGs, steroids, rituximab, and plasma exchange, no clinical improvement was observed.","PeriodicalId":324885,"journal":{"name":"Journal of Multiple Sclerosis and Neuroimmunology","volume":"21 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"124852613","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-06-30DOI: 10.59578/jmsni.2023.14.1.64
Euihyun Sung, J. Jeon, Young Seo Kim
베체트병은 구강궤양, 외음부궤양 및 포도막염을 특징으로 하는 전신염증성질환이다. 피부와 눈뿐만 아니라 관절, 위장관, 심장 등 장기에서도 질환이 나타날 수 있으며, 중추신경계 침범 은 비교적 드물지만 이환율과 사망률이 높다는 점에서 중요하 다. 신경베체트병은 대뇌반구, 뇌간, 척수 등 뇌 실질을 침범하 는 경우와 실질 밖 혈관을 침범하는 경우, 신경 또는 근육 등 말 초를 침범하는 경우로 분류할 수 있다. 혈액검사, 뇌척수액검 사, 영상검사 등을 시행하여 다발성경화증, 시신경척수염범주 질환, 뇌경색, 뇌수막염 등을 배제 진단함으로써 진단한다. 치 료는 일반적인 베체트병의 치료와 유사하게 글루코코티코이드, 아자싸이오프린, 메토트렉세이트, 사이클로포스퍼마이드와 같 은 면역억제제를 투여하며, 치료저항성의 경우 생물학적 제제 도 사용하고 있다. 저자들은 베체트병 환자에서 반복적으로 뇌간 및 대뇌반구를 침범하는 병변이 발생하였으나 특별한 신 경학적 증상 없이 반복적인 두통을 호소하였고 빠른 스테로이 드 치료 이후 호전되는 증례를 경험하였기에 이를 보고하는 바 이다. ■ ■ 증례
{"title":"Recurrent Parenchymal Neuro-Behcet’s Disease Presenting Only with Headache","authors":"Euihyun Sung, J. Jeon, Young Seo Kim","doi":"10.59578/jmsni.2023.14.1.64","DOIUrl":"https://doi.org/10.59578/jmsni.2023.14.1.64","url":null,"abstract":"베체트병은 구강궤양, 외음부궤양 및 포도막염을 특징으로 하는 전신염증성질환이다. 피부와 눈뿐만 아니라 관절, 위장관, 심장 등 장기에서도 질환이 나타날 수 있으며, 중추신경계 침범 은 비교적 드물지만 이환율과 사망률이 높다는 점에서 중요하 다. 신경베체트병은 대뇌반구, 뇌간, 척수 등 뇌 실질을 침범하 는 경우와 실질 밖 혈관을 침범하는 경우, 신경 또는 근육 등 말 초를 침범하는 경우로 분류할 수 있다. 혈액검사, 뇌척수액검 사, 영상검사 등을 시행하여 다발성경화증, 시신경척수염범주 질환, 뇌경색, 뇌수막염 등을 배제 진단함으로써 진단한다. 치 료는 일반적인 베체트병의 치료와 유사하게 글루코코티코이드, 아자싸이오프린, 메토트렉세이트, 사이클로포스퍼마이드와 같 은 면역억제제를 투여하며, 치료저항성의 경우 생물학적 제제 도 사용하고 있다. 저자들은 베체트병 환자에서 반복적으로 뇌간 및 대뇌반구를 침범하는 병변이 발생하였으나 특별한 신 경학적 증상 없이 반복적인 두통을 호소하였고 빠른 스테로이 드 치료 이후 호전되는 증례를 경험하였기에 이를 보고하는 바 이다. ■ ■ 증례","PeriodicalId":324885,"journal":{"name":"Journal of Multiple Sclerosis and Neuroimmunology","volume":"1 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"129471315","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-06-30DOI: 10.59578/jmsni.2023.14.1.59
Sung-chul Lim, J. Oh, Jeong-U Park, Sanghyun Kim, Jusuck Lee
Reversible splenial lesion syndrome (RESLES) is a complex clinicoradiological entity that has been reported to occur secondary to several disorders, including acute encephalitis/encephalopathy. On magnetic resonance imaging, it presents as reversible hyperintense signal lesions on diffusion-weighted images and hypointense on apparent diffusion coefficient images. Most case of RESLES associated with encephalitis/encephalopathy have a good prognosis, but some require intensive care or have poor prognosis. We report a case of RESLES associated with encephalitis/encephalopathy in which neurological deterioration improved after steroid treatment.
{"title":"Reversible Splenial Lesion Syndrome Associated with Encephalitis/Encephalopathy Improved with Short-Term Steroid Treatment","authors":"Sung-chul Lim, J. Oh, Jeong-U Park, Sanghyun Kim, Jusuck Lee","doi":"10.59578/jmsni.2023.14.1.59","DOIUrl":"https://doi.org/10.59578/jmsni.2023.14.1.59","url":null,"abstract":"Reversible splenial lesion syndrome (RESLES) is a complex clinicoradiological entity that has been reported to occur secondary to several disorders, including acute encephalitis/encephalopathy. On magnetic resonance imaging, it presents as reversible hyperintense signal lesions on diffusion-weighted images and hypointense on apparent diffusion coefficient images. Most case of RESLES associated with encephalitis/encephalopathy have a good prognosis, but some require intensive care or have poor prognosis. We report a case of RESLES associated with encephalitis/encephalopathy in which neurological deterioration improved after steroid treatment.","PeriodicalId":324885,"journal":{"name":"Journal of Multiple Sclerosis and Neuroimmunology","volume":"16 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"128048849","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-06-30DOI: 10.59578/jmsni.2023.14.1.51
Suhyung Kim, Eun-Jae Lee, J. Min, Sung-Min Kim, H. Shin, Y. Kwon, Woojun Kim, Jeeyoung Oh, Jun Soon Kim, S. Baek, I. Joo, Yoon-Ho Hong, M. Park, E. Sohn, T. Nam, Sun-Young Oh, S. Huh, Tae-Kyeong Lee, Jiwon Yang, Sunyoung Kim, H. Seok, Nam-Hee Kim, Jin-Hong Shin, J. B. Bong, Soonwook Kwon, Seong-il Oh, E. Cho, Hyun-June Shin, H. Lee, K. Park, W. Kim, J. Bae, H. Kim
Background: In the present era of rapidly evolving treatment options, there is a need for better understanding of the epidemiology and current management of patients with multiple sclerosis (MS). A survey was conducted to determine the epidemiologic characteristics and the current treatment status of patients with MS in Korea.Methods: In April 2022, we collected data on patients with MS, including number participating in follow up, sex ratio, current disease modifying treatment (DMT), and disability status (Expanded Disability Status Score, EDSS) from 30 major MS centers in Korea.Results: A total of 1,290 patients with MS was identified, with a female to male ratio of 2.4:1. Geographically, 1,047 patients (81%) were being monitored at hospitals in the capital regions (Seoul, Incheon, and Gyeonggi-do), while 243 (19%) were receiving care at hospitals outside the capital regions. There were 1,199 patients on DMTs, with 876 (73%) taking drugs with moderate efficacy and 264 (22%) taking drugs with high efficacy. Patients with current EDSS score greater than 3.0 and 5.5 numbered 242 (20%) and 105 (9%), respectively.Conclusion: The hospital-based prevalence survey revealed the number and geographical distribution of patients with MS in Korea.
{"title":"Multiple Sclerosis in Korea: A Hospital-Based, Multicenter, Epidemiological Study","authors":"Suhyung Kim, Eun-Jae Lee, J. Min, Sung-Min Kim, H. Shin, Y. Kwon, Woojun Kim, Jeeyoung Oh, Jun Soon Kim, S. Baek, I. Joo, Yoon-Ho Hong, M. Park, E. Sohn, T. Nam, Sun-Young Oh, S. Huh, Tae-Kyeong Lee, Jiwon Yang, Sunyoung Kim, H. Seok, Nam-Hee Kim, Jin-Hong Shin, J. B. Bong, Soonwook Kwon, Seong-il Oh, E. Cho, Hyun-June Shin, H. Lee, K. Park, W. Kim, J. Bae, H. Kim","doi":"10.59578/jmsni.2023.14.1.51","DOIUrl":"https://doi.org/10.59578/jmsni.2023.14.1.51","url":null,"abstract":"Background: In the present era of rapidly evolving treatment options, there is a need for better understanding of the epidemiology and current management of patients with multiple sclerosis (MS). A survey was conducted to determine the epidemiologic characteristics and the current treatment status of patients with MS in Korea.Methods: In April 2022, we collected data on patients with MS, including number participating in follow up, sex ratio, current disease modifying treatment (DMT), and disability status (Expanded Disability Status Score, EDSS) from 30 major MS centers in Korea.Results: A total of 1,290 patients with MS was identified, with a female to male ratio of 2.4:1. Geographically, 1,047 patients (81%) were being monitored at hospitals in the capital regions (Seoul, Incheon, and Gyeonggi-do), while 243 (19%) were receiving care at hospitals outside the capital regions. There were 1,199 patients on DMTs, with 876 (73%) taking drugs with moderate efficacy and 264 (22%) taking drugs with high efficacy. Patients with current EDSS score greater than 3.0 and 5.5 numbered 242 (20%) and 105 (9%), respectively.Conclusion: The hospital-based prevalence survey revealed the number and geographical distribution of patients with MS in Korea.","PeriodicalId":324885,"journal":{"name":"Journal of Multiple Sclerosis and Neuroimmunology","volume":"41 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"129961987","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-06-30DOI: 10.59578/jmsni.2023.14.1.44
Ilhan Yoo
Rituximab is a monoclonal chimeric antibody that targets CD20, which is expressed on the surface of B cells ranging from pre-B cells to mature B cells; rituximab treatment leads to depletion of B cells. It is widely used to treat various autoimmune neurological diseases including neuromyelitis optica spectrum disorder, multiple sclerosis, myasthenia gravis, chronic inflammatory demyelinating polyradiculoneuropathy, and autoimmune encephalitis. When administering rituximab for treatment, it is essential to consider potential adverse reactions, such as hypersensitivity, infection, and hypogammaglobulinemia, and to monitor B cell levels regularly to maintain the efficacy of treatment and prevent relapse. This review aims to explore the treatment of neuroimmunologic disorders with rituximab.
{"title":"Rituximab Treatment for Neuroimmunologic Disorders","authors":"Ilhan Yoo","doi":"10.59578/jmsni.2023.14.1.44","DOIUrl":"https://doi.org/10.59578/jmsni.2023.14.1.44","url":null,"abstract":"Rituximab is a monoclonal chimeric antibody that targets CD20, which is expressed on the surface of B cells ranging from pre-B cells to mature B cells; rituximab treatment leads to depletion of B cells. It is widely used to treat various autoimmune neurological diseases including neuromyelitis optica spectrum disorder, multiple sclerosis, myasthenia gravis, chronic inflammatory demyelinating polyradiculoneuropathy, and autoimmune encephalitis. When administering rituximab for treatment, it is essential to consider potential adverse reactions, such as hypersensitivity, infection, and hypogammaglobulinemia, and to monitor B cell levels regularly to maintain the efficacy of treatment and prevent relapse. This review aims to explore the treatment of neuroimmunologic disorders with rituximab.","PeriodicalId":324885,"journal":{"name":"Journal of Multiple Sclerosis and Neuroimmunology","volume":"13 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"122757579","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-06-30DOI: 10.59578/jmsni.2023.14.1.15
Woojun Kim
Neuromyelitis optica spectrum disorder (NMOSD) is caused by antibodies that target the aquaporin-4 (AQP4) water channel expressed on astrocytes. Specific antibody binding to AQP4 produces complement-dependent cytotoxicity, resulting in inflammation and demyelination. New biologic treatments demonstrate high efficacy and good safety for patients with AQP4-immunoglobulin G-positive NMOSD. They were eculizumab, an anti-complement C5 antibody, satralizumab, an anti-interleukin-6 receptor antibody, and inebilizumab and rituximab, which targets CD19 and CD20, respectively, causing depletion of B-cells. In this review, the pathophysiology of NMOSD, the methodology and results of the recent studies examining monoclonal antibody therapies, and the optimal therapeutic strategy for NMOSD were covered.
{"title":"Monoclonal Antibody Therapies for Neuromyelitis Optica Spectrum Disorder","authors":"Woojun Kim","doi":"10.59578/jmsni.2023.14.1.15","DOIUrl":"https://doi.org/10.59578/jmsni.2023.14.1.15","url":null,"abstract":"Neuromyelitis optica spectrum disorder (NMOSD) is caused by antibodies that target the aquaporin-4 (AQP4) water channel expressed on astrocytes. Specific antibody binding to AQP4 produces complement-dependent cytotoxicity, resulting in inflammation and demyelination. New biologic treatments demonstrate high efficacy and good safety for patients with AQP4-immunoglobulin G-positive NMOSD. They were eculizumab, an anti-complement C5 antibody, satralizumab, an anti-interleukin-6 receptor antibody, and inebilizumab and rituximab, which targets CD19 and CD20, respectively, causing depletion of B-cells. In this review, the pathophysiology of NMOSD, the methodology and results of the recent studies examining monoclonal antibody therapies, and the optimal therapeutic strategy for NMOSD were covered.","PeriodicalId":324885,"journal":{"name":"Journal of Multiple Sclerosis and Neuroimmunology","volume":"88 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"124179689","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-06-30DOI: 10.59578/jmsni.2023.14.1.56
Dong-Young Seo, H. Shin, Jin ho Kim, Se Hoon Kim, Seung Woo Kim
Immune-related neuromuscular complications due to immune checkpoint inhibitors (ICIs) are widely reported. However, cases involving vasculitic neuropathy (VN) are rare. We report a case of pembrolizumab-associated VN, confirmed by nerve biopsy, presenting as mononeuritis multiplex with erythematous skin lesions. Based on negative serological tests for other causes of vasculitis and temporal relationship with ICI treatment, vasculitis was suspected to be associated with pembrolizumab. VN should be considered in patients with progressive neurological symptoms and skin lesions after receiving ICI therapy.
{"title":"Vasculitic Neuropathy Associated with Pembrolizumab","authors":"Dong-Young Seo, H. Shin, Jin ho Kim, Se Hoon Kim, Seung Woo Kim","doi":"10.59578/jmsni.2023.14.1.56","DOIUrl":"https://doi.org/10.59578/jmsni.2023.14.1.56","url":null,"abstract":"Immune-related neuromuscular complications due to immune checkpoint inhibitors (ICIs) are widely reported. However, cases involving vasculitic neuropathy (VN) are rare. We report a case of pembrolizumab-associated VN, confirmed by nerve biopsy, presenting as mononeuritis multiplex with erythematous skin lesions. Based on negative serological tests for other causes of vasculitis and temporal relationship with ICI treatment, vasculitis was suspected to be associated with pembrolizumab. VN should be considered in patients with progressive neurological symptoms and skin lesions after receiving ICI therapy.","PeriodicalId":324885,"journal":{"name":"Journal of Multiple Sclerosis and Neuroimmunology","volume":"109 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"125577730","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-06-30DOI: 10.59578/jmsni.2023.14.1.71
K. Shin, Sukyoon Lee, Seong-il Oh
Copyright c 2023 by Korean Society of Neuroimmunology 71 38세 남자가 수개월 전부터 발생한 보행장애로 방문하였다. 이전의 매독 병력은 뚜렷하지 않았으나, 수주 전부터 보행장애 가 악화되었다. 다리에서 온도감각과 통증감각은 정상이지만, 고유감각과 진동감각의 저하 그리고 심부건반사 저하가 있었다. 척수 자기공명영상에서 C6부터 T10 척수분절까지 고신호강도 가 있었다(Fig. 1). 긴광범위횡단척수염으로 판단 하에 고용량 스테로이드(1 g/일, 5일간) 정맥주사를 하였다. 뇌척수액검사에 서 백혈구는 10/mm, 단백질 80.7 mg/dL, 포도당 50 mg/dL (혈청 포도당 93 mg/dL)였다. 매독혈청검사(venereal disease research laboratory test, VDRL)는 약양성이었고 뇌척수액 VDRL의 역가는 1:4로 확인되어, 신경매독에 의한 긴광범위횡 단척수염으로 진단하여 페니실린G칼륨(2,400만 U/일) 정맥주 사를 14일간 투여하였고, 보행장애는 일부 호전되었다. 척수매 독은 후기 신경매독의 중요한 합병증이지만, 첫 임상 표현형으 로 긴광범위횡단척수염으로 나타나는 것은 드물다. 긴광범위횡 단척수염에 대한 원인으로 염증성 척수병 외에도 척수매독에 대 한 감별을 필요로 할 것이다.
Copyright c 2023 by Korean Society of Neuroimmunology 71岁38岁男子因数月前发生的步行障碍而访问。以前的梅毒病史并不明显，但步行障碍从几周前开始恶化。腿上的温度感觉和疼痛感觉正常，但有固有感觉和振动感觉的下降，还有深部键盘下降。在脊髓核磁共振成像中，从C6到T10脊髓分节有高信号强度(Fig)。1).判断为长范围横断脊髓炎，静脉注射高剂量类固醇(1克/天，5天)。在脑脊液检查中，白细胞为10/mm、蛋白质80.7 mg/dL、葡萄糖50 mg/dL(血清葡萄糖93 mg/dL)。梅毒血清检测(venereal disease research laboratory test, VDRL)呈药物阳性，脑脊液VDRL的逆值为1:4，诊断为神经梅毒引起的长范围横断脊髓炎，静脉注射青霉素G钾(2400万U/天)14天，步行障碍有所好转。脊髓梅毒是后期神经梅毒的重要并发症，但作为第一个临床表现型，很少出现长范围横断脊髓炎。作为长范围横断脊髓炎的原因，除了炎症性脊髓病外，还需要对脊髓梅毒进行鉴别。
{"title":"Neurosyphilis Presenting as Longitudinally Extensive Transverse Myelitis","authors":"K. Shin, Sukyoon Lee, Seong-il Oh","doi":"10.59578/jmsni.2023.14.1.71","DOIUrl":"https://doi.org/10.59578/jmsni.2023.14.1.71","url":null,"abstract":"Copyright c 2023 by Korean Society of Neuroimmunology 71 38세 남자가 수개월 전부터 발생한 보행장애로 방문하였다. 이전의 매독 병력은 뚜렷하지 않았으나, 수주 전부터 보행장애 가 악화되었다. 다리에서 온도감각과 통증감각은 정상이지만, 고유감각과 진동감각의 저하 그리고 심부건반사 저하가 있었다. 척수 자기공명영상에서 C6부터 T10 척수분절까지 고신호강도 가 있었다(Fig. 1). 긴광범위횡단척수염으로 판단 하에 고용량 스테로이드(1 g/일, 5일간) 정맥주사를 하였다. 뇌척수액검사에 서 백혈구는 10/mm, 단백질 80.7 mg/dL, 포도당 50 mg/dL (혈청 포도당 93 mg/dL)였다. 매독혈청검사(venereal disease research laboratory test, VDRL)는 약양성이었고 뇌척수액 VDRL의 역가는 1:4로 확인되어, 신경매독에 의한 긴광범위횡 단척수염으로 진단하여 페니실린G칼륨(2,400만 U/일) 정맥주 사를 14일간 투여하였고, 보행장애는 일부 호전되었다. 척수매 독은 후기 신경매독의 중요한 합병증이지만, 첫 임상 표현형으 로 긴광범위횡단척수염으로 나타나는 것은 드물다. 긴광범위횡 단척수염에 대한 원인으로 염증성 척수병 외에도 척수매독에 대 한 감별을 필요로 할 것이다.","PeriodicalId":324885,"journal":{"name":"Journal of Multiple Sclerosis and Neuroimmunology","volume":"18 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"132108265","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-06-30DOI: 10.59578/jmsni.2023.14.1.1
Jiwon Yang
Since the McDonald criteria were first established for the diagnosis of multiple sclerosis (MS) in 2010, the role of MRI has become increasingly important. In order to diagnose MS as accurately as possible, neurologists should understand the McDonald MRI criteria and apply it appropriately. In this article, we review the definition of MS lesions, the exact location stated as dissemination in space in the 2017 McDonald criteria, and the new MS-specific MRI features.
{"title":"Understanding MRI Features of Multiple Sclerosis: Based on the 2017 McDonald Criteria","authors":"Jiwon Yang","doi":"10.59578/jmsni.2023.14.1.1","DOIUrl":"https://doi.org/10.59578/jmsni.2023.14.1.1","url":null,"abstract":"Since the McDonald criteria were first established for the diagnosis of multiple sclerosis (MS) in 2010, the role of MRI has become increasingly important. In order to diagnose MS as accurately as possible, neurologists should understand the McDonald MRI criteria and apply it appropriately. In this article, we review the definition of MS lesions, the exact location stated as dissemination in space in the 2017 McDonald criteria, and the new MS-specific MRI features.","PeriodicalId":324885,"journal":{"name":"Journal of Multiple Sclerosis and Neuroimmunology","volume":"31 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134067931","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
京公网安备 11010802042870号
京ICP备2023020795号-1
扫码关注我们
求助内容：
应助结果提醒方式：