Elsberg syndrome is a rare disease of lumbosacral myeloradiculitis, mostly caused by herpes simplex virus type 2 (HSV-2). It frequently has concomitant myelitis, and immunocompromised patients can be fatal with ascending myelitis. Since anti-viral agents might lead to good recovery, clinicians need to be suspicious for conditions of cauda equina syndrome. Detection of viral DNA from cerebrospinal fluid ensures the diagnosis. We report a case of Elsberg syndrome caused by HSV-2 and are to delineate the clinical and radiologic spectrum.
埃尔斯伯格综合征是一种罕见的腰骶部脊髓脊膜炎，主要由单纯疱疹病毒 2 型（HSV-2）引起。这种疾病经常会并发脊髓炎，免疫力低下的患者可能会因升天性脊髓炎而致命。由于抗病毒药物可能会使患者恢复良好，临床医生需要对马尾综合征的病症保持警惕。从脑脊液中检测病毒 DNA 可确保诊断。我们报告了一例由 HSV-2 引起的埃尔斯伯格综合征，并对其临床和影像学特征进行了描述。
{"title":"Atypical Presentation of Elsberg Syndrome Caused by Herpes Simplex Virus Type 2 Infection","authors":"Yuri Je, Jin Woo No, Young-Eun Park","doi":"10.17340/jkna.2023.0078","DOIUrl":"https://doi.org/10.17340/jkna.2023.0078","url":null,"abstract":"Elsberg syndrome is a rare disease of lumbosacral myeloradiculitis, mostly caused by herpes simplex virus type 2 (HSV-2). It frequently has concomitant myelitis, and immunocompromised patients can be fatal with ascending myelitis. Since anti-viral agents might lead to good recovery, clinicians need to be suspicious for conditions of cauda equina syndrome. Detection of viral DNA from cerebrospinal fluid ensures the diagnosis. We report a case of Elsberg syndrome caused by HSV-2 and are to delineate the clinical and radiologic spectrum.","PeriodicalId":437080,"journal":{"name":"Journal of the Korean Neurological Association","volume":"187 ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139825848","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}
Although immune checkpoint inhibitor-related encephalitis has been reported, its underlying mechanism and treatment strategies remain unclear. Here, we present the case of a 60-yearold male patient with small cell lung cancer who developed autoimmune encephalitis during treatment with atezolizumab. Notably, this case involved the concurrent detection of two paraneoplastic autoantibodies (anti-Hu and anti-SOX1). Encephalitis was treated by discontinuing atezolizumab and administering steroids, despite the persistence of the antibodies. This case provides insights into the interaction between immune checkpoint inhibitors and paraneoplastic autoantibodies.
{"title":"Coexistence of Anti-Hu and Anti-SOX1 Autoantibodies in Atezolizumab-related Encephalitis","authors":"Mi-Yeun Eun, Hyungseok Hah, Jaechun Hwang","doi":"10.17340/jkna.2023.0070","DOIUrl":"https://doi.org/10.17340/jkna.2023.0070","url":null,"abstract":"Although immune checkpoint inhibitor-related encephalitis has been reported, its underlying mechanism and treatment strategies remain unclear. Here, we present the case of a 60-yearold male patient with small cell lung cancer who developed autoimmune encephalitis during treatment with atezolizumab. Notably, this case involved the concurrent detection of two paraneoplastic autoantibodies (anti-Hu and anti-SOX1). Encephalitis was treated by discontinuing atezolizumab and administering steroids, despite the persistence of the antibodies. This case provides insights into the interaction between immune checkpoint inhibitors and paraneoplastic autoantibodies.","PeriodicalId":437080,"journal":{"name":"Journal of the Korean Neurological Association","volume":"16 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139826440","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}
Yi-Seul Choo, Ga Eun Koo, Yu-Jin Kang, Dongwook Kang, Young Jun Ko, Ji Young Park, Chan-Young Park, Su-Hyun Han
Noncirrhotic hyperammonemia as a cause of acute confusion remains diagnostic challenge. Deficiency of ornithine transcarbamylase (OTC) is the urea cycle disorder, inborn errors caused by a defect of the enzymes in the urea cycle, leading to an accumulation of ammonia mainly in newborn. There were very few cases, in which OTC deficiency result in hyperammonemia in adulthood. Herein, we report a young adult woman of hyperammonemic encephalopathy with OTC deficiency, diagnosed by high blood ammonia, glutamine and low plasma levels of citrulline. Next generation sequencing showed the c.386+5G>A mutation of the OTC gene.
{"title":"Hyperammonemic Encephalopathy Caused by the c.386+5G>A Mutation in OTC Gene in a Young Adult Woman","authors":"Yi-Seul Choo, Ga Eun Koo, Yu-Jin Kang, Dongwook Kang, Young Jun Ko, Ji Young Park, Chan-Young Park, Su-Hyun Han","doi":"10.17340/jkna.2023.0075","DOIUrl":"https://doi.org/10.17340/jkna.2023.0075","url":null,"abstract":"Noncirrhotic hyperammonemia as a cause of acute confusion remains diagnostic challenge. Deficiency of ornithine transcarbamylase (OTC) is the urea cycle disorder, inborn errors caused by a defect of the enzymes in the urea cycle, leading to an accumulation of ammonia mainly in newborn. There were very few cases, in which OTC deficiency result in hyperammonemia in adulthood. Herein, we report a young adult woman of hyperammonemic encephalopathy with OTC deficiency, diagnosed by high blood ammonia, glutamine and low plasma levels of citrulline. Next generation sequencing showed the c.386+5G>A mutation of the OTC gene.","PeriodicalId":437080,"journal":{"name":"Journal of the Korean Neurological Association","volume":"93 5","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139872810","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}
W. Jeong, Seung Min Kim, Heewon Bae, Miju Cheon, S. Ha
{"title":"Recent Cerebral Infarction in the Occipital Lobe Incidentally Detected by 18F-FP-CIT Positron Emission Tomography","authors":"W. Jeong, Seung Min Kim, Heewon Bae, Miju Cheon, S. Ha","doi":"10.17340/jkna.2023.0077","DOIUrl":"https://doi.org/10.17340/jkna.2023.0077","url":null,"abstract":"","PeriodicalId":437080,"journal":{"name":"Journal of the Korean Neurological Association","volume":"49 6","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139880833","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}
{"title":"OnabotulinumtoxinA (Botox®) Injection in the Treatment for Chronic Migraine","authors":"Sanghyo Ryu","doi":"10.17340/jkna.2023.0041","DOIUrl":"https://doi.org/10.17340/jkna.2023.0041","url":null,"abstract":"","PeriodicalId":437080,"journal":{"name":"Journal of the Korean Neurological Association","volume":"20 6","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139889593","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}
H. Kim, Doo Ri Kim, Joong-Goo Kim, Sook Keun Song, Chul-Hoo Kang
Hyperammonemic encephalopathy commonly occur in patients with liver disease, but it can also occur due to other causes. A portosystemic shunt without liver cirrhosis is one of the causes of non-cirrhotic hyperammonemic encephalopathy. Herein, we present a case of a 78-year-old male patient with hyperammonemic encephalopathy resulting from portosystemic shunts without liver cirrhosis, which was successfully treated with endovascular shunt closure.
{"title":"Non-cirrhotic Hyperammonemic Encephalopathy with Portosystemic Shunt","authors":"H. Kim, Doo Ri Kim, Joong-Goo Kim, Sook Keun Song, Chul-Hoo Kang","doi":"10.17340/jkna.2023.0080","DOIUrl":"https://doi.org/10.17340/jkna.2023.0080","url":null,"abstract":"Hyperammonemic encephalopathy commonly occur in patients with liver disease, but it can also occur due to other causes. A portosystemic shunt without liver cirrhosis is one of the causes of non-cirrhotic hyperammonemic encephalopathy. Herein, we present a case of a 78-year-old male patient with hyperammonemic encephalopathy resulting from portosystemic shunts without liver cirrhosis, which was successfully treated with endovascular shunt closure.","PeriodicalId":437080,"journal":{"name":"Journal of the Korean Neurological Association","volume":"27 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139893022","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}
{"title":"IgG4-related Spinal Pachymeningitis Causing Spinal Cord Compression","authors":"Jeong Bin Bong, Byeol-A Yoon","doi":"10.17340/jkna.2023.0074","DOIUrl":"https://doi.org/10.17340/jkna.2023.0074","url":null,"abstract":"","PeriodicalId":437080,"journal":{"name":"Journal of the Korean Neurological Association","volume":"66 5-6","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139879876","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}
{"title":"Mnemonics for Key Function of Prefrontal Cortex","authors":"Hanim Kwon, Hyung-Soo Lee, Moon Ho Park","doi":"10.17340/jkna.2023.0098","DOIUrl":"https://doi.org/10.17340/jkna.2023.0098","url":null,"abstract":"","PeriodicalId":437080,"journal":{"name":"Journal of the Korean Neurological Association","volume":"24 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139880362","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}
{"title":"IgG4-related Spinal Pachymeningitis Causing Spinal Cord Compression","authors":"Jeong Bin Bong, Byeol-A Yoon","doi":"10.17340/jkna.2023.0074","DOIUrl":"https://doi.org/10.17340/jkna.2023.0074","url":null,"abstract":"","PeriodicalId":437080,"journal":{"name":"Journal of the Korean Neurological Association","volume":"53 7","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139820244","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}
{"title":"Mnemonics for Key Function of Prefrontal Cortex","authors":"Hanim Kwon, Hyung-Soo Lee, Moon Ho Park","doi":"10.17340/jkna.2023.0098","DOIUrl":"https://doi.org/10.17340/jkna.2023.0098","url":null,"abstract":"","PeriodicalId":437080,"journal":{"name":"Journal of the Korean Neurological Association","volume":"555 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139820707","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}
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