{"title":"Acute Necrotizing Encephalopathy Associated with COVID-19","authors":"Sumin Kim, J. Sunwoo","doi":"10.17340/jkna.2023.0085","DOIUrl":"https://doi.org/10.17340/jkna.2023.0085","url":null,"abstract":"","PeriodicalId":437080,"journal":{"name":"Journal of the Korean Neurological Association","volume":"256 ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139832665","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":"393 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139833107","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}
Maternally inherited diabetes and deafness (MIDD) is a disorder characterized by the occurrence of diabetes mellitus and hearing impairment at a young age, caused by point mutations in mitochondrial DNA. MIDD can be accompanied by various neurological symptoms. In this patient, memory impairment and ataxia were the predominant neurological symptoms. Herein, we report MIDD patients with the m.3243A>G mutation who present with cognitive decline and gait disturbance at the age of 59.
母系遗传性糖尿病和耳聋（MIDD）是由线粒体 DNA 的点突变引起的一种疾病，其特征是在幼年时就出现糖尿病和听力障碍。MIDD 可伴有各种神经系统症状。在该患者中，记忆障碍和共济失调是主要的神经系统症状。在此，我们报告了 m.3243A>G 突变的 MIDD 患者，他们在 59 岁时出现认知能力下降和步态障碍。
{"title":"Maternally Inherited Diabetes and Deafness Presenting with Memory Impairment and Gait Disturbance","authors":"Jung Seok Lee","doi":"10.17340/jkna.2023.0067","DOIUrl":"https://doi.org/10.17340/jkna.2023.0067","url":null,"abstract":"Maternally inherited diabetes and deafness (MIDD) is a disorder characterized by the occurrence of diabetes mellitus and hearing impairment at a young age, caused by point mutations in mitochondrial DNA. MIDD can be accompanied by various neurological symptoms. In this patient, memory impairment and ataxia were the predominant neurological symptoms. Herein, we report MIDD patients with the m.3243A>G mutation who present with cognitive decline and gait disturbance at the age of 59.","PeriodicalId":437080,"journal":{"name":"Journal of the Korean Neurological Association","volume":"23 11","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139885684","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}
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":"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":"139885737","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}
A 38-year-old male was diagnosed with spinal muscular atrophy (SMA) type 3a, previously misdiagnosed as muscular dystrophy. He began treatment with nusinersen after getting approval from health insurance service. Cerebrospinal fluid (CSF) was collected when nusinersen was intrathecally injected. We measured the neurofilament light chain (NfL) level using a single molecular array as an efficacy biomarker. The CSF NfL level was markedly decreased after finishing the loading dosage. Our case implies that nusinersen is also effective in adults with SMA as a biomarker from real-world evidence.
一名 38 岁的男性被诊断患有脊髓性肌萎缩症（SMA）3a 型，之前曾被误诊为肌肉萎缩症。在获得医疗保险服务机构的批准后，他开始接受努西那生治疗。在鞘内注射奴西能生时收集了脑脊液（CSF）。我们使用单分子阵列测量了神经丝蛋白轻链（NfL）水平，作为疗效生物标志物。完成负荷剂量后，CSF NfL水平明显下降。我们的病例表明，从现实世界的证据来看，作为一种生物标志物，奴西那生对成人 SMA 患者同样有效。
{"title":"A Case of Spinal Muscular Atrophy Type 3a Showing Improvement in Neurofilament Light Chain of Cerebrospinal Fluid: Real-world Evidence","authors":"Pan-Woo Ko, Jong-Mok Lee","doi":"10.17340/jkna.2023.0049","DOIUrl":"https://doi.org/10.17340/jkna.2023.0049","url":null,"abstract":"A 38-year-old male was diagnosed with spinal muscular atrophy (SMA) type 3a, previously misdiagnosed as muscular dystrophy. He began treatment with nusinersen after getting approval from health insurance service. Cerebrospinal fluid (CSF) was collected when nusinersen was intrathecally injected. We measured the neurofilament light chain (NfL) level using a single molecular array as an efficacy biomarker. The CSF NfL level was markedly decreased after finishing the loading dosage. Our case implies that nusinersen is also effective in adults with SMA as a biomarker from real-world evidence.","PeriodicalId":437080,"journal":{"name":"Journal of the Korean Neurological Association","volume":"131 ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139830219","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":"Patient with Creutzfeldt-Jakob Disease Who Presented Peripheral Type Facial Palsy","authors":"Y. Soung, Sang-Jae Lee, So-Hee Lee, Jae-Won Jang","doi":"10.17340/jkna.2023.0073","DOIUrl":"https://doi.org/10.17340/jkna.2023.0073","url":null,"abstract":"","PeriodicalId":437080,"journal":{"name":"Journal of the Korean Neurological Association","volume":"18 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139888296","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":"Acute Necrotizing Encephalopathy Associated with COVID-19","authors":"Sumin Kim, J. Sunwoo","doi":"10.17340/jkna.2023.0085","DOIUrl":"https://doi.org/10.17340/jkna.2023.0085","url":null,"abstract":"","PeriodicalId":437080,"journal":{"name":"Journal of the Korean Neurological Association","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139892834","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":"249 ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139820889","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}
Namyoung Kim, Jeungwan Han, Sang Jin Park, Yoon Shik Chun, Yohan Ju, Hyun-Jae Kim
Optic neuritis is a rare extraintestinal manifestation of inflammatory bowel disease. Antitumor necrosis factor alpha (anti-TNF-α) agents are essential treatment options in inflammatory bowel diseases. However, anti-TNF-α agents can implicate optic neuritis and other demyelinating diseases as complications of these agents. We report a patient of infliximab-induced optic neuritis in a patient with ulcerative colitis who was treated with high-dose steroids after discontinuation of infliximab.
{"title":"Optic Neuritis after Infliximab Treatment in a Patient with Ulcerative Colitis","authors":"Namyoung Kim, Jeungwan Han, Sang Jin Park, Yoon Shik Chun, Yohan Ju, Hyun-Jae Kim","doi":"10.17340/jkna.2023.0087","DOIUrl":"https://doi.org/10.17340/jkna.2023.0087","url":null,"abstract":"Optic neuritis is a rare extraintestinal manifestation of inflammatory bowel disease. Antitumor necrosis factor alpha (anti-TNF-α) agents are essential treatment options in inflammatory bowel diseases. However, anti-TNF-α agents can implicate optic neuritis and other demyelinating diseases as complications of these agents. We report a patient of infliximab-induced optic neuritis in a patient with ulcerative colitis who was treated with high-dose steroids after discontinuation of infliximab.","PeriodicalId":437080,"journal":{"name":"Journal of the Korean Neurological Association","volume":"16 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139883881","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":"Giant Brainstem Arteriovenous Malformation with Spiral Venous Ectasia","authors":"Jusuck Lee, Yoonha Hwang, Sang-Mi Noh","doi":"10.17340/jkna.2023.0056","DOIUrl":"https://doi.org/10.17340/jkna.2023.0056","url":null,"abstract":"","PeriodicalId":437080,"journal":{"name":"Journal of the Korean Neurological Association","volume":"1081 ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139820436","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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