{"title":"Understanding new-onset refractory status epilepticus from an immunological point of view.","authors":"Yong-Won Shin","doi":"10.47936/encephalitis.2021.00045","DOIUrl":"https://doi.org/10.47936/encephalitis.2021.00045","url":null,"abstract":"<p><p>New-onset refractory status epilepticus (NORSE) is unexpected onset of refractory status epilepticus in individuals with no preexisting relevant neurologic condition. The etiologies remain largely cryptogenic; treatment is challenging after failure to control seizures despite use of multiple antiepileptic drugs and anesthetic agents. Frequent fever and other infectious prodromes, elevated proinflammatory cytokine/chemokine levels, and limbic or multifocal brain lesions indicate active inflammation in NORSE. Among identified causes, autoimmune encephalitis is the most common and accounts for more than one-third of all known NORSE cases, followed by infection-related etiologies. Although more evidence is needed, anti-cytokine therapies with tocilizumab and anakinra along with other immunotherapeutic agents used in autoimmune encephalitis can aid in alleviating or hindering the inflammatory cascade and controlling seizures.</p>","PeriodicalId":72904,"journal":{"name":"Encephalitis (Seoul, Korea)","volume":"1 3","pages":"61-67"},"PeriodicalIF":0.0,"publicationDate":"2021-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/fe/ae/encephalitis-2021-00045.PMC10295883.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9845030","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Jung-Ick Byun, Ji-Yeon Bae, Jangsup Moon, Soon-Tae Lee, Keun-Hwa Jung, Kyung-Il Park, Manho Kim, Sang Kun Lee, Kon Chu
{"title":"Proportion of peripheral regulatory T cells in patients with autoimmune encephalitis.","authors":"Jung-Ick Byun, Ji-Yeon Bae, Jangsup Moon, Soon-Tae Lee, Keun-Hwa Jung, Kyung-Il Park, Manho Kim, Sang Kun Lee, Kon Chu","doi":"10.47936/encephalitis.2021.00052","DOIUrl":"https://doi.org/10.47936/encephalitis.2021.00052","url":null,"abstract":"<p><strong>Purpose: </strong>Regulatory T cells (Tregs) play a crucial role in maintaining immune tolerance. Any deficiency or dysfunction of the Tregs can influence the pathogenesis of autoimmune disease. This study aimed to assess the role of Tregs among patients with autoimmune encephalitis (AE) with different autoantibody types and to evaluate their association with clinical features.</p><p><strong>Methods: </strong>This was a cross-sectional observational study involving 29 patients with AE. Peripheral blood was sampled from each patient for flow cytometric analysis. Proportions of CD4<sup>+</sup>CD25<sup>+</sup> and CD4<sup>+</sup>CD25<sup>+</sup>Foxp3<sup>+</sup> Tregs were calculated and compared between the antibody types (synaptic, paraneoplastic, and undetermined). Associations between the proportion of Tregs and clinical features were also evaluated.</p><p><strong>Results: </strong>Five patients had synaptic autoantibodies, five had paraneoplastic autoantibodies, and the others were of an undetermined type. The proportion of CD4<sup>+</sup>CD25<sup>+</sup> Tregs tended to be higher in those with paraneoplastic antibodies than in those with synaptic antibodies (<i>post-hoc</i> p = 0.028) and undetermined antibody status (<i>post-hoc</i> p = 0.043). A significant negative correlation was found between the proportion of Tregs and the initial modified Rankin score (r = -0.391, p = 0.036). Those who received intravenous immunoglobulin had lower proportions of Tregs than those who did not.</p><p><strong>Conclusion: </strong>The results of the present study suggest that Tregs may play different roles according to the type of AE and may be linked to disease severity.</p>","PeriodicalId":72904,"journal":{"name":"Encephalitis (Seoul, Korea)","volume":"1 3","pages":"68-72"},"PeriodicalIF":0.0,"publicationDate":"2021-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/f9/4e/encephalitis-2021-00052.PMC10295879.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10221453","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Symptomatologic pathomechanism of <i>N</i>-methyl D-aspartate receptor encephalitis.","authors":"Woo-Jin Lee","doi":"10.47936/encephalitis.2021.00017","DOIUrl":"https://doi.org/10.47936/encephalitis.2021.00017","url":null,"abstract":"<p><p><i>N</i>-methyl D-aspartate receptor (NMDAR) encephalitis is a well-characterized clinical syndrome. The main molecular mechanism of NMDAR encephalitis is autoantibody-mediated NMDAR hypofunction in the neuronal synapse. Several pathomechanistic hypotheses might explain how NMDAR hypofunction causes the typical symptoms and prognosis of NMDAR encephalitis. Suppression of NMDAR-dependent gamma-aminobutyric acid interneurons provokes an accelerated activation of the positive feedback loops of the dorsolateral prefrontal cortex/subiculum-nucleus accumbens circuit in the striatum, the ventral tegmental area (VTA), and the nucleus reuniens in the thalamus-hippocampus-VTA loop. Dysregulated activation of the VTA and cortex via those positive feedback loops may explain the rapid clinical deterioration at acute stages of the disease and the well-characterized syndrome that includes limbic system dysfunction, intractable seizures, dyskinesia, coma, and the characteristic extreme delta brush. Progressive cerebellar atrophy is correlated with cumulative disease burden and is associated with worse long-term outcomes, which might be explained by the NMDAR-dependent pathways required to maintain neuronal survival. Those pathomechanistic hypotheses for NMDAR encephalitis support the rationale for the early introduction of combination immunotherapy and the use of adjuvant immunotherapy in patients with persisting symptoms in chronic disease phases.</p>","PeriodicalId":72904,"journal":{"name":"Encephalitis (Seoul, Korea)","volume":"1 2","pages":"36-44"},"PeriodicalIF":0.0,"publicationDate":"2021-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/51/e6/encephalitis-2021-00017.PMC10295887.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9845015","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Jung-Ju Lee, Kyusik Kang, Woong-Woo Lee, Byung-Kun Kim, Jong-Moo Park, Ohyun Kwon, Soohyun Cho
{"title":"Cerebral <i>Toxocara canis</i> larval infection presenting as delayed encephalopathy and nonconvulsive status epilepticus: a case report.","authors":"Jung-Ju Lee, Kyusik Kang, Woong-Woo Lee, Byung-Kun Kim, Jong-Moo Park, Ohyun Kwon, Soohyun Cho","doi":"10.47936/encephalitis.2021.00024","DOIUrl":"https://doi.org/10.47936/encephalitis.2021.00024","url":null,"abstract":"<p><p>Adult cerebral <i>Toxocara canis</i> larval infections are rare. Humans are infected by contacting soil that contains infectious eggs or by eating undercooked meat. We encountered a patient with cerebral <i>T. canis</i> infection who showed recurrent altered mentality with nonconvulsive status epilepticus (NCSE). A 71-year-old male was admitted with complaints of drowsy mentality and abnormal behavior. Magnetic resonance imaging revealed non-enhanced multiple gliosis and calcified lesions. Electroencephalography revealed NCSE, which was cured with valproate. However, he was readmitted because of recurrence of symptoms. On cerebrospinal fluid examination, <i>T. canis</i> antibodies were observed. Subsequently, he was treated with albendazole and corticosteroids. Although corticosteroid improved his symptoms, the therapy could not be continued because the patient developed septic shock. This is the first report of cerebral <i>T. canis</i> larval infection presenting as delayed encephalopathy and NCSE. In patients with unexplainable progressive encephalopathy, particularly when gliosis or calcified lesions are observed on neuroimaging, cerebral <i>T. canis</i> larval infection should be considered and treated promptly.</p>","PeriodicalId":72904,"journal":{"name":"Encephalitis (Seoul, Korea)","volume":"1 2","pages":"51-55"},"PeriodicalIF":0.0,"publicationDate":"2021-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/f4/d1/encephalitis-2021-00024.PMC10295886.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9835140","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ji-Hun Choi, Dong-Woo Kim, Hye-Jee La, Hyoshin Son, Soon-Tae Lee
{"title":"Food memory test to evaluate memory function.","authors":"Ji-Hun Choi, Dong-Woo Kim, Hye-Jee La, Hyoshin Son, Soon-Tae Lee","doi":"10.47936/encephalitis.2021.00031","DOIUrl":"https://doi.org/10.47936/encephalitis.2021.00031","url":null,"abstract":"<p><strong>Purpose: </strong>To evaluate cognitive function with ease at bedside, we developed a novel neurologic exam called the \"food memory test (FMT)\" and evaluated its validity for use in clinical practice.</p><p><strong>Methods: </strong>In this prospective study in a neurology clinic, we asked patients about what they had eaten for the soup and main dish at the last meal [FMT1] and the second-to-last meal [FMT2]. If they answered correctly for both the soup and main dish, they received a \"pass\" score. If they did not answer or chose the wrong food, they received a \"fail\" score. We also performed conventional cognitive function tests for comparison.</p><p><strong>Results: </strong>A total of 27 patients was enrolled, and 12 (44.4%) passed the FMT1 test. FMT1 has a strong correlation with conventional memory function tests, including time-place orientation, three-word recall, the Seoul Verbal Learning Test, and the Rey Complex Figure Test . FMT1 was not correlated with a depression score or with frontal lobe function tests. FMT2 showed less significant correlation with conventional memory tests.</p><p><strong>Conclusion: </strong>These results suggest that FMT1 is a reliable bedside test to evaluate recent memory. Clinical application of FMT in daily clinical practice is warranted.</p>","PeriodicalId":72904,"journal":{"name":"Encephalitis (Seoul, Korea)","volume":"1 2","pages":"45-50"},"PeriodicalIF":0.0,"publicationDate":"2021-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/26/f0/encephalitis-2021-00031.PMC10295888.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9835136","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"New-onset epilepsy in a patient with myelin oligodendrocyte glycoprotein antibodies.","authors":"Hansol Im, Taewon Kim","doi":"10.47936/encephalitis.2020.00073","DOIUrl":"https://doi.org/10.47936/encephalitis.2020.00073","url":null,"abstract":"<p><p>In the literature, several adult cases with myelin oligodendrocyte glycoprotein (MOG) antibodies (Abs)-associated disorders have been reported to have seizure and acute disseminated encephalomyelitis (ADEM) as the main clinical manifestations, but the number is somewhat low. Because of its rarity, the clinical characteristics and a consensus on treatment have not yet been established in the adult form of ADEM and seizure phenotypes in MOG-Abs-associated disorders. In this report, we described an adult patient who presented with status epilepticus as the index event, had been suffering from chronic epilepsy, and had positive antibodies for MOG. Neither increasing the doses of the antiseizure drugs (ASDs) nor adding another new ASD reduced the prevalence of the seizures. However, he became seizure-free after the addition of azathioprine and incremental increases of methylprednisolone dosage. This case clearly indicates the effectiveness of corticosteroid and azathioprine, as well as the futility of ASDs in the management of seizure control by showing the temporal trajectory relationship among ASDs, steroids, azathioprine and seizure frequency.</p>","PeriodicalId":72904,"journal":{"name":"Encephalitis (Seoul, Korea)","volume":"1 2","pages":"56-59"},"PeriodicalIF":0.0,"publicationDate":"2021-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/a3/4b/encephalitis-2020-00073.PMC10295884.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9845016","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Narcolepsy, autoimmunity, and influenza A H1N1 vaccination.","authors":"Jun-Sang Sunwoo","doi":"10.47936/encephalitis.2021.00010","DOIUrl":"https://doi.org/10.47936/encephalitis.2021.00010","url":null,"abstract":"<p><p>Narcolepsy is a rare neurological disorder characterized by excessive daytime sleepiness (EDS) with or without cataplexy. A main pathophysiology of narcolepsy is hypocretin deficiency in the central nervous system resulting from a selective loss of hypocretin neurons in the lateral hypothalamus. To date, the pathogenesis of hypocretin neuron loss in narcolepsy is the most commonly accepted autoimmune hypothesis which is supported by genetic risk factors for narcolepsy such as HLA‑DQB1*06:02 allele and T-cell receptor alpha polymorphisms. Other evidence supporting the immune-mediated mechanisms include the presence of anti-Tribbles homolog 2 (TRIB2) and anti-streptococcal antibodies in patients with narcolepsy, seasonal patterns of narcolepsy onset, and increased incidence of narcolepsy after the H1N1 pandemic influenza A infections and vaccinations. Among several types of vaccines, the AS03-adjuvanted vaccine Pandemrix (GlaxoSmithKline) was the only vaccine found to increase the risk of narcolepsy. However, the comprehensive results of several epidemiological studies indicate the adjuvant AS03 alone cannot cause the disease. The genetic predisposition, environmental triggers, molecular mimicry of specific H1N1 antigens, and bystander immune activation caused by the adjuvant AS03 may have combined to contribute to autoimmunity against hypocretin neurons and development of narcolepsy.</p>","PeriodicalId":72904,"journal":{"name":"Encephalitis (Seoul, Korea)","volume":"1 2","pages":"31-35"},"PeriodicalIF":0.0,"publicationDate":"2021-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/a7/8c/encephalitis-2021-00010.PMC10295885.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9835142","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Sungeun Hwang, Hyoshin Son, Jangsup Moon, Soon-Tae Lee, Keun Hwa Jung, Kyung-Il Park, Sang Kun Lee, Kon Chu
{"title":"Refractory neuro-Sweet disease successfully treated with tocilizumab and mycophenolate mofetil.","authors":"Sungeun Hwang, Hyoshin Son, Jangsup Moon, Soon-Tae Lee, Keun Hwa Jung, Kyung-Il Park, Sang Kun Lee, Kon Chu","doi":"10.47936/encephalitis.2020.00059","DOIUrl":"https://doi.org/10.47936/encephalitis.2020.00059","url":null,"abstract":"<p><p>Sweet syndrome, or acute febrile neutrophilic dermatosis, is mainly a dermatologic condition presenting with erythematous plaques; however, neutrophils infiltrate multiple systems. Neuro-Sweet disease is a neurological manifestation of Sweet syndrome and a rare cause of recurrent aseptic meningoencephalitis, which needs to be distinguished from neuro-Behçet disease. Although neuro-Sweet disease generally responds well to corticosteroids, relapsing neuro-Sweet disease is not an exceptional case. Herein, we present a case of a 51-year-old male with recurrent encephalitis followed by erythematous plaques. The patient was confirmed as Sweet syndrome based on skin biopsy and showed partial response to corticosteroids. With intravenous immunoglobulin, rituximab, tocilizumab, and mycophenolate mofetil, his neurologic symptoms were fully recovered.</p>","PeriodicalId":72904,"journal":{"name":"Encephalitis (Seoul, Korea)","volume":"1 1","pages":"20-24"},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/8a/3a/encephalitis-2020-00059.PMC10295875.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9930031","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Congratulatory remarks on expanding the research field for encephalitis and neuroinflammation.","authors":"Sang Kun Lee","doi":"10.47936/encephalitis.2020.00001","DOIUrl":"https://doi.org/10.47936/encephalitis.2020.00001","url":null,"abstract":"","PeriodicalId":72904,"journal":{"name":"Encephalitis (Seoul, Korea)","volume":"1 1","pages":"1"},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/53/64/encephalitis-2020-00001.PMC10295878.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10229146","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"With a new journal \"encephalitis,\" hope to feel inspired.","authors":"Kyung-Il Park","doi":"10.47936/encephalitis.2020.00003","DOIUrl":"https://doi.org/10.47936/encephalitis.2020.00003","url":null,"abstract":"","PeriodicalId":72904,"journal":{"name":"Encephalitis (Seoul, Korea)","volume":"1 1","pages":"3"},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/89/01/encephalitis-2020-00003.PMC10295874.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10229148","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}