Kevser Erdogan, Nermin Tepe, Gülay Turan, Musa Öztürk
{"title":"Immunoglobulin G4-related disease in an elderly patient with treatment-resistant headache: a case report.","authors":"Kevser Erdogan, Nermin Tepe, Gülay Turan, Musa Öztürk","doi":"10.47936/encephalitis.2024.00073","DOIUrl":"https://doi.org/10.47936/encephalitis.2024.00073","url":null,"abstract":"<p><p>Immunoglobulin G4 (IgG4)-related disease is an immune-mediated, fibroinflammatory condition that causes multisystemic contrast enhancement and is predominantly observed in elderly male patients. The most prominent features of IgG4-related disease are systemic involvement affecting from two to six systems. In the central nervous system, these contrast enhancements are typically found in the meninges and orbit. This case study describes a 78-year-old female patient with persistent headaches and constant diarrhea who underwent treatment for IgG4-related disease. Despite two months of treatment with non-steroidal and opioid analgesics, the patient remained unresponsive and continued to experience diarrhea for 4 months. Brain magnetic resonance imaging revealed contrast enhancement in the leptomeningeal surfaces, and a biopsy of the gastrointestinal mucosa confirmed the diagnosis of IgG4-related disease, showing widespread plasma cell infiltration and IgG4 expression on plasma cells. The patient was initially treated with 1.0 g/day of pulse therapy for 5 days, followed by a maintenance dose of 1.0 mg/kg oral azathioprine. When azathioprine caused significant pancytopenia, rituximab therapy was initiated. The patient's headaches resolved completely, and the diarrheal attacks were controlled. This case highlights the importance of considering IgG4-related disease as a potential cause of headache or multiorgan symptoms in elderly patients with new-onset headache unresponsive to conventional analgesics. IgG4-related disease can often be effectively treated with steroids and monoclonal antibodies.</p>","PeriodicalId":72904,"journal":{"name":"Encephalitis (Seoul, Korea)","volume":"4 4","pages":"87-90"},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11472142/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142482282","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":"Stress as a possible trigger in anti-N-methyl-ᴅ-aspartate receptor encephalitis: a case series.","authors":"Sheikh Hilal Ahmad, Atif Kawoosa, Tanveer Hassan, Bashir Sanaie","doi":"10.47936/encephalitis.2024.00059","DOIUrl":"10.47936/encephalitis.2024.00059","url":null,"abstract":"<p><p>Anti-N-methyl-ᴅ-aspartate receptor (NMDAR) encephalitis is a rare disease with a range of neurological and psychiatric presentations. Antibodies against NMDAR receptor are purported to be pathologic, and the two known potential immunological triggers are tumors and viral infection. In half of the cases, the trigger is not known. We present two cases where stress seemed to have triggered encephalitis. These cases illustrate the possible role of stress in leading to immune dysregulation, which can lead to encephalitis. We review the role of stress in anti-NMDAR encephalitis and possible mechanisms by which stress can trigger an immune dysregulation.</p>","PeriodicalId":72904,"journal":{"name":"Encephalitis (Seoul, Korea)","volume":" ","pages":"76-82"},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11472143/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142302464","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":"Movement disorders in dengue encephalitis: a case report and literature review.","authors":"Pranjali Batra, Neetu Rani Dhiman, Ibrahim Hussain, Anand Kumar, Deepika Joshi","doi":"10.47936/encephalitis.2024.00066","DOIUrl":"10.47936/encephalitis.2024.00066","url":null,"abstract":"<p><p>Dengue is a mosquito-borne viral disease caused by the dengue virus (DENV). The clinical manifestations of DENV infection range from mild febrile illness to severe dengue shock syndrome and dengue hemorrhagic fever. Recently, its neurological manifestations have been reported. The mechanisms of neurological complications in DENV infection are often attributed to neurotropism or may be immune-mediated. A double-doughnut sign is a radiological pattern of signal changes in the bilateral thalami, resembling a doughnut. Although this sign has been reported with dengue encephalitis, Japanese encephalitis, and other neurotropic infections, its co-occurrence with mixed movement disorders is rare. We report a case of dengue encephalitis involving a spectrum of movement disorders in the form of jaw opening dystonia, stereotypies, parkinsonism, and tremors during recovery. Magnetic resonance imaging showed bilateral thalami involvement with a double-doughnut sign. The patient was managed with pulse steroid therapy and benzodiazepines and showed gradual improvement in symptoms. Movement disorders with DENV infection are rare and self-limiting.</p>","PeriodicalId":72904,"journal":{"name":"Encephalitis (Seoul, Korea)","volume":" ","pages":"83-86"},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11472140/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142382616","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":"The relationship between sleep and innate immunity.","authors":"Yoonkyung Lee, Kyung-Il Park","doi":"10.47936/encephalitis.2024.00017","DOIUrl":"10.47936/encephalitis.2024.00017","url":null,"abstract":"<p><p>Sleep regulates inflammatory responses, and the innate immune system affects sleep. Interleukin-1 beta, tumor necrosis factor alpha, growth hormone-releasing hormone, prolactin, and nitric oxide are somnogenic substances. Sleep deprivation, such as chronic insomnia or obstructive sleep apnea, affects cytokine production, glial function, natural killer cell activity, and inflammasome function. This review will discuss the relationship between sleep and innate immunity.</p>","PeriodicalId":72904,"journal":{"name":"Encephalitis (Seoul, Korea)","volume":" ","pages":"69-75"},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11472141/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141072454","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":"Understanding epileptogenesis from molecules to network alteration.","authors":"Kyung-Il Park","doi":"10.47936/encephalitis.2024.00038","DOIUrl":"10.47936/encephalitis.2024.00038","url":null,"abstract":"<p><p>Epilepsy is characterized by recurrent seizures. Following an initial insult, a latent period precedes the onset of spontaneous seizures, a process referred to as epileptogenesis. This period plays a critical role in halting the progression toward epilepsy before the onset of abnormal molecular and network alterations. In this study, the fundamental concepts of epileptogenesis as well as the associated molecular and cellular targets are reviewed.</p>","PeriodicalId":72904,"journal":{"name":"Encephalitis (Seoul, Korea)","volume":" ","pages":"47-54"},"PeriodicalIF":0.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11237188/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141422084","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":"Sleep and neuroimmunology: a narrative review.","authors":"Jin Myoung Seok, Kwang Ik Yang","doi":"10.47936/encephalitis.2024.00024","DOIUrl":"10.47936/encephalitis.2024.00024","url":null,"abstract":"<p><p>Numerous neuroimmunological disorders present with sleep-related symptoms. The identification of novel autoantibodies introduces new clinical categories in autoimmune diseases of the central nervous system and generates interest in the dynamic interaction between sleep and the immune system. In this review, the complex relationship among sleep, immune regulation, and neuroimmunological disorders was examined with emphasis on the vital role of sleep in modulating immune function and its influence on these conditions, This relationship emphasizes the importance of assessments and management of sleep quality in the treatment approaches for neuroimmunological disorders.</p>","PeriodicalId":72904,"journal":{"name":"Encephalitis (Seoul, Korea)","volume":" ","pages":"55-61"},"PeriodicalIF":0.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11237187/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141447763","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}
Hussein Algahtani, Bader Shirah, Hussam A Jamaluddin, Nawal Abdelghaffar
{"title":"Insights into adjunctive corticosteroid therapy in fulminant herpes simplex virus encephalitis: case analysis with contrasting outcomes.","authors":"Hussein Algahtani, Bader Shirah, Hussam A Jamaluddin, Nawal Abdelghaffar","doi":"10.47936/encephalitis.2024.00031","DOIUrl":"10.47936/encephalitis.2024.00031","url":null,"abstract":"<p><p>Herpes simplex virus (HSV) encephalitis, predominantly caused by HSV-1, presents with significant morbidity and mortality challenges. This research investigates the particular role of adjunctive corticosteroid therapy in fulminant HSV encephalitis through in-depth analyses of two contrasting cases. Corticosteroids show potential benefits to improve an exaggerated immune response and limit viral dissemination within the brain. Daily assessments and frequent neuroimaging, particularly using magnetic resonance imaging, aid in the management of fulminant cases. Although existing evidence relies on limited case series and retrospective comparisons, the results of the present study emphasize the necessity for large-scale controlled trials to establish definitive guidelines. The discretion of the treating neurologist governs the decision to implement corticosteroids, emphasizing the imperative need for continued research and evidence-based strategies for this challenging neurological condition.</p>","PeriodicalId":72904,"journal":{"name":"Encephalitis (Seoul, Korea)","volume":"4 3","pages":"62-68"},"PeriodicalIF":0.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11237186/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141565311","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":"Chronic social stress in early life can predispose mice to antisocial maltreating behavior.","authors":"Daejong Jeon, Sangwoo Kim, Sang Kun Lee, Kon Chu","doi":"10.47936/encephalitis.2023.00199","DOIUrl":"10.47936/encephalitis.2023.00199","url":null,"abstract":"<p><strong>Purpose: </strong>In our previous study, we developed an assay system to evaluate antisocial maltreating behavior of conspecific mice using a perpetrator-victim paradigm. We also generated a mouse model for the maltreating behavior by mimicking child maltreatment or abuse. Here, we further investigate the antisocial behavior using anti-aggressive and antipsychotic drugs.</p><p><strong>Methods: </strong>Model mice sequentially subjected to maternal separation (MS), social defeat (SD), and social isolation (SI) in that order (MS/SD/SI model) were subjected to a maltreating behavioral task. The MS/SD/SI mice were treated with oxytocin (OXY), clozapine (CLZ), haloperidol (HAL), and 8-hydroxy-2-(di-n-propylamino)tetralin (8-OH-DPAT). Western blotting and enzyme-linked immunosorbent assay were used for protein analysis.</p><p><strong>Results: </strong>A substantial portion of the MS/SD/SI model mice (46% of males and 40% of females) showed a higher number of nose pokes than the control. OXY or 8-OH-DPAT treatment reduced the high number of nose pokes by the MS/SD/SI mice, whereas HAL increased it. CLZ did not affect the number of nose pokes by the MS/SD/SI mice. Interestingly, although the OXY level in the MS/SD/SI mice was similar to that in the control, the amount of OXY receptor was lower in the MS/SD/SI mice. The amount of 5-HT1A receptor was also decreased in the MS/SD/SI mice.</p><p><strong>Conclusion: </strong>Chronic social stress in childhood might predispose a mouse to antisocial behavior. Our maltreating behavior assay system, including the MS/SD/SI model, is a good animal system for research on and drug screening for brain disorders associated with antisocial or psychotic behavior.</p>","PeriodicalId":72904,"journal":{"name":"Encephalitis (Seoul, Korea)","volume":" ","pages":"23-30"},"PeriodicalIF":0.0,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11007547/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140041014","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}
Soo Hyun Ahn, Seon-Jae Ahn, Seung Ae Kim, Han Sang Lee, Kon Chu
{"title":"Eosinophilic meningoencephalitis successfully treated with glucocorticoids and intravenous immunoglobulin: a case report.","authors":"Soo Hyun Ahn, Seon-Jae Ahn, Seung Ae Kim, Han Sang Lee, Kon Chu","doi":"10.47936/encephalitis.2024.00010","DOIUrl":"10.47936/encephalitis.2024.00010","url":null,"abstract":"<p><p>Eosinophilic meningoencephalitis is a rare inflammatory condition of the central nervous system. As a limited number of cases has been reported, debate remains on the optimal treatment. We present a case of idiopathic eosinophilic meningoencephalitis successfully treated with glucocorticoids and intravenous immunoglobulin (IVIG). After extensive evaluation to rule out other possible causes, the patient was treated with intravenous (IV) dexamethasone and showed significant improvement within a few days. However, neurologic impairment persisted, and follow-up lumbar puncture results showed only a mild decrease in pleocytosis. Even after an additional 5 days of IV methylprednisolone, cerebrospinal fluid (CSF) pleocytosis persisted, and brain magnetic resonance imaging (MRI) showed an increase in enhanced lesions, implying persistent neuroinflammation. The patient was maintained on high-dose oral prednisolone for 2 months, and additional immune-modulatory effects were treated with IVIG. Follow-up MRI at 2 months showed a significant decrease in the extent of multiple enhanced lesions and a normalized CSF profile. The patient was maintained on regular maintenance doses of IVIG for an additional 6 months without any neurologic signs or symptoms. Inflammation is the key pathophysiology underlying neurological damage in eosinophilic meningoencephalitis. A literature review revealed that corticosteroid treatment is the only anti-inflammatory treatment used in cases of idiopathic meningoencephalitis, resulting in sufficient response in most patients but only partial response or death in a few cases. This is the first case report of IVIG use in idiopathic eosinophilic meningoencephalitis, suggesting the possibility of a new treatment modality for refractory cases.</p>","PeriodicalId":72904,"journal":{"name":"Encephalitis (Seoul, Korea)","volume":" ","pages":"40-46"},"PeriodicalIF":0.0,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11007548/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140308164","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}
Yoonjeong Na, Jung-Ju Lee, Byung Kun Kim, Woong-Woo Lee, Yong Soo Kim, Ilhan Yoo
{"title":"Herpes simplex encephalitis initially presenting without fever or cerebrospinal fluid pleocytosis and with typical neuroimaging findings: a case report.","authors":"Yoonjeong Na, Jung-Ju Lee, Byung Kun Kim, Woong-Woo Lee, Yong Soo Kim, Ilhan Yoo","doi":"10.47936/encephalitis.2023.00220","DOIUrl":"10.47936/encephalitis.2023.00220","url":null,"abstract":"<p><p>Herpes simplex encephalitis (HSE) is a common viral encephalitis that can be fatal if not adequately treated. Fever, cerebrospinal fluid (CSF) pleocytosis, and typical neuroimaging findings are commonly observed in HSE cases. We encountered a patient with HSE who did not exhibit these classic clinical features. A 63-year-old male presented with his first-ever seizure. Fever did not develop until the fourth day of admission, and neither neuroimaging nor CSF analysis revealed abnormalities. Under suspicion of autoimmune encephalitis, methylprednisolone was administered. Subsequently, when the patient developed fever, a follow-up neuroimaging study was performed and revealed abnormalities consistent with HSE. The patient was promptly treated with acyclovir, which led to a full recovery. Diagnosing HSE in patients who present without fever or CSF pleocytosis and with typical neuroimaging findings poses a challenge. Therefore, prior to initiating immunosuppressive treatment, it is crucial to closely observe patients and to conduct follow-up tests, including neuroimaging and CSF analysis.</p>","PeriodicalId":72904,"journal":{"name":"Encephalitis (Seoul, Korea)","volume":" ","pages":"31-34"},"PeriodicalIF":0.0,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11007550/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140041015","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}