Neuroimmunology Reports最新文献_第7页

Rapidly progressive cerebral dysfunction in a 5-year-old child: A reminder of subacute sclerosing panencephalitis (SSPE) and the importance of measles vaccination coverage 一名 5 岁儿童出现快速进展性脑功能障碍：提醒人们注意亚急性硬化性泛脑炎（SSPE）和麻疹疫苗接种的重要性

Neuroimmunology Reports Pub Date : 2023-12-12 DOI: 10.1016/j.nerep.2023.100195

Mustafa Bakir , Nadir Khan , Jonathan Gehlbach

{"title":"Rapidly progressive cerebral dysfunction in a 5-year-old child: A reminder of subacute sclerosing panencephalitis (SSPE) and the importance of measles vaccination coverage","authors":"Mustafa Bakir , Nadir Khan , Jonathan Gehlbach","doi":"10.1016/j.nerep.2023.100195","DOIUrl":"https://doi.org/10.1016/j.nerep.2023.100195","url":null,"abstract":"<div><p>We present a case of a preschool boy admitted to the pediatric intensive care unit with generalized weakness and encephalopathy that progressed to coma and spastic paralysis over the next few weeks. Extensive evaluation of a wide range of possible diagnoses, including infectious, post-infectious, autoimmune, and paraneoplastic disorders, proved unrevealing. Owing to concerns regarding autoimmune encephalitis, the patient received plasmapheresis, intravenous immunoglobulin, high-dose glucocorticoids, anakinra, rituximab, and empirical botulinum antitoxin. Despite these treatments, the patient's neurological condition continued to deteriorate, requiring endotracheal intubation. The patient developed repeated tremors and dystonic events, which progressed to hypertonia with gaze deviation. His-EEG in the third week of admission showed Radermecker complexes most consistent with SSPE. Because of his history of travel to Afghanistan at 8 months of age, before he was vaccinated for measles, and after a measles-like illness upon return, we checked the measles IgG titer in the CSF from the initial lumbar puncture and found it to be elevated. Global coverage with the first dose of the measles vaccine has dropped to 81 % by 2021, the lowest rate since 2008. This decline raises the concern of a possible increase in the incidence of measles and its fatal complications such as SSPE.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"5 ","pages":"Article 100195"},"PeriodicalIF":0.0,"publicationDate":"2023-12-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X23000335/pdfft?md5=ef8643cee9acbe6325a3886533c4de17&pid=1-s2.0-S2667257X23000335-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138769624","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}

引用次数: 0

Regaining walking and restoring corticospinal tract excitability after neuromyelitis optica spectrum disorder: A case report 视神经脊髓炎后恢复行走和皮质脊髓束兴奋性1例

Neuroimmunology Reports Pub Date : 2023-11-20 DOI: 10.1016/j.nerep.2023.100193

Hui-Ting Shih , Hui-Ting Goh , Seema Sikka , Rita Hamilton , Chad Swank

引用次数: 0

Decades-delayed onset of subcutaneous granulomata and calcifications from interferon-beta-1a in a patient with multiple sclerosis 多发性硬化症患者皮下肉芽肿和钙化的干扰素- β -1a延迟发作数十年

Neuroimmunology Reports Pub Date : 2023-11-17 DOI: 10.1016/j.nerep.2023.100190

Mara Bahri , Kristi Epstein , Yinan Zhang

引用次数: 0

A case of refractory tumefactive multiple sclerosis in an African American woman 非裔美国妇女难治性肿瘤性多发性硬化症1例

Neuroimmunology Reports Pub Date : 2023-11-15 DOI: 10.1016/j.nerep.2023.100192

Conor Kelly , Jennings Gyedu , Rock Heyman , Cigdem Isitan Alkawadri

{"title":"A case of refractory tumefactive multiple sclerosis in an African American woman","authors":"Conor Kelly , Jennings Gyedu , Rock Heyman , Cigdem Isitan Alkawadri","doi":"10.1016/j.nerep.2023.100192","DOIUrl":"https://doi.org/10.1016/j.nerep.2023.100192","url":null,"abstract":"<div><h3>Background</h3><p>Tumefactive multiple sclerosis (TMS) is a rare subtype of multiple sclerosis (MS) that poses a diagnostic and therapeutic challenge, with relatively little available published data. Though studies have demonstrated similar disease course in TMS with non-tumefactive disease, refractory cases requiring early escalation of therapy are noted and risk factors are unclear. Furthermore, no studies have presented data specifically on adult African American patients with TMS.</p></div><div><h3>Case report</h3><p>We present a case of TMS refractory to steroids and plasma exchange in an African-American woman. Disease progression was halted after treatment with cyclophosphamide. The patient was later transitioned to rituximab maintenance therapy.</p></div><div><h3>Conclusions</h3><p>This case contributes to the limited data on disease course and treatment response of this rare disease process in a population that is under-represented in the literature. We note the importance of further studies to identify risk factors for refractory disease requiring early initiation of high-efficacy disease modifying treatment (DMT).</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"4 ","pages":"Article 100192"},"PeriodicalIF":0.0,"publicationDate":"2023-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X2300030X/pdfft?md5=b1b38b50fa7f97267d8101a19bd57fd0&pid=1-s2.0-S2667257X2300030X-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136696887","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}

引用次数: 0

Approach to Anti-MuSK Myasthenia gravis: The ultimate mimicker 抗MuSK重症肌无力的方法：终极模仿者

Neuroimmunology Reports Pub Date : 2023-10-14 DOI: 10.1016/j.nerep.2023.100189

Abeer Sabry Safan , Nadir Kharma , Beatriz Canibaño , Mohammed Al-Hatou

{"title":"Approach to Anti-MuSK Myasthenia gravis: The ultimate mimicker","authors":"Abeer Sabry Safan , Nadir Kharma , Beatriz Canibaño , Mohammed Al-Hatou","doi":"10.1016/j.nerep.2023.100189","DOIUrl":"https://doi.org/10.1016/j.nerep.2023.100189","url":null,"abstract":"<div><h3>Background</h3><p>Anti-Muscle-specific tyrosine kinase – Myasthenia gravis (MuSK-MG) is a rare neuromuscular junction (NMJ) disease subtype with variable clinical presentation and often atypical electromyography findings. While amyotrophic lateral sclerosis (ALS) can present with respiratory failure, its median respiratory insufficiency onset is estimated at six months from the onset of diagnosis, with variability predicted by baseline functional vital capacity (FVC) and bulbar onset-ALS. Anti-MuSK-MG presentation with the predominately irritable myopathic diaphragm is rarely reported.</p></div><div><h3>Clinical presentation</h3><p>We report a case of Anti-MuSK-MG presenting with persistent respiratory insufficiency and bulbar dysfunction initially misdiagnosed as bulbar-type ALS due to bulbar findings and tongue atrophy. Electromyography (EMG) and single fiber EMG (SFEMG) defied former diagnosis (ALS) with findings of asymmetrical right ulnar and spinal accessory decrements on slow rate repetitive nerve stimulation (RNS), abnormal jitter on SFEMG, and irritable myopathy pattern of the diaphragm and proximal muscles. The serology marker is positive for Anti-MuSK Antibody, and negative AhCR anti-body. With supportive care and Rituximab, the patient's bulbar and respiratory function gradually improved.</p></div><div><h3>Conclusions</h3><p>Anti<strong>-</strong>MusK-MG presenting with persistent respiratory insufficiency has been reported with atypical electromyography findings of myopathy and denervation. We report an intriguing case of MuSK-MG with irritable diaphragm myopathy pattern presenting with myasthenic crisis mimicking bulbar subtype ALS.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"4 ","pages":"Article 100189"},"PeriodicalIF":0.0,"publicationDate":"2023-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"50193890","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}

引用次数: 0

Tumefactive demyelinating lesion due to neuromyelitis optica spectrum disorder: Successful relapse prevention with eculizumab 视神经脊髓炎谱系障碍引起的肿瘤性脱髓鞘病变：埃库珠单抗成功预防复发

Neuroimmunology Reports Pub Date : 2023-09-17 DOI: 10.1016/j.nerep.2023.100188

Kimberly A. O'Neill , Ukuemi Edema , Lauren Gluck

引用次数: 0

CADASIL progression after neurologic infectious insults: Case report of a new pathogenic NOTCH3 mutation 神经系统感染性损伤后CADASIL进展：一例新的致病性NOTCH3突变病例报告

Neuroimmunology Reports Pub Date : 2023-09-17 DOI: 10.1016/j.nerep.2023.100186

Kelli M. Money , Jamie Cronin , Amy Guimaraes-Young , Aaron Carlson , Mark A. Lovell , Elizabeth Matthews , Karen D. Orjuela , Daniel M. Pastula , Eric P. Wartchow , Amanda L. Piquet

{"title":"CADASIL progression after neurologic infectious insults: Case report of a new pathogenic NOTCH3 mutation","authors":"Kelli M. Money , Jamie Cronin , Amy Guimaraes-Young , Aaron Carlson , Mark A. Lovell , Elizabeth Matthews , Karen D. Orjuela , Daniel M. Pastula , Eric P. Wartchow , Amanda L. Piquet","doi":"10.1016/j.nerep.2023.100186","DOIUrl":"https://doi.org/10.1016/j.nerep.2023.100186","url":null,"abstract":"<div><h3>Background</h3><p>Cerebral autosomal dominant arteriopathy with subcortical infarcts (CADASIL) is the most common monogenic inherited small vessel disease. Autoimmune disorders, including multiple sclerosis, have been documented to co-exist with CADASIL, which can complicate the initial recognition. Inflammatory insults have been suggested to be associated with disease progression with SARS-CoV-2 infection.</p></div><div><h3>Case Report</h3><p>We present a case of a white woman in her 50 s who demonstrated progressive white matter changes with initial demyelinating characteristics in 2010. Their course was notable for accelerated progression after multiple central nervous system insults, including herpes simplex virus (HSV) encephalitis in 2017, suspected post-infectious autoimmune encephalitis in 2018, and neuroinvasive West Nile virus (WNV) infection with confirmed para-infectious NMDA receptor encephalitis in 2022. CADASIL was suspected given confluent anterior temporal and external capsule white matter changes. She was found to have a novel <em>NOTCH3</em> missense mutation in exon 3 (c.313T>C, p.(Ser105Pro)), and electron microscopy of a skin biopsy demonstrated granular osmiophilic material deposits diagnostic of CADASIL.</p></div><div><h3>Conclusion</h3><p>This case demonstrates a novel pathogenic <em>NOTCH3</em> mutation as well as the complexity of CADASIL diagnosis in the setting of possible concomitant demyelinating disease and other central nervous system insults. Significant phenotypic variability and overlapping acquired pathologies can make CADASIL recognition difficult. Given precipitous decline with each central nervous system insult in this case, we suspect these events hastened CADASIL progression.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"4 ","pages":"Article 100186"},"PeriodicalIF":0.0,"publicationDate":"2023-09-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"50193891","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}

引用次数: 0

Heart rate variability analysis of electrocardiography in pediatric immune-mediated autonomic neuropathy 儿童免疫介导的自主神经病变心电图的心率变异性分析

Neuroimmunology Reports Pub Date : 2023-09-17 DOI: 10.1016/j.nerep.2023.100187

Masafumi Sanefuji , Toshiyuki Maeda , Takashi Kumamoto , Yoshiyasu Ogata , Fumio Ichinose , Takuji Nakamura , Hiroto Doi , Shunya Nakane , Muneaki Matsuo

{"title":"Heart rate variability analysis of electrocardiography in pediatric immune-mediated autonomic neuropathy","authors":"Masafumi Sanefuji , Toshiyuki Maeda , Takashi Kumamoto , Yoshiyasu Ogata , Fumio Ichinose , Takuji Nakamura , Hiroto Doi , Shunya Nakane , Muneaki Matsuo","doi":"10.1016/j.nerep.2023.100187","DOIUrl":"https://doi.org/10.1016/j.nerep.2023.100187","url":null,"abstract":"<div><h3>Introduction</h3><p>Immune-mediated autonomic neuropathies are difficult to diagnose in childhood because of the extreme rarity, nonspecific symptoms, and infrequent detection of autoantibodies specific to the disorders. There is a need for objective testing methods that are easily applicable to pediatric patients.</p></div><div><h3>Description of case series</h3><p>Case 1 was a 13-year-old boy who showed autonomic symptoms of fatigue, tachycardia, and hypertension with sensorimotor impairments, including diminished tendon reflexes, delayed nerve conduction, and enhanced peripheral nerves on MRI. Case 2 was a 9-year-old boy who exhibited autonomic signs of postural orthostatic tachycardia with no abnormalities in a nerve conduction test and MRI but decreased heart rate variability (HRV) on conventional electrocardiography (ECG). Case 3 was a 14-year-old girl with autonomic symptoms of hypohidrosis, constipation, and coughing episodes, with alteration of various HRV metrics on 24-hour Holter ECG. Although serum autoantibodies to ganglionic acetylcholine receptor were not detected in any patients, intravenous immunoglobulin therapy was effective in all cases, suggesting immune-mediated mechanisms.</p></div><div><h3>Conclusion</h3><p>In the latter two patients, the diagnosis of immune-mediated autonomic neuropathies was underscored by HRV on ECG, especially 24-hour Holter ECG. Further studies are required to establish its clinical significance.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"4 ","pages":"Article 100187"},"PeriodicalIF":0.0,"publicationDate":"2023-09-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"50193889","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}

引用次数: 0

A case of stress cardiomyopathy (Takotsubo syndrome) associated with neuromyelitis optica spectrum disorder (NMOSD) 一例应激性心肌病（Takotsubo综合征）伴视神经脊髓炎谱系障碍（NMOSD）

Neuroimmunology Reports Pub Date : 2023-09-09 DOI: 10.1016/j.nerep.2023.100185

Rezafarhad Manteghifasaei, Mohammad Ali Arami

引用次数: 0

Successful treatment of refractory MOG-IgG-associated disease with ofatumumab: A first case report 奥法单抗成功治疗难治性MOG-IgG相关疾病：一例报告

Neuroimmunology Reports Pub Date : 2023-09-09 DOI: 10.1016/j.nerep.2023.100183

Hao Chen , Yu Zhou , Mengjie Zhang , WenJuan Gong , Yingxiong Xiong , Bo Wang , Yiyi Zhou , Jin Chen , Xiaomu Wu , Daojun Hong

{"title":"Successful treatment of refractory MOG-IgG-associated disease with ofatumumab: A first case report","authors":"Hao Chen , Yu Zhou , Mengjie Zhang , WenJuan Gong , Yingxiong Xiong , Bo Wang , Yiyi Zhou , Jin Chen , Xiaomu Wu , Daojun Hong","doi":"10.1016/j.nerep.2023.100183","DOIUrl":"https://doi.org/10.1016/j.nerep.2023.100183","url":null,"abstract":"<div><h3>Background</h3><p>Myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a recently identified and recurrent inflammatory demyelinating disease of the central nervous system (CNS) in both adults and children. Although MOGAD acute attacks usually appear to be very responsive to high dose steroids and plasma exchange, the long-term side effects of steroids should be highlighted especially in children. Attack-prevention treatments also lack of class-I evidence and prospective randomized placebo-controlled trials are needed to better guide prevention treatment in refractory cases. Long-term immunosuppressive maintenance with mycophenolate mofetil, azathioprine or rituximab may not always effective in patients experienced recurrent demyelinating attacks.</p></div><div><h3>Case presentation</h3><p>We reported a rare case of 17-year-old MOGAD patient who experienced eleven relapses with azathioprine, mycophenolate mofetil and rituximab in five years, and finally effectively treated with ofatumumab (OFA), a novel fully humanized anti-CD20 mAb.</p></div><div><h3>Conclusions</h3><p>Timely and efficient treatment is crucial for better prognosis in refractory MOGAD. The fully humanized OFA may be an optimal choice if multiple therapeutic drugs fail to achieve remission. Further research on comparing OFA and RTX in MOG patient is needed.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"4 ","pages":"Article 100183"},"PeriodicalIF":0.0,"publicationDate":"2023-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"50193927","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}

引用次数: 0