Clinical and Experimental Neuroimmunology最新文献

{"title":"Safety and effectiveness of eculizumab in Japanese patients with generalized myasthenia gravis: Analysis of 1-year postmarketing surveillance","authors":"Hiroyuki Murai,&nbsp;Shigeaki Suzuki,&nbsp;Yuji Fukamizu,&nbsp;Takehiko Osawa,&nbsp;Hidekazu Kikui,&nbsp;Kimiaki Utsugisawa","doi":"10.1111/cen3.12716","DOIUrl":"10.1111/cen3.12716","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>Eculizumab, a terminal complement protein C5 inhibitor, is approved in Japan for the treatment of patients with anti-acetylcholine receptor antibody-positive (AChR Ab+) generalized myasthenia gravis (gMG) that is difficult to control with plasmapheresis or high-dose intravenous immunoglobulin therapy.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This analysis of mandatory postmarketing surveillance in Japan assessed the safety and effectiveness of eculizumab in patients with AChR Ab+ gMG who had completed case-report forms at 26 wk after eculizumab initiation up to the cutoff date of April 2021. Changes from baseline were assessed for Myasthenia Gravis-Activities of Daily Living (MG-ADL) and Quantitative Myasthenia Gravis (QMG) total scores overall, and MG-ADL scores in patient subgroups according to sex, age at diagnosis and baseline, and baseline disease severity. The change in concomitant corticosteroid use was also evaluated.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Data were available for 134 adults (67.2% female; mean age 51.9 y); the effectiveness-analysis set comprised 126 patients. After 26 wk, 78% of patients were continuing eculizumab treatment. Adverse drug reactions were reported by 49 patients (37%) (most frequently headache [n = 10]). Improvements in MG-ADL scores were seen regardless of sex; age at diagnosis (&lt;50/≥50 y); baseline age (18 to &lt;40/≥40 to &lt;65/≥65 y); Myasthenia Gravis Foundation of America disease classification (IIa/IIb/IIIa/IIIb/IVa/IVb/V); or baseline MG-ADL score (&lt;6/≥6). Of patients receiving corticosteroids, the proportion receiving low doses (average ≤5 mg/d) increased from 7.0% before eculizumab initiation to 26.4% by Week 52.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Eculizumab was well tolerated and effective in treating AChR Ab+ gMG across a broad spectrum of adult Japanese patients with difficult-to-control gMG.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-06-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/cen3.12716","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46662986","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":"Anti-Zic4 encephalitis in endometrial cancer: Autoimmunity triggered by PD-1 inhibitor or recent CMV encephalitis?","authors":"Eugenia Filippakopoulou,&nbsp;Persefoni Karachalia,&nbsp;Maria Gryllia,&nbsp;Stella Marousi","doi":"10.1111/cen3.12715","DOIUrl":"10.1111/cen3.12715","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Autoimmune encephalitis has been well described in oncological patients treated with immune checkpoint inhibitors (ICIs), as a paraneoplastic neurological syndrome. Central nervous system (CNS) infections from herpes viruses are also known to trigger secondary neurologic autoimmunity.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>We hereby describe a patient with a recent history of cytomegalovirus (CMV) CNS infection and ongoing immunotherapy with the programmed-cell-death-protein-1(PD-1) inhibitor pembrolizumab for metastatic endometrial cancer. The patient presented with progressive encephalopathy along with cerebellar signs, rapidly evolving to status epilepticus requiring mechanical ventilation and advanced care unit (ACU) support. A serum paraneoplastic antibody panel revealed strongly positive anti-Zic4 antibodies. Despite first-line immunotherapy and aggressive anticonvulsant treatment, the patient died a few days postintubation.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>In anti-Zic4 encephalitis, both PD-1 inhibitor treatment and recent CMV viral insult may contribute to the development of neurological autoimmunity. Further research is needed to identify ways of predicting neurological damage in oncological patients treated with ICIs.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-05-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48160748","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":"Changes in the amplitude decremental response to repetitive nerve stimulation following fast-acting treatment in patients with myasthenia gravis during hospitalization","authors":"Hiroyuki Naito,&nbsp;Takamichi Sugimoto,&nbsp;Katsumi Kurokawa,&nbsp;Kazuki Kimoto,&nbsp;Takafumi Abe,&nbsp;Chika Matsuoka,&nbsp;Narumi Ohno,&nbsp;Mayumi Giga,&nbsp;Tomoyuki Kono,&nbsp;Hiroki Ueno,&nbsp;Eiichi Nomura","doi":"10.1111/cen3.12714","DOIUrl":"10.1111/cen3.12714","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objectives</h3>\u0000 \u0000 <p>The aim was to determine the amplitude changes in repetitive nerve stimulation (RNS) decrement values after fast-acting treatments (FTs), including plasmapheresis, high-dose intravenous methylprednisolone and intravenous immunoglobulin, in patients with myasthenia gravis (MG).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We retrospectively enrolled 41 patients with MG who received RNS after FT. The median, ulnar, facial, accessory and axillary nerves were tested. An improvement in the quantitative MG score of ≥3 or in the MG activities of daily living scale score of ≥2 was used to define clinical improvement posttreatment. A decrement of ≥10% was defined as abnormal.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Among 41 enrolled patients (ocular type 13 and generalized type 28), 22 (53.7%) showed clinical improvement after treatment. Among generalized MG patients, the proportion of improvement of abnormal RNS decrement in the proximal muscles, including frontalis, trapezius and deltoid, was significantly greater in the group with clinical improvement than in the group without clinical improvement (46.7% <i>vs</i> 0%, <i>P</i> = 0.005). Logistic regression analysis showed that the quantitative myasthenia gravis score at admission and improvement in abnormal amplitude decrement were associated with a response to FT (<i>P</i> = 0.015 and <i>P</i> = 0.045, respectively).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>The changes in amplitude decrements detected by RNS reflected the therapeutic effect of FT in patients with generalized MG. RNS responses in the proximal muscles might be potentially useful as an objective measure of improvement after aggressive immunotherapy in patients with MG during hospitalization.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-05-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44376873","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":"Intravenous immunoglobulin in the management and outcome of Stiff-Person syndrome: A systematic review","authors":"Roshan Aryal,&nbsp;Suraj Shrestha,&nbsp;Sushan Homagain,&nbsp;Sansar Babu Tiwari,&nbsp;Rajeev Ojha","doi":"10.1111/cen3.12713","DOIUrl":"10.1111/cen3.12713","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Stiff Person Syndrome (SPS), a rare immune-mediated neuromuscular disorder, is characterized by rigidity, stiffness, and intermittent spasms of axial and extremity muscles. Various immunotherapies including intravenous immunoglobulin (IVIG) have been used for this autoimmune condition. Here we aim to review the role of IVIG in the treatment of SPS along with its outcome.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>A systematic literature search of PubMed and Embase was conducted to identify the relevant published articles against the predefined criteria using suitable keywords combinations till September 20, 2021. Data were extracted to produce descriptive information of SPS patients on demographics, diagnostics, treatment with IVIG, and outcome.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Twelve studies with 216 patients were included in the review and 63.89% of them had classical SPS. Glutamic acid decarboxylase (GAD) autoantibodies were present in 72.68% of the patients and 57.89% in whom electromyography (EMG) was performed had continuous motor activity. IVIG therapy was given to 95 patients in different regimens in various studies and varying scoring systems were used to assess the outcome, and 83.16% showed some form of improvement, 14.74% showed no improvement, while 2.10% worsened. None of the included studies mentioned an adverse effect of IVIG in the patients.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>IVIG may benefit patients with SPS along with other medications. Owing to the rarity of the disease and insufficient studies on the assessment of immunotherapy in SPS, longitudinal studies with a sizable number of patients are required to clarify clinical course, treatment, and outcome in SPS with the use of IVIG.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-05-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46409865","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":"MOG antibody-related disorder associated with peripheral facial nerve palsy","authors":"Yuto Morishima,&nbsp;Akane Satake,&nbsp;Takanori Hata,&nbsp;Kishin Koh,&nbsp;Takamura Nagasaka,&nbsp;Toshiyuki Takahashi,&nbsp;Kazumasa Shindo,&nbsp;Yoshihisa Takiyama","doi":"10.1111/cen3.12708","DOIUrl":"10.1111/cen3.12708","url":null,"abstract":"<p>A 44-year-old Japanese woman exhibited neurological symptoms including unsteadiness of gait, headache, hypoesthesia of the left face, and left-sided peripheral facial nerve palsy. Brain magnetic resonance imaging (MRI) revealed abnormal intensities in the hemispheres, brainstem, and corpus callosum. Although routine blood and cerebrospinal fluid studies were unremarkable, serum anti-myelin oligodendrocyte glycoprotein (MOG) antibody was positive. With two-time intravenous methylprednisolone pulse therapy, her symptoms gradually improved. We report here a rare case of a MOG antibody-related disorder with peripheral facial nerve palsy as a predominant symptom. We should be aware of this disease even in a case of peripheral facial nerve palsy.</p>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-05-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45909624","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":"Thalamic atrophy, duration of illness, and years of education are the best predictors of cognitive impairment in multiple sclerosis","authors":"Eman M. Khedr,&nbsp;Tarek Desoky,&nbsp;Ayman Gamea,&nbsp;Mohammed Y. Ezzeldin,&nbsp;Ahmed F. Zaki","doi":"10.1111/cen3.12705","DOIUrl":"10.1111/cen3.12705","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Cognitive impairment (CI) is common in multiple sclerosis. We assessed the relationship between CI and demographic, motor disability, and volumetric brain atrophy in relapsing remitting multiple sclerosis (RRMS).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>In all, 43 RRMS patients and 40 age- sex-, and education-matched normal volunteers were enrolled. Assessments included: demographic, Expanded Disability Status Scale (EDSS); Brief International Cognitive Assessment for Multiple Sclerosis (BICAMS); and brain volumetric assessment.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>The mean age of onset of RRMS patients was 25.72 y and the mean number of attacks was 3.30 ± 2.33/year. The total brain volume, cerebral, and cerebellar hemispheres, brainstem as well as thalamic and hippocampal gray matter were significantly reduced in RRMS patients compared with controls.</p>\u0000 \u0000 <p>CI was detected in 41.9% of patients. Patients with CI had fewer years of education (<i>P</i> = .001), a higher number of attacks (<i>P</i> = .002), worse EDSS scores (<i>P</i> = .030); reduced gray matter volume, particularly of the thalamus, and hippocampus compared with patients without CI. There were significant negative correlations between CI with duration of illness, number of attacks, time elapsed between onset and diagnosis, and EDSS. Thalamic volume showed strong positive correlations with CI (<i>P</i> &lt; .0001). The best predictors of CI were thalamic atrophy, duration of illness, and years of education (<i>P</i> = .001, .010, and .035 respectively).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>We confirmed the presence of gray matter atrophy of the brain in RRMS patients. Volumetric magnetic resonance imaging measures of thalamus, duration of illness, and years of education could be useful biomarkers of CI in RRMS.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-05-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42855693","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":"Double trouble: A case of spinal muscular atrophy type III found to be complicated by myasthenia gravis due to subacute dysphagia","authors":"Ruoyi Ishikawa,&nbsp;Takamichi Sugimoto,&nbsp;Takafumi Abe,&nbsp;Narumi Ohno,&nbsp;Mayumi Giga,&nbsp;Hiroyuki Naito,&nbsp;Tomoyuki Kono,&nbsp;Eiichi Nomura,&nbsp;Takemori Yamawaki","doi":"10.1111/cen3.12707","DOIUrl":"10.1111/cen3.12707","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Spinal muscular atrophy (SMA) is a genetic disorder caused by the progressive loss of lower motor neurons. Myasthenia gravis (MG) is an acquired, autoimmune, neuromuscular junction disorder. We encountered a rare case of coexisting SMA and MG.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>A man was diagnosed with SMA type III when aged in his 20s. At the age 71 years, he started experiencing difficulty flexing his elbow, with dysphagia appearing within a few months. He had difficulty eating and was referred to our hospital to undergo gastrostomy. Other diseases that could cause dysphagia were investigated. Tests for acetylcholine receptor antibody and edrophonium showed positive results, based on which he was diagnosed with MG. A genetic test was carried out that reconfirmed SMA. He was treated with intravenous methylprednisolone, pyridostigmine and tacrolimus, resulting in dysphagia gradually recovering and he could consume a full meal. The patient was discharged without undergoing gastrostomy.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>SMA type III is a disease manifesting as slowly progressive muscle weakness. When the muscle weakness progresses and dysphagia appears subacutely in SMA type III patients, it is necessary to consider the complications of other diseases, such as MG.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-05-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49619178","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":"Association of the Processing Speed Test with depression and brain magnetic resonance imaging metrics in Japanese patients with multiple sclerosis","authors":"Yusei Miyazaki,&nbsp;Masaaki Niino,&nbsp;Eri Takahashi,&nbsp;Kei Oiwa,&nbsp;Ryoji Naganuma,&nbsp;Itaru Amino,&nbsp;Sachiko Akimoto,&nbsp;Naoya Minami,&nbsp;Seiji Kikuchi","doi":"10.1111/cen3.12706","DOIUrl":"10.1111/cen3.12706","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objectives</h3>\u0000 \u0000 <p>Cognitive dysfunction occurs in a substantial proportion of patients with multiple sclerosis (MS). The Processing Speed Test (PST) is a self-administered, tablet-based cognitive screening tool that closely resembles the Symbol Digit Modalities Test and can be quickly administered. In this study we assessed the performance of the PST and its correlation with the clinical and radiological aspects of MS in Japanese patients.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>PST was administered to 52 Japanese patients with MS. The Beck Depression Inventory Second Edition (BDI-II), Fatigue Severity Scale (FSS), and Functional Assessment of MS (FAMS) were used to evaluate depression, fatigue, and health-related quality of life, respectively. Global and regional brain volumes and cortical thickness were analyzed based on 3D T1-weighted magnetic resonance images.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>The mean ± standard deviation of the PST score was 52.5 ± 16.5. Negative correlations were observed between PST score and age, disease duration, and the Expanded Disability Status Scale (EDSS). The PST score showed significant correlation with BDI-II after adjusting for disease duration and EDSS. In contrast, no significant association was observed between PST score and FSS, FAMS total score, and employment status after adjusting for disease duration, EDSS, and BDI-II. The PST score showed significant correlation with global and regional brain volumes, and the thickness of bilateral temporal cortexes.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>The PST score was associated with the severity of depression and both global and regional brain volume in Japanese patients with MS. Our findings support the validity of the PST as a useful tool for assessing the cognitive function of MS patients in clinical practice.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-05-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47343516","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":"Unusual phenotype of acute motor sensory axonal neuropathy with ophthalmoplegia, and anti-GD1a, -GD1b and -GM1 antibodies","authors":"Theodora Afrantou,&nbsp;Vasilios Kimiskidis,&nbsp;Roza Lagoudaki,&nbsp;Thomai Stardeli,&nbsp;Vasileios Papaliagkas,&nbsp;Georgia Zafeiridou,&nbsp;Giannoula Thoma,&nbsp;Zoi Aidoni,&nbsp;Nikolaos Grigoriadis","doi":"10.1111/cen3.12704","DOIUrl":"10.1111/cen3.12704","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Acute motor sensory axonal neuropathy (AMSAN) is a severe form of Guillain–Barré syndrome, which typically is not related to ocular palsy, and is pathologically associated with antibodies against GM1 and GD1a gangliosides.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case presentation</h3>\u0000 \u0000 <p>We present a patient with a severe, drug-resistant form of AMSAN with ophthalmoplegia related to antiganglioside (anti-GD1a, -GD1b, -GM1) antibodies in the serum and cerebrospinal fluid, and with a poor long-term outcome.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Rare cases of AMSAN with ophthalmoplegia are described in the literature. The present patient is a case of an unusual clinical phenotype of AMSAN, the characteristics of which warn of a serious clinical course that requires timely aggressive treatment.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43201510","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":"Inflammatory reflex disruption in COVID-19","authors":"Khalil Hajiasgharzadeh,&nbsp;Mahdi Jafarlou,&nbsp;Behzad Mansoori,&nbsp;Narges Dastmalchi,&nbsp;Behzad Baradaran,&nbsp;Alireza Khabbazi","doi":"10.1111/cen3.12703","DOIUrl":"10.1111/cen3.12703","url":null,"abstract":"<p>Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) was identified in Wuhan, China, in late 2019 and caused coronavirus disease 2019 (COVID-19), which is still a global pandemic. In most infected people, SARS-CoV-2 only causes moderate symptoms, whereas in other patients, it leads to severe illness and eventually death. Although the main clinical manifestation of COVID-19 is often seen in the lungs, this disease affects almost all body organs. The excessive and prolonged release of inflammatory cytokines that might occur in COVID-19 patients, known as cytokine storms, stimulates undesired immune responses and can cause various tissues damage. In the current review article, we focus on the potential advantages of the intrinsic cholinergic anti-inflammatory pathway as the efferent arm of the inflammatory reflex in COVID-19 management. Considering this endogenous protective mechanism against chronic inflammation, we focused on the effects of SARS-CoV-2 in the destruction of this anti-inflammatory system. Several studies showed the interaction of SARS-CoV-2 with the alpha-7 subtype of the nicotinic acetylcholine receptor as the effector molecule of the inflammatory reflex. In contrast, neurological manifestations have increasingly been identified as significant extrapulmonary manifestations of COVID-19. The rational connection between these findings and COVID-19 pathogenesis might be an important issue in both our understanding of and dealing with this disease. COVID-19 is deeply rooted in our daily life and requires an urgent need for the establishment of effective therapeutic options, and all the possible treatments must be considered for the control of such inflammatory conditions.</p>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-04-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/cen3.12703","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77636090","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}