Neuromyelitis optica spectrum disorder (NMOSD) can cause severe disability after a single attack. This study aimed to investigate the reduction of oral medications, including steroids, in patients using biologics within 1 year after the onset or relapse of NMOSD.
Methods
Patients with NMOSD who were using biologics and attending the Department of Neurology at St. Marianna University School of Medicine from November 2019 to February 2023 were enrolled. The patients were introduced to biologics within 1 year of their last relapses. The study examined whether these patients experienced clinical relapse, changes in prednisolone dosage of 5 mg/day or less and changes in the number of oral medications.
Results
A total of 14 patients were included. The mean prednisolone dosage was 17.5 ± 5.8 mg/day, and the mean number of medications was 6.2 ± 4.2. No relapse was reported in any patient after the introduction of biologics. The prednisolone dosage gradually decreased to 5.3 ± 2.9 mg/day and 3.1 ± 1.7 mg/day at 6 and 12 months, respectively, and the rates of achieving a prednisolone dosage of ≤5 mg/day at 6 and 12 months were 61.5% and 100%, respectively. The total number of medications decreased to 4.4 ± 4.6.
Conclusions
Biologics can be introduced early after NMOSD relapse to reduce prednisolone dosage and counteract polypharmacy. The high relapse prevention rate of biologics makes them a valuable option for preventing relapse in patients with NMOSD.
{"title":"Impact of early introduction of biologics on internal medications for neuromyelitis optica spectrum disorder","authors":"Kenzo Sakurai, Kenji Isahaya, Takeshi Imai, Yoshihisa Yamano","doi":"10.1111/cen3.12760","DOIUrl":"10.1111/cen3.12760","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objectives</h3>\u0000 \u0000 <p>Neuromyelitis optica spectrum disorder (NMOSD) can cause severe disability after a single attack. This study aimed to investigate the reduction of oral medications, including steroids, in patients using biologics within 1 year after the onset or relapse of NMOSD.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Patients with NMOSD who were using biologics and attending the Department of Neurology at St. Marianna University School of Medicine from November 2019 to February 2023 were enrolled. The patients were introduced to biologics within 1 year of their last relapses. The study examined whether these patients experienced clinical relapse, changes in prednisolone dosage of 5 mg/day or less and changes in the number of oral medications.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>A total of 14 patients were included. The mean prednisolone dosage was 17.5 ± 5.8 mg/day, and the mean number of medications was 6.2 ± 4.2. No relapse was reported in any patient after the introduction of biologics. The prednisolone dosage gradually decreased to 5.3 ± 2.9 mg/day and 3.1 ± 1.7 mg/day at 6 and 12 months, respectively, and the rates of achieving a prednisolone dosage of ≤5 mg/day at 6 and 12 months were 61.5% and 100%, respectively. The total number of medications decreased to 4.4 ± 4.6.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Biologics can be introduced early after NMOSD relapse to reduce prednisolone dosage and counteract polypharmacy. The high relapse prevention rate of biologics makes them a valuable option for preventing relapse in patients with NMOSD.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-07-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46636394","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}
Mario B. Prado Jr, Maria Franchesca Quinio, Karen Joy B. Adiao
Background
To date, there is no journal that reports the coexistence of thymoma-associated myasthenia gravis, alopecia areata, and twenty-nail dystrophy. This paper hypothesizes that the presence of thymoma increases the risk of these conditions.
Case presentation
The patient was a 37-year-old Filipino who came in for a year of history of fluctuating, but progressive, left-sided ptosis and diplopia; difficulty swallowing; dysarthria; and aspiration and symmetric proximal muscle weakness confirmed to be myasthenia gravis by abnormal repetitive nerve stimulation and the presence of thymoma on histology. Before his thymectomy, he also developed gradually worsening thinning and longitudinal ridging of all fingernails and toenails; and patches of bald areas on the scalp; diagnosed to be twenty-nail dystrophy and alopecia areata, respectively, by a board-certified dermatologist.
Conclusion
Thymoma is the possible risk factor and link for the co-occurrence of the three conditions in the present patient.
{"title":"Twenty-nail dystrophy and alopecia areata in an adult male with thymoma-associated myasthenia gravis: A case report","authors":"Mario B. Prado Jr, Maria Franchesca Quinio, Karen Joy B. Adiao","doi":"10.1111/cen3.12761","DOIUrl":"10.1111/cen3.12761","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>To date, there is no journal that reports the coexistence of thymoma-associated myasthenia gravis, alopecia areata, and twenty-nail dystrophy. This paper hypothesizes that the presence of thymoma increases the risk of these conditions.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case presentation</h3>\u0000 \u0000 <p>The patient was a 37-year-old Filipino who came in for a year of history of fluctuating, but progressive, left-sided ptosis and diplopia; difficulty swallowing; dysarthria; and aspiration and symmetric proximal muscle weakness confirmed to be myasthenia gravis by abnormal repetitive nerve stimulation and the presence of thymoma on histology. Before his thymectomy, he also developed gradually worsening thinning and longitudinal ridging of all fingernails and toenails; and patches of bald areas on the scalp; diagnosed to be twenty-nail dystrophy and alopecia areata, respectively, by a board-certified dermatologist.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Thymoma is the possible risk factor and link for the co-occurrence of the three conditions in the present patient.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46129032","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}
Neuroinflammation, mediated by activated glial cells and infiltrated lymphocytes, leads to the subsequent production of pro-inflammatory cytokines and related molecules. It is associated with the pathomechanisms of various neurodegenerative diseases, including Alzheimer's disease (AD). Microglia, innate immune cells in the central nervous system, are the principal component of neuroinflammation in health and disease. In particular, the phenotypic heterogeneity of microglia has been extensively examined through single-cell RNA sequencing technology, providing a clue to further understanding of neuroinflammation in AD and related neurological diseases. In addition, the role of brain lymphocytes has gained attention in the disease setting. In the past, the central nervous system is known to be an immuneprivileged tissue in which adaptive immunity and inflammation are highly restricted and controlled. However, an increasing number of reports show the detrimental and protective role of T lymphocytes in AD rodent models. In this issue of Clinical and Experimental Neuroimmunology, we invited three review articles by leading researchers in the fields of AD and neuroimmunology. Saito et al. reviewed the role of neuroinflammation in rodent models for AD. The authors have contributed significantly to the development of novel mouse models of AD, such as App knock-in mice. In this review, they focused on the role of the glial cell network in the AD continuum, the pathological sequence of amyloid β, tau and neurodegeneration in AD, and discussed the contribution of disease-associated microglia/homeostatic microglia in disease. Furthermore, they discussed the brain–periphery interaction in AD pathogenesis, which is a hot topic in AD research. Finally, multimorbidity in AD pathogenesis was discussed based on the clinical evidence. Chihara et al. reviewed the role of T lymphocytes in AD pathogenesis. Prominent T-lymphocyte infiltration, with its contribution to disease development and progression, is well known in multiple sclerosis. Increased permeability of the blood–brain barrier and infiltration of T lymphocytes have been documented in various studies using the human AD brain. In this regard, further investigation of the immune mechanisms resulting from T-cell infiltration into the central nervous system during disease initiation and exacerbation is required. They also provided an extensive review on the contribution of each subset of T lymphocytes in various AD models. Although the effects of each T lymphocyte on AD pathology are variable among AD mouse models, we need to carefully investigate the role of T lymphocytes in AD pathogenesis. Maekawa and Yamanaka reviewed the role of sex steroid hormone in AD, based on human and experimental evidence. As the incidence and prevalence of AD are dominant in women, the putative roles of sex hormones in AD pathology have been investigated. Although the role of estrogens was extensively investigated in the studies of
{"title":"Neuroinflammation and neuroimmunology in Alzheimer's disease","authors":"Koji Yamanaka","doi":"10.1111/cen3.12750","DOIUrl":"10.1111/cen3.12750","url":null,"abstract":"Neuroinflammation, mediated by activated glial cells and infiltrated lymphocytes, leads to the subsequent production of pro-inflammatory cytokines and related molecules. It is associated with the pathomechanisms of various neurodegenerative diseases, including Alzheimer's disease (AD). Microglia, innate immune cells in the central nervous system, are the principal component of neuroinflammation in health and disease. In particular, the phenotypic heterogeneity of microglia has been extensively examined through single-cell RNA sequencing technology, providing a clue to further understanding of neuroinflammation in AD and related neurological diseases. In addition, the role of brain lymphocytes has gained attention in the disease setting. In the past, the central nervous system is known to be an immuneprivileged tissue in which adaptive immunity and inflammation are highly restricted and controlled. However, an increasing number of reports show the detrimental and protective role of T lymphocytes in AD rodent models. In this issue of Clinical and Experimental Neuroimmunology, we invited three review articles by leading researchers in the fields of AD and neuroimmunology. Saito et al. reviewed the role of neuroinflammation in rodent models for AD. The authors have contributed significantly to the development of novel mouse models of AD, such as App knock-in mice. In this review, they focused on the role of the glial cell network in the AD continuum, the pathological sequence of amyloid β, tau and neurodegeneration in AD, and discussed the contribution of disease-associated microglia/homeostatic microglia in disease. Furthermore, they discussed the brain–periphery interaction in AD pathogenesis, which is a hot topic in AD research. Finally, multimorbidity in AD pathogenesis was discussed based on the clinical evidence. Chihara et al. reviewed the role of T lymphocytes in AD pathogenesis. Prominent T-lymphocyte infiltration, with its contribution to disease development and progression, is well known in multiple sclerosis. Increased permeability of the blood–brain barrier and infiltration of T lymphocytes have been documented in various studies using the human AD brain. In this regard, further investigation of the immune mechanisms resulting from T-cell infiltration into the central nervous system during disease initiation and exacerbation is required. They also provided an extensive review on the contribution of each subset of T lymphocytes in various AD models. Although the effects of each T lymphocyte on AD pathology are variable among AD mouse models, we need to carefully investigate the role of T lymphocytes in AD pathogenesis. Maekawa and Yamanaka reviewed the role of sex steroid hormone in AD, based on human and experimental evidence. As the incidence and prevalence of AD are dominant in women, the putative roles of sex hormones in AD pathology have been investigated. Although the role of estrogens was extensively investigated in the studies of ","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-05-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48658686","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}
Anti-immunoglobulin-like cell adhesion molecule 5 (IgLON5) disease is an autoimmune encephalitis that targets the cell adhesion molecule, IgLON5. The disease presents with various clinical features, including sleep disorders, bulbar palsy, movement disorders, cognitive dysfunction and neuromuscular manifestations. Sleep disorders are characterized by parasomnias and sleep-disordered breathing (stridor and sleep apnea). Bulbar palsy includes dysarthria, dysphagia, vocal cord paralysis and stridor. Movement disorders include a variety of symptoms and signs, such as chorea, dystonia, rigidity, tremor, myoclonus and myorhythmia. Cognitive dysfunction includes executive dysfunction, impairment of attention, and verbal and visual memory dysfunction. Neuromuscular manifestations include fasciculations in the tongue and peripheral muscles, limb weakness, and muscle atrophy. Some patients resemble those with neurodegenerative diseases, such as progressive supranuclear palsy or amyotrophic lateral sclerosis. On video polysomnography, undifferentiated non-rapid eye movement sleep and poorly structured N2 sleep are characteristic. Brain magnetic resonance imaging and cerebrospinal fluid studies are often normal or non-specific. Human leukocyte antigen testing shows that HLA-DRB1*10:01-DQB1*05:01 is highly associated with the disease. Pathologically, neuronal deposition of both hyperphosphorylated 3-repeat and 4-repeat tau isoforms, neuronal loss, and gliosis in the hypothalamus, brainstem tegmentum, and upper cervical cord are observed. Some patients are responsive to immunotherapies, such as steroids, intravenous immunoglobulin, plasma exchange therapy and rituximab. Factors associated with a favorable response to immunotherapies include early initiation of treatment, cerebrospinal fluid inflammation and immunoglobulin G1 (IgG1) predominance of IgLON5 antibody compared with immunoglobulin G4 (IgG4). Anti-IgLON5 disease should be suspected in patients with atypical movement disorders complicated by sleep disturbances.
{"title":"Pathogenesis, clinical features and treatment of anti-IgLON5 disease","authors":"Yoya Ono, Akio Kimura, Takayoshi Shimohata","doi":"10.1111/cen3.12759","DOIUrl":"10.1111/cen3.12759","url":null,"abstract":"<p>Anti-immunoglobulin-like cell adhesion molecule 5 (IgLON5) disease is an autoimmune encephalitis that targets the cell adhesion molecule, IgLON5. The disease presents with various clinical features, including sleep disorders, bulbar palsy, movement disorders, cognitive dysfunction and neuromuscular manifestations. Sleep disorders are characterized by parasomnias and sleep-disordered breathing (stridor and sleep apnea). Bulbar palsy includes dysarthria, dysphagia, vocal cord paralysis and stridor. Movement disorders include a variety of symptoms and signs, such as chorea, dystonia, rigidity, tremor, myoclonus and myorhythmia. Cognitive dysfunction includes executive dysfunction, impairment of attention, and verbal and visual memory dysfunction. Neuromuscular manifestations include fasciculations in the tongue and peripheral muscles, limb weakness, and muscle atrophy. Some patients resemble those with neurodegenerative diseases, such as progressive supranuclear palsy or amyotrophic lateral sclerosis. On video polysomnography, undifferentiated non-rapid eye movement sleep and poorly structured N2 sleep are characteristic. Brain magnetic resonance imaging and cerebrospinal fluid studies are often normal or non-specific. Human leukocyte antigen testing shows that HLA-DRB1*10:01-DQB1*05:01 is highly associated with the disease. Pathologically, neuronal deposition of both hyperphosphorylated 3-repeat and 4-repeat tau isoforms, neuronal loss, and gliosis in the hypothalamus, brainstem tegmentum, and upper cervical cord are observed. Some patients are responsive to immunotherapies, such as steroids, intravenous immunoglobulin, plasma exchange therapy and rituximab. Factors associated with a favorable response to immunotherapies include early initiation of treatment, cerebrospinal fluid inflammation and immunoglobulin G1 (IgG1) predominance of IgLON5 antibody compared with immunoglobulin G4 (IgG4). Anti-IgLON5 disease should be suspected in patients with atypical movement disorders complicated by sleep disturbances.</p>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-05-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42578899","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}
Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is a rare inflammatory disease of the central nervous system. Unilateral cortical fluid-attenuated inversion recovery-hyperintense lesions in anti-MOG-associated encephalitis with seizures (FLAMES) has recently been proposed as a subcategory of MOGAD. FLAMES is characterized by fluid-attenuated inversion recovery (FLAIR) imaging showing hyperintense cortical lesions in MOG-associated encephalitis with seizures.
Case Presentation
We herein report two cases of unilateral cortical FLAMES. The first case was a 29-year-old woman who developed headaches, fever, convulsions and right hemiparesis. Brain FLAIR magnetic resonance imaging (MRI) showed hyperintense cortical lesions on the left side. The second case was a 37-year-old women who developed headaches and fever. Brain FLAIR MRI showed hyperintense cortical lesions on the left side. Both cases were positive for anti-MOG antibodies in cerebrospinal fluid and serum, and were diagnosed with unilateral cortical FLAMES in MOGAD. Both patients were treated with intravenous methylprednisolone followed by oral corticosteroids, which improved MRI findings and clinical symptoms.
Conclusions
Both patients were diagnosed with MOGAD due to characteristic unilateral cortical encephalitis on brain FLAIR MRI. Unilateral cortical FLAMES is an important clue for the clinical diagnosis of MOGAD.
{"title":"Two cases of unilateral cortical fluid-attenuated inversion recovery-hyperintense lesions in anti-myelin oligodendrocyte glycoprotein-associated encephalitis with seizures (FLAMES)","authors":"Kunihiko Ishizawa, Osamu Iwasaki, Hironori Oka, Takashi Sugawara, Masakuni Amari, Takeshi Kawarabayashi, Koichi Okamoto, Kimihiko Kaneko, Toshiyuki Takahashi, Yoshio Ikeda, Masamitsu Takatama, Mikio Shoji","doi":"10.1111/cen3.12753","DOIUrl":"10.1111/cen3.12753","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is a rare inflammatory disease of the central nervous system. Unilateral cortical fluid-attenuated inversion recovery-hyperintense lesions in anti-MOG-associated encephalitis with seizures (FLAMES) has recently been proposed as a subcategory of MOGAD. FLAMES is characterized by fluid-attenuated inversion recovery (FLAIR) imaging showing hyperintense cortical lesions in MOG-associated encephalitis with seizures.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>We herein report two cases of unilateral cortical FLAMES. The first case was a 29-year-old woman who developed headaches, fever, convulsions and right hemiparesis. Brain FLAIR magnetic resonance imaging (MRI) showed hyperintense cortical lesions on the left side. The second case was a 37-year-old women who developed headaches and fever. Brain FLAIR MRI showed hyperintense cortical lesions on the left side. Both cases were positive for anti-MOG antibodies in cerebrospinal fluid and serum, and were diagnosed with unilateral cortical FLAMES in MOGAD. Both patients were treated with intravenous methylprednisolone followed by oral corticosteroids, which improved MRI findings and clinical symptoms.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Both patients were diagnosed with MOGAD due to characteristic unilateral cortical encephalitis on brain FLAIR MRI. Unilateral cortical FLAMES is an important clue for the clinical diagnosis of MOGAD.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-05-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44532334","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}
Among the different forms of cerebellar ataxia, autoimmune cerebellar ataxia (ACA) or immune-mediated cerebellar ataxia (IMCA), which appears to be based on an autoimmune mechanism, has long been considered important from the viewpoint of paraneoplastic syndrome. With the expansion of the concept of immune-mediated neurological diseases and the identification of many novel autoantibodies, ACA has recently become an important neurological syndrome. Although no definitive diagnostic criteria of ACA exist, Hadjivassilou et al suggested diagnostic criteria for primary ACA in 2020 and Dalmau and Graus showed proposed diagnostic criteria for ACA in 2022. The proposed diagnostic criteria for ACA by Dalmau and Graus emphasize the importance of antibody reliability. In the future, additional studies should be conducted to identify new antibodies associated with ACA and to clarify the importance of each antibody. Moreover, ACA is a disease for which immunological therapeutic intervention is feasible and requires early treatment to maintain cerebellar function. Many cases with ACA have reported the efficacy of immunotherapy. The concept of ACA may have the potential for further expansion and becomes increasingly important in the diagnosis of cerebellar ataxia.
{"title":"Autoimmune cerebellar ataxia","authors":"Hiroaki Yaguchi, Akihiko Kudo, Ichiro Yabe","doi":"10.1111/cen3.12752","DOIUrl":"10.1111/cen3.12752","url":null,"abstract":"<p>Among the different forms of cerebellar ataxia, autoimmune cerebellar ataxia (ACA) or immune-mediated cerebellar ataxia (IMCA), which appears to be based on an autoimmune mechanism, has long been considered important from the viewpoint of paraneoplastic syndrome. With the expansion of the concept of immune-mediated neurological diseases and the identification of many novel autoantibodies, ACA has recently become an important neurological syndrome. Although no definitive diagnostic criteria of ACA exist, Hadjivassilou et al suggested diagnostic criteria for primary ACA in 2020 and Dalmau and Graus showed proposed diagnostic criteria for ACA in 2022. The proposed diagnostic criteria for ACA by Dalmau and Graus emphasize the importance of antibody reliability. In the future, additional studies should be conducted to identify new antibodies associated with ACA and to clarify the importance of each antibody. Moreover, ACA is a disease for which immunological therapeutic intervention is feasible and requires early treatment to maintain cerebellar function. Many cases with ACA have reported the efficacy of immunotherapy. The concept of ACA may have the potential for further expansion and becomes increasingly important in the diagnosis of cerebellar ataxia.</p>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-05-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44695003","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}
Various neurological disorders have been reported after vaccination against coronavirus disease 2019, one of which is Guillain–Barré Syndrome (GBS).
Case Presentation
We report a case of a 73-year-old woman who developed GBS and extra-GBS manifestations 19 days after the second dose of BNT162b2 mRNA vaccine. She presented lower limb predominant muscle weakness and loss of tendon reflexes. Nerve conduction study showed acute motor and sensory axonal neuropathy. In addition, she developed notable deep sensory ataxia, and showed positive pathological reflex, gaze-evoked nystagmus and altered consciousness, which suggested brainstem involvement.
Conclusions
This is the first coronavirus disease 2019 vaccine-related GBS complicated with such central nervous system manifestations.
据报道，接种2019冠状病毒疫苗后出现了各种神经系统疾病，其中一种是格林-巴罗综合征(GBS)。
{"title":"Post-COVID-19 vaccination Guillain–Barré syndrome with sensory ataxia, gaze-evoked nystagmus, mental-status change and positive pathological reflex","authors":"Nanami Saso, Shuta Toru, Keiichi Iwasaru, Hiroaki Yokote, Toshiki Uchihara","doi":"10.1111/cen3.12754","DOIUrl":"10.1111/cen3.12754","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Various neurological disorders have been reported after vaccination against coronavirus disease 2019, one of which is Guillain–Barré Syndrome (GBS).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>We report a case of a 73-year-old woman who developed GBS and extra-GBS manifestations 19 days after the second dose of BNT162b2 mRNA vaccine. She presented lower limb predominant muscle weakness and loss of tendon reflexes. Nerve conduction study showed acute motor and sensory axonal neuropathy. In addition, she developed notable deep sensory ataxia, and showed positive pathological reflex, gaze-evoked nystagmus and altered consciousness, which suggested brainstem involvement.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>This is the first coronavirus disease 2019 vaccine-related GBS complicated with such central nervous system manifestations.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-04-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41821434","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}
Inflammation in the central nervous system (CNS) leads to local damage causing peripheral disability. Adaptive immune responses may lead the way in autoimmune neuroinflammatory disease, such as multiple sclerosis (MS), as indicated by large genome-wide association studies, histologic analysis, and the efficacy of drugs targeting lymphocytes. 1 Research from our group and others indicates a previously underappreciated key role for T-helper (Th) cells in progressive MS and other neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS) and Alzheimer's disease (AD). 2,3 Such Th cells initiate neuroinflammation after becoming inappropriately activated against self-antigen, leading to immune responses against self-tissues. Rapid identification of aberrant Th cells would
{"title":"Neuropilin-1 (NRP1): A new marker for pathogenic autoreactive T-helper cells in autoimmune disease","authors":"Ben J. E. Raveney, Takashi Yamamura, Shinji Oki","doi":"10.1111/cen3.12751","DOIUrl":"10.1111/cen3.12751","url":null,"abstract":"Inflammation in the central nervous system (CNS) leads to local damage causing peripheral disability. Adaptive immune responses may lead the way in autoimmune neuroinflammatory disease, such as multiple sclerosis (MS), as indicated by large genome-wide association studies, histologic analysis, and the efficacy of drugs targeting lymphocytes. 1 Research from our group and others indicates a previously underappreciated key role for T-helper (Th) cells in progressive MS and other neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS) and Alzheimer's disease (AD). 2,3 Such Th cells initiate neuroinflammation after becoming inappropriately activated against self-antigen, leading to immune responses against self-tissues. Rapid identification of aberrant Th cells would","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-04-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46238916","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}
Mohammad Aladawi, Daniel Crespo, Renfeng Xu, Rana Zabad, Amrita-Amanda Vuppala
Background
Severe acute respiratory syndrome coronavirus 2 (SARSCoV2) has been associated with several neuro-ophthalmic manifestations. We report a case of bilateral longitudinally extensive optic perineuritis suspected due to SARSCoV2.
Case Presentation
A 32-year-old woman developed headaches, photophobia, pulsatile tinnitus, and blurred vision 8 d after having a positive SARS-CoV-2 qualitative polymerase chain reaction (PCR) testing for coronavirus disease 2019 (COVID-19). She was diagnosed with and treated for idiopathic intracranial hypertension (IIH) elsewhere. Repeat evaluation at our institution showed a poor visual acuity in both eyes with Frisen grade II papilledema and cotton wool spots on fundoscopic examination. Orbital magnetic resonance imaging (MRI) showed bilateral longitudinally extensive optic nerve sheath enhancement. Repeat lumbar puncture revealed an elevated cerebrospinal fluid (CSF) opening pressure and protein, a finding that is incompatible with the diagnosis of IIH. Myelin oligodendrocyte glycoprotein, aquaporin-4 (AQP4)-IgG antibodies, and other serological tests for optic neuritis were unremarkable. Her visual acuity partially improved after corticosteroids. With the growing association of demyelinating disorders and COVID-19, unremarkable serological workup, and temporal relation of the patient's symptoms to the infection, we believe that her diagnosis is SARS-CoV-2 associated bilateral optic neuritis.
Conclusion
There is a growing association between demyelinating disorders and COVID-19 and COVID-19 vaccination, and it is essential to recognize CSF abnormalities that are incompatible with a diagnosis of IIH, such as increased protein in our case, and may lead to an incorrect diagnosis.
{"title":"Bilateral longitudinally extensive optic perineuritis post-COVID-19 presenting as “idiopathic” intracranial hypertension: A case report","authors":"Mohammad Aladawi, Daniel Crespo, Renfeng Xu, Rana Zabad, Amrita-Amanda Vuppala","doi":"10.1111/cen3.12749","DOIUrl":"10.1111/cen3.12749","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Severe acute respiratory syndrome coronavirus 2 (SARSCoV2) has been associated with several neuro-ophthalmic manifestations. We report a case of bilateral longitudinally extensive optic perineuritis suspected due to SARSCoV2.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>A 32-year-old woman developed headaches, photophobia, pulsatile tinnitus, and blurred vision 8 d after having a positive SARS-CoV-2 qualitative polymerase chain reaction (PCR) testing for coronavirus disease 2019 (COVID-19). She was diagnosed with and treated for idiopathic intracranial hypertension (IIH) elsewhere. Repeat evaluation at our institution showed a poor visual acuity in both eyes with Frisen grade II papilledema and cotton wool spots on fundoscopic examination. Orbital magnetic resonance imaging (MRI) showed bilateral longitudinally extensive optic nerve sheath enhancement. Repeat lumbar puncture revealed an elevated cerebrospinal fluid (CSF) opening pressure and protein, a finding that is incompatible with the diagnosis of IIH. Myelin oligodendrocyte glycoprotein, aquaporin-4 (AQP4)-IgG antibodies, and other serological tests for optic neuritis were unremarkable. Her visual acuity partially improved after corticosteroids. With the growing association of demyelinating disorders and COVID-19, unremarkable serological workup, and temporal relation of the patient's symptoms to the infection, we believe that her diagnosis is SARS-CoV-2 associated bilateral optic neuritis.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>There is a growing association between demyelinating disorders and COVID-19 and COVID-19 vaccination, and it is essential to recognize CSF abnormalities that are incompatible with a diagnosis of IIH, such as increased protein in our case, and may lead to an incorrect diagnosis.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-03-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/cen3.12749","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49199311","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}
To the Editor, Multifocal motor neuropathy (MMN) is a rare immune-mediated neuropathy characterized by progressive asymmetric limb weakness with the expression of anti-GM1 immunoglobulin M (IgM) antibodies. Conduction block (CB) shown by nerve conduction studies (NCS) is the most important feature for its diagnosis. A 28-year-old Japanese woman presented with a week-long history of progressive right hand muscle weakness without any sensory dysfunction. Her grip strength was 12/25 kg (right/left), the muscle strength of both the abductor pollicis brevis and abductor digiti minimi was 5/5. That of both the wrist extensor and wrist flexor was 5/5 according to the Medical Research Council grade. Her tendon reflexes were normal in the upper and lower limbs. The Babinski sign was bilaterally negative. Routine NCS recorded from the abductor pollicis brevis (Figure 1a) and abductor digiti minimi (Figure 1b) was in the normal range (skin temperature was maintained at 32 C). A cerebrospinal fluid examination detected no cells and showed a normal protein concentration (22.3 mg/ dL). Serum immunoglobulin G (1603 mg/dL) and IgM (166 mg/dL) were within the normal range, and serum anti-nuclear antibodies were negative. Serological testing for anti-ganglioside antibodies were positive for anti-GM1 IgM (+) and anti-GalNAc-GD1a IgM (++) antibodies. We initially suspected MMN without CB. The patient was given a 5-day course of high-dose intravenous immunoglobulin (IVIG) therapy (0.4 g/kg/day). Her right grip strength improved to 25 kg (left 25 kg). Both the anti-GM1 IgM (+) and antiGalNAc-GD1a IgM (++) antibodies were still positive 8 weeks after the first IVIG therapy. After another 6 months, her right grip strength declined to 12 kg. The muscle strength of the flexor digitorum profundus (FDP) was 4/5 (right/left), although that of the abductor pollicis brevis, abductor digiti minimi, extensor carpi radialis longus, extensor carpi ulnaris, flexor carpi radialis, extensor digitorum communis, abductor pollicis longus, extensor pollicis brevis, extensor pollicis longus, flexor pollicis longus, wrist extensor and wrist flexor was 5/5 (right/left; Medical Research Council grade). We carried out NCS of the FDP muscle innervated from the ulnar nerve (Figure 1c), CB was shown by a 19.6% reduction in compound muscle action potentials (CMAP; 5.1 to 4.1 mV) and 33.5% reduction in the CMAP area (33.1 to 22.0 mVms) between 42 and 18 mm from the medial epicondyle with normal motor conduction velocities (Figure 1d,e; 42 to 18 mm from the medial epicondyle = 68.6 m/s). These electrophysiological findings fulfilled the European Federation of Neurological Societies' electrophysiological criteria for possible motor CB in MMN; we finally diagnosed her with MMN. IVIG therapy was effective and her right grip strength improved to 28 kg. One month after the second IVIG treatment, the CB improved to 6.5% (4.6 to 4.3 mV) for the CMAP and 9.9% for CMAP area (26.3 to 23.6 mVms) at the same d
{"title":"Multifocal motor neuropathy with conduction block that was shown by the flexor digitorum profundus muscle innervated from the ulnar nerve","authors":"Kazuyuki Saito, Hiroaki Yokote, Shuta Toru","doi":"10.1111/cen3.12748","DOIUrl":"10.1111/cen3.12748","url":null,"abstract":"To the Editor, Multifocal motor neuropathy (MMN) is a rare immune-mediated neuropathy characterized by progressive asymmetric limb weakness with the expression of anti-GM1 immunoglobulin M (IgM) antibodies. Conduction block (CB) shown by nerve conduction studies (NCS) is the most important feature for its diagnosis. A 28-year-old Japanese woman presented with a week-long history of progressive right hand muscle weakness without any sensory dysfunction. Her grip strength was 12/25 kg (right/left), the muscle strength of both the abductor pollicis brevis and abductor digiti minimi was 5/5. That of both the wrist extensor and wrist flexor was 5/5 according to the Medical Research Council grade. Her tendon reflexes were normal in the upper and lower limbs. The Babinski sign was bilaterally negative. Routine NCS recorded from the abductor pollicis brevis (Figure 1a) and abductor digiti minimi (Figure 1b) was in the normal range (skin temperature was maintained at 32 C). A cerebrospinal fluid examination detected no cells and showed a normal protein concentration (22.3 mg/ dL). Serum immunoglobulin G (1603 mg/dL) and IgM (166 mg/dL) were within the normal range, and serum anti-nuclear antibodies were negative. Serological testing for anti-ganglioside antibodies were positive for anti-GM1 IgM (+) and anti-GalNAc-GD1a IgM (++) antibodies. We initially suspected MMN without CB. The patient was given a 5-day course of high-dose intravenous immunoglobulin (IVIG) therapy (0.4 g/kg/day). Her right grip strength improved to 25 kg (left 25 kg). Both the anti-GM1 IgM (+) and antiGalNAc-GD1a IgM (++) antibodies were still positive 8 weeks after the first IVIG therapy. After another 6 months, her right grip strength declined to 12 kg. The muscle strength of the flexor digitorum profundus (FDP) was 4/5 (right/left), although that of the abductor pollicis brevis, abductor digiti minimi, extensor carpi radialis longus, extensor carpi ulnaris, flexor carpi radialis, extensor digitorum communis, abductor pollicis longus, extensor pollicis brevis, extensor pollicis longus, flexor pollicis longus, wrist extensor and wrist flexor was 5/5 (right/left; Medical Research Council grade). We carried out NCS of the FDP muscle innervated from the ulnar nerve (Figure 1c), CB was shown by a 19.6% reduction in compound muscle action potentials (CMAP; 5.1 to 4.1 mV) and 33.5% reduction in the CMAP area (33.1 to 22.0 mVms) between 42 and 18 mm from the medial epicondyle with normal motor conduction velocities (Figure 1d,e; 42 to 18 mm from the medial epicondyle = 68.6 m/s). These electrophysiological findings fulfilled the European Federation of Neurological Societies' electrophysiological criteria for possible motor CB in MMN; we finally diagnosed her with MMN. IVIG therapy was effective and her right grip strength improved to 28 kg. One month after the second IVIG treatment, the CB improved to 6.5% (4.6 to 4.3 mV) for the CMAP and 9.9% for CMAP area (26.3 to 23.6 mVms) at the same d","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-03-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41301271","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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