Clinical and Experimental Neuroimmunology最新文献

{"title":"Author's Reply to “Autoimmune Cerebellar Ataxia During TNF-α Inhibitor Therapy: Causality or Coincidence?”","authors":"Tomohide Ando, Nobuaki Yoshikura, Akira Takekoshi, Akio Kimura, Takayoshi Shimohata","doi":"10.1111/cen3.70071","DOIUrl":"https://doi.org/10.1111/cen3.70071","url":null,"abstract":"","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"17 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-06-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148324577","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":"Involvement of Regulatory T Cells in the Pathophysiology of Myasthenia Gravis: From a Pediatrician's Perspective","authors":"Masatoshi Hayashi,&nbsp;Koji Nagatani","doi":"10.1111/cen3.70069","DOIUrl":"https://doi.org/10.1111/cen3.70069","url":null,"abstract":"<div>\u0000 \u0000 <p>Myasthenia gravis (MG) is thought to be primarily caused by autoantibodies targeting proteins at the neuromuscular junction. It has been established that autoantibodies, primarily acetylcholine receptor (AChR) antibodies, act on the neuromuscular junction, impairing neuromuscular transmission or destroying the structure of the neuromuscular junction. However, cellular immunity also plays a significant role, activating various T-cell subsets and stimulating B cells to produce antibodies, ultimately leading to disease onset. Immune responsiveness changes throughout life. The immune system develops and matures after birth, then declines with age. At each stage, mechanisms protect the body against pathogenic microorganism invasion. It can also lead to conditions where the immune response is insufficient, even against neoplasms formed within the body. This immune response fundamentally operates through the balance between effector T (Teff) cells and regulatory T (Treg) cells. Imbalances in this equilibrium can occur during the course of MG and various other pathologies. Treatment strategies and methods often achieve therapeutic effects by normalizing this balance. In this review, from the perspective of a pediatrician who has long treated patients with not only adult MG but also childhood MG, we would like to discuss the role Treg cells play in regulating this activation of cellular immunity.</p>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"17 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-06-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148324133","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":"Involvement of Regulatory T Cells in the Pathophysiology of Myasthenia Gravis: From a Pediatrician's Perspective","authors":"Masatoshi Hayashi,&nbsp;Koji Nagatani","doi":"10.1111/cen3.70069","DOIUrl":"https://doi.org/10.1111/cen3.70069","url":null,"abstract":"<div>\u0000 \u0000 <p>Myasthenia gravis (MG) is thought to be primarily caused by autoantibodies targeting proteins at the neuromuscular junction. It has been established that autoantibodies, primarily acetylcholine receptor (AChR) antibodies, act on the neuromuscular junction, impairing neuromuscular transmission or destroying the structure of the neuromuscular junction. However, cellular immunity also plays a significant role, activating various T-cell subsets and stimulating B cells to produce antibodies, ultimately leading to disease onset. Immune responsiveness changes throughout life. The immune system develops and matures after birth, then declines with age. At each stage, mechanisms protect the body against pathogenic microorganism invasion. It can also lead to conditions where the immune response is insufficient, even against neoplasms formed within the body. This immune response fundamentally operates through the balance between effector T (Teff) cells and regulatory T (Treg) cells. Imbalances in this equilibrium can occur during the course of MG and various other pathologies. Treatment strategies and methods often achieve therapeutic effects by normalizing this balance. In this review, from the perspective of a pediatrician who has long treated patients with not only adult MG but also childhood MG, we would like to discuss the role Treg cells play in regulating this activation of cellular immunity.</p>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"17 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-06-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148324134","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":"Concurrent Hashimoto's Encephalopathy and Papillary Thyroid Carcinoma: A Case Report","authors":"Ryo Hara,&nbsp;Toshiyuki Kakumoto,&nbsp;Anna Suzuki,&nbsp;Satoshi Yamashita,&nbsp;Takayoshi Niwa,&nbsp;Kanako Hayashi,&nbsp;Arisa Morizono,&nbsp;Ayaka Sato,&nbsp;Masahiko Tanabe,&nbsp;Noritaka Kudo,&nbsp;Masashi Hamada,&nbsp;Wataru Satake,&nbsp;Tatsushi Toda","doi":"10.1111/cen3.70070","DOIUrl":"https://doi.org/10.1111/cen3.70070","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Hashimoto's encephalopathy (HE) is a rare steroid-responsive autoimmune syndrome. Although HE is associated with anti-thyroid antibodies, its concurrence with malignant thyroid tumors has rarely been described and its clinical implications remain poorly understood.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>A 54-year-old euthyroid woman presented with a 5-month history of progressive cognitive decline and behavioral changes, leading to profound hypolalia and restlessness. Serum anti-thyroglobulin and anti-thyroid peroxidase antibodies were markedly elevated. Brain MRI, electroencephalography, and cerebrospinal fluid tests were unremarkable. Corticosteroid therapy resulted in rapid resolution of neuropsychiatric symptoms and cognitive dysfunction. Subsequent thyroid evaluation identified papillary thyroid carcinoma (PTC) on a background of chronic lymphocytic thyroiditis. The patient underwent total thyroidectomy and radioiodine therapy. At 1-year follow-up, she remained free of HE relapses and cancer recurrence, with a corresponding decline in anti-thyroid antibody titers.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>This is the first reported case of HE occurring concurrently with PTC. The findings raise the possibility that a shared autoimmune milieu may predispose to both HE and PTC. This case underscores the importance of comprehensive thyroid evaluation, including oncological screening, in patients with HE.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"17 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/cen3.70070","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148282548","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":"Concurrent Hashimoto's Encephalopathy and Papillary Thyroid Carcinoma: A Case Report","authors":"Ryo Hara,&nbsp;Toshiyuki Kakumoto,&nbsp;Anna Suzuki,&nbsp;Satoshi Yamashita,&nbsp;Takayoshi Niwa,&nbsp;Kanako Hayashi,&nbsp;Arisa Morizono,&nbsp;Ayaka Sato,&nbsp;Masahiko Tanabe,&nbsp;Noritaka Kudo,&nbsp;Masashi Hamada,&nbsp;Wataru Satake,&nbsp;Tatsushi Toda","doi":"10.1111/cen3.70070","DOIUrl":"https://doi.org/10.1111/cen3.70070","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Hashimoto's encephalopathy (HE) is a rare steroid-responsive autoimmune syndrome. Although HE is associated with anti-thyroid antibodies, its concurrence with malignant thyroid tumors has rarely been described and its clinical implications remain poorly understood.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>A 54-year-old euthyroid woman presented with a 5-month history of progressive cognitive decline and behavioral changes, leading to profound hypolalia and restlessness. Serum anti-thyroglobulin and anti-thyroid peroxidase antibodies were markedly elevated. Brain MRI, electroencephalography, and cerebrospinal fluid tests were unremarkable. Corticosteroid therapy resulted in rapid resolution of neuropsychiatric symptoms and cognitive dysfunction. Subsequent thyroid evaluation identified papillary thyroid carcinoma (PTC) on a background of chronic lymphocytic thyroiditis. The patient underwent total thyroidectomy and radioiodine therapy. At 1-year follow-up, she remained free of HE relapses and cancer recurrence, with a corresponding decline in anti-thyroid antibody titers.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>This is the first reported case of HE occurring concurrently with PTC. The findings raise the possibility that a shared autoimmune milieu may predispose to both HE and PTC. This case underscores the importance of comprehensive thyroid evaluation, including oncological screening, in patients with HE.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"17 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/cen3.70070","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148282549","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":"IgG4-Related Disease Overlapping With Anti-Acetylcholine Receptor Antibody–Positive Myasthenia Gravis","authors":"Yumiko Yoshida,&nbsp;Ayami Okuzumi,&nbsp;Yuka Hosaka,&nbsp;Yuji Tomizawa,&nbsp;Taku Hatano","doi":"10.1111/cen3.70068","DOIUrl":"https://doi.org/10.1111/cen3.70068","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>IgG4-related disease (IgG4-RD) is a systemic, immune-mediated, fibroinflammatory disorder with multiorgan involvement and variable clinical presentation, often creating diagnostic difficulty. Myasthenia gravis (MG) is an antibody-mediated autoimmune disorder of the neuromuscular junction, most commonly associated with anti-acetylcholine receptor (AChR) antibodies. Although both conditions involve immune dysregulation and affect the ocular region, their coexistence is extremely rare. We report a rare case of anti-AChR antibody–positive MG overlapping with IgG4-RD.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>A 66-year-old man developed bilateral diplopia, ptosis, and mild eyelid swelling over 3 months. Anti-AChR antibodies were positive, and an edrophonium test improved ptosis. Single-fiber electromyography showed abnormal jitter, leading to a diagnosis of ocular MG. Chest CT revealed an anterior mediastinal nodule, and thymectomy showed follicular hyperplasia. Despite pyridostigmine, tacrolimus, and surgery, the patient's ocular symptoms progressively worsened and he was admitted for re-evaluation. Although serum IgG4 was within the normal range, imaging showed bilateral enlargement and enhancement of the lacrimal glands and extraocular muscles. Lacrimal gland biopsy showed dense lymphoplasmacytic infiltration, fibrosis, approximately 20 IgG4-positive plasma cells per high-power field, and an IgG4/IgG ratio &gt; 40%. The patient fulfilled the 2019 ACR/EULAR classification criteria for IgG4-RD. Intravenous methylprednisolone followed by oral prednisolone markedly improved eyelid swelling, although ocular movement limitation persisted at 6 months.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>IgG4-RD may closely mimic ocular MG and delay diagnosis, particularly in seronegative cases. Unexplained eyelid or lacrimal gland swelling in refractory MG should prompt consideration of IgG4-RD and early histopathological evaluation.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"17 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/cen3.70068","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148282051","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":"IgG4-Related Disease Overlapping With Anti-Acetylcholine Receptor Antibody–Positive Myasthenia Gravis","authors":"Yumiko Yoshida,&nbsp;Ayami Okuzumi,&nbsp;Yuka Hosaka,&nbsp;Yuji Tomizawa,&nbsp;Taku Hatano","doi":"10.1111/cen3.70068","DOIUrl":"https://doi.org/10.1111/cen3.70068","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>IgG4-related disease (IgG4-RD) is a systemic, immune-mediated, fibroinflammatory disorder with multiorgan involvement and variable clinical presentation, often creating diagnostic difficulty. Myasthenia gravis (MG) is an antibody-mediated autoimmune disorder of the neuromuscular junction, most commonly associated with anti-acetylcholine receptor (AChR) antibodies. Although both conditions involve immune dysregulation and affect the ocular region, their coexistence is extremely rare. We report a rare case of anti-AChR antibody–positive MG overlapping with IgG4-RD.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>A 66-year-old man developed bilateral diplopia, ptosis, and mild eyelid swelling over 3 months. Anti-AChR antibodies were positive, and an edrophonium test improved ptosis. Single-fiber electromyography showed abnormal jitter, leading to a diagnosis of ocular MG. Chest CT revealed an anterior mediastinal nodule, and thymectomy showed follicular hyperplasia. Despite pyridostigmine, tacrolimus, and surgery, the patient's ocular symptoms progressively worsened and he was admitted for re-evaluation. Although serum IgG4 was within the normal range, imaging showed bilateral enlargement and enhancement of the lacrimal glands and extraocular muscles. Lacrimal gland biopsy showed dense lymphoplasmacytic infiltration, fibrosis, approximately 20 IgG4-positive plasma cells per high-power field, and an IgG4/IgG ratio &gt; 40%. The patient fulfilled the 2019 ACR/EULAR classification criteria for IgG4-RD. Intravenous methylprednisolone followed by oral prednisolone markedly improved eyelid swelling, although ocular movement limitation persisted at 6 months.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>IgG4-RD may closely mimic ocular MG and delay diagnosis, particularly in seronegative cases. Unexplained eyelid or lacrimal gland swelling in refractory MG should prompt consideration of IgG4-RD and early histopathological evaluation.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"17 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/cen3.70068","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148282065","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":"Autoimmune Cerebellar Ataxia During TNF-α Inhibitor Therapy: Causality or Coincidence?","authors":"Christian Messina","doi":"10.1111/cen3.70067","DOIUrl":"https://doi.org/10.1111/cen3.70067","url":null,"abstract":"","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"17 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148237856","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":"Autoimmune Cerebellar Ataxia During TNF-α Inhibitor Therapy: Causality or Coincidence?","authors":"Christian Messina","doi":"10.1111/cen3.70067","DOIUrl":"https://doi.org/10.1111/cen3.70067","url":null,"abstract":"","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"17 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148237857","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":"A Rare Overlap of AQP4 and MOG Seropositivity in Neuromyelitis Optica Spectrum Disorder: A Case Report","authors":"Sarah E. Butler,&nbsp;Siew Kar Chen,&nbsp;Mastura Monif","doi":"10.1111/cen3.70066","DOIUrl":"https://doi.org/10.1111/cen3.70066","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Neuromyelitis optica spectrum disorder (NMOSD) is an immune-mediated disorder of the central nervous system associated with autoantibodies against aquaporin-4 (AQP4). This is distinct from myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD), defined by anti-MOG antibodies. The coexistence of AQP4 and MOG antibodies is rare and remains incompletely understood.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>We present a rare case of a 53-year-old woman with dual AQP4 and MOG antibodies in serum and a clinical presentation of recurrent myelitis. She presented with four discrete episodes of bilateral lower limb sensory deficits and mild weakness. Cerebrospinal fluid analysis demonstrated lymphocytic pleocytosis without oligoclonal bands. An MRI spine demonstrated high T2 signal in the right anterior hemicord; MRI brain demonstrated nodular enhancement within the posterior horn and atrium of the right lateral ventricle. High-titre anti-AQP4 and anti-MOG antibodies were detected in serum and remained positive on serial cell-based assays. Despite maintenance intravenous immunoglobulin, she relapsed and was commenced on rituximab, achieving subsequent clinical stability.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Dual anti-AQP4 and anti-MOG seropositivity is rare and presents diagnostic and therapeutic challenges. This case highlights overlapping clinical features of NMOSD and MOGAD and the need for further research to guide classification and management.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"17 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-06-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/cen3.70066","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148237579","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}