Journal of neuroimmunology最新文献

{"title":"Exosomes derived from neural stem cells contribute to cerebral ischemia/reperfusion injury via inhibiting autophagy in rats","authors":"Li Xiong ,&nbsp;Siying Huo ,&nbsp;Yuan Yang ,&nbsp;Qi Zhang ,&nbsp;Junjie Li ,&nbsp;Wenya Bai ,&nbsp;Jia Liu ,&nbsp;Jianlin Shao","doi":"10.1016/j.jneuroim.2026.578875","DOIUrl":"10.1016/j.jneuroim.2026.578875","url":null,"abstract":"<div><h3>Background</h3><div>Cerebral ischemia-reperfusion injury (CIRI) represents a critical pathological mechanism underlying stroke. Exosomes (EXOs) are biological vesicles released by cells, containing active components and functional properties of their cell of origin. This study investigates the impact of human neural stem cell-derived exosomes (hNSC-EXOs) on the CIRI rat model, focusing on neuronal autophagy, and provides a novel theoretical foundation for future clinical interventions in CIRI management.</div></div><div><h3>Methods</h3><div>PKH26-labeled hNSC-EXOs were traced in vivo and in vitro. After establishing CIRI model, hNSC-EXOs were administered for treatment. Brain injury and variations in inflammatory factors were compared at 24 h after operation. The impact of hNSC-EXOs on neuronal autophagy was examined by detecting the expression of Beclin-1, Atg-5, and LC3B, and observing the changes in the number of autophagosomes using TEM of the right cerebral cortex of rats.</div></div><div><h3>Results</h3><div>The average particle size and concentration of hNSC-EXO were 64.47 nm and 8.96 × 10¹⁰/ml, respectively. PKH26-labeled hNSC-EXOs was taken up by BV2 and HT22 cells, and was mainly located in the brain injury area in vivo, with their total flux showing a time-dependent characteristic. hNSC-EXO treatment reduced the neurological score, cerebral infarct volume, and cerebral edema in the CIRI model. Brain tissue staining showed that hNSC-EXO attenuated Nissl body damage and neuronal apoptosis in the CIRI model. Regarding inflammatory factors, hNSC-EXO increased serum levels of IL-4 and IL-10, and decreased levels of TNF-α, iNOS, and IL-6 in the CIRI model. Notably, hNSC-EXO reduced the expression of Beclin-1, Atg-5, and LC3 and the number of autophagosomes in the brain tissue of the CIRI model.</div></div><div><h3>Conclusion</h3><div>hNSC-EXOs exert an antagonistic effect with CIRI, effectively inhibiting excessive neuronal autophagy, reducing the inflammatory response and neuronal apoptosis.</div></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"413 ","pages":"Article 578875"},"PeriodicalIF":2.5,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146079049","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"TMEM132A autoimmunity in patients with suspected autoimmune cerebellar ataxia","authors":"Akio Kimura ,&nbsp;Akira Takekoshi ,&nbsp;Yuri Miyazaki ,&nbsp;Kentaro Oh-hashi ,&nbsp;Sachiko Kamada ,&nbsp;Yukie Taguchi ,&nbsp;Masashiro Sugawara ,&nbsp;Tsubasa Watanabe ,&nbsp;Yuji Ueno ,&nbsp;Hiroaki Yaguchi ,&nbsp;Ichiro Yabe ,&nbsp;Yuko Fukata ,&nbsp;Masaki Fukata ,&nbsp;Takayoshi Shimohata","doi":"10.1016/j.jneuroim.2026.578867","DOIUrl":"10.1016/j.jneuroim.2026.578867","url":null,"abstract":"<div><div>Autoimmune cerebellar ataxia (ACA) refers to immune-mediated cerebellar ataxia. The autoantibodies involved in ACA can be detected in patients and are important biomarkers. In this study, we aimed to identify a novel autoantibody in patients with ACA. We screened for autoantibodies in serum samples from patients with cerebellar ataxia using immunohistochemical and immunocytochemical assays. Immunoprecipitation and mass spectrometry were used to identify the target antigens of the detected autoantibodies. We found that IgG reacted with transmembrane protein family 132 A (TMEM132A), which is enriched in the central nervous system (CNS), in serum samples of patients with cerebellar ataxia. A cell-based binding assay (CBA) was used to identify TMEM132A-IgG in serum samples obtained from 436 patients with cerebellar ataxia requiring differential diagnosis for ACA, 62 patients with autoimmune encephalitis, 27 patients with multiple system atrophy with predominant cerebellar ataxia, 24 patients with multiple sclerosis, 17 patients with neuromyelitis optica spectrum disorders, 17 patients with Parkinson's disease, 7 patients with anti-myelin oligodendrocyte glycoprotein antibody-associated disease, and 14 healthy subjects. We detected TMEM132A-IgG in serum samples from two patients with cerebellar ataxia, including the patient whose study first led to the identification of this autoantibody. None of the other participants had these autoantibodies. The two autoantibody-positive patients showed progressive predominant cerebellar ataxia with pyramidal tract signs and albuminocytologic dissociation. Brain MRI findings indicated cerebellar atrophy in one patient and bilateral inferior olivary nuclei hyperintensity changes and pseudohypertrophy in the other patient. TMEM132 A-IgG may be a novel autoantibody associated with autoimmune CNS diseases, including ACA.</div></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"413 ","pages":"Article 578867"},"PeriodicalIF":2.5,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146018859","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Neuroinflammation and metabolic dysfunction drive nerve conduction deficits in diabetic neuropathy: Clinical and in silico insights","authors":"Faaz Bin Razi ,&nbsp;Sangeeta Singhal ,&nbsp;Hamid Ashraf ,&nbsp;Shagufta Moin","doi":"10.1016/j.jneuroim.2025.578849","DOIUrl":"10.1016/j.jneuroim.2025.578849","url":null,"abstract":"<div><div>Diabetic neuropathy is a debilitating complication of type 2 diabetes mellitus (T2DM), largely driven by systemic inflammation and metabolic disturbances. This cross-sectional study assessed the relationship between interleukin-6 (IL-6), vitamin B12, lipid profile, and nerve conduction parameters in T2DM patients with and without neuropathy. Sixty participants were divided evenly into two groups: T2DM without neuropathy (control) and T2DM with confirmed neuropathy (case). Biochemical parameters measured included fasting and postprandial glucose, glycated hemoglobin (HbA1c), vitamin B12, IL-6, renal function, and detailed lipid profiling. Nerve conduction studies assessed sensory and motor nerves in upper and lower limbs. The neuropathy group showed higher IL-6 levels, lower vitamin B12 concentrations, and an atherogenic lipid profile characterised by increased triglycerides, VLDL, and non-HDL cholesterol. Marked reductions in nerve conduction velocities were evident in both sensory and motor nerves, especially in the common peroneal and posterior tibial nerves, which had high diagnostic accuracy with AUC values of 0.88 and 0.84, respectively. Univariate regression identified IL-6 as significantly associated with decreased conduction velocity in radial sensory and posterior tibial nerves. Supporting these clinical findings, in silico pathway enrichment analyses highlighted key roles for IL-6 signalling, vitamin B12 metabolism, lipid pathways, and myelination in diabetic neuropathy pathogenesis. These integrated data underscore inflammation and metabolic dysfunction as pivotal drivers, with IL-6 and nerve conduction studies serving as promising early biomarkers and therapeutic targets.</div></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"412 ","pages":"Article 578849"},"PeriodicalIF":2.5,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145849488","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The activation of classical and/or lectin complement pathway for the pathogenesis of chronic inflammatory demyelinating polyradiculoneuropathy","authors":"Keishu Murakami ,&nbsp;Hiroshi Tsujimoto ,&nbsp;Yoshiaki Nakayama ,&nbsp;Hiroki Takeuchi ,&nbsp;Kenji Yamamoto ,&nbsp;Kei-Ichi Katayama ,&nbsp;Yusuke Inada ,&nbsp;Yutaka Inaba ,&nbsp;Masatoshi Jinnin ,&nbsp;Nobuyuki Oka ,&nbsp;Norimitsu Inoue ,&nbsp;Katsuichi Miyamoto","doi":"10.1016/j.jneuroim.2025.578848","DOIUrl":"10.1016/j.jneuroim.2025.578848","url":null,"abstract":"<div><h3>Background</h3><div>Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a heterogeneous immune-mediated neuropathy. The activation of the complement system has been implicated in CIDP, but it remains unclear which complement pathway activation is essential for the pathogenesis of CIDP. We therefore examined the deposition and production of several activated complement factors in tissues and serum of CIDP, respectively.</div></div><div><h3>Methods</h3><div>We immunohistochemically stained C4d, C3c, and C5b-9 using sural nerve specimens of CIDP and controls. Images were evaluated for the density and fluorescence intensity of complement factor deposition. Serum complement factors (factor H, Ba, sC5b-9, C3, C4 and CH50) were measured in CIDP and in controls. We also investigated clinical data in relation to complement deposition and serum complement factors.</div></div><div><h3>Results</h3><div>C4d was deposited on the myelin sheaths in all patients with CIDP, with significantly higher density and intensity than in controls. C3c was detected in three of the nine patients with CIDP, while C5b-9 was not detected. Serum levels of sC5b-9 were higher than the normal range in some patients, but no significant differences in serum complement levels were observed between those with CIDP and controls. The C4d density and serum C3 levels were positively correlated with Hughes functional grading scale scores.</div></div><div><h3>Conclusions</h3><div>C4d deposition was observed in all patients with CIDP. Few patients showed the activated terminal pathway. The classical and/or lectin pathways are predominantly involved in CIDP pathogenesis. Sural nerve pathology and activated complement factors in the plasma may be useful for selecting therapeutic agents including C2 inhibitors.</div></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"412 ","pages":"Article 578848"},"PeriodicalIF":2.5,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145839090","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The potential role of S-nitrosoglutathione reductase in astrocyte BAFF expression and B cell activation in experimental autoimmune encephalomyelitis","authors":"Jeseong Won ,&nbsp;Judong Kim ,&nbsp;S.M. Touhidul Islam ,&nbsp;Fei Qiao ,&nbsp;Avtar K. Singh ,&nbsp;Inderjit Singh","doi":"10.1016/j.jneuroim.2025.578847","DOIUrl":"10.1016/j.jneuroim.2025.578847","url":null,"abstract":"<div><div>B cell-activating factor (BAFF) is a key mediator of B cell-driven autoimmune pathology. In mice with experimental autoimmune encephalomyelitis (EAE), a widely used model of multiple sclerosis (MS), we observed a pronounced accumulation of B cells within the spinal cord, alongside elevated BAFF expression, particularly in GFAP⁺ activated astrocytes located in the subpial-region. This upregulation of BAFF coincided with the infiltration of CD4⁺ T cells, notably Th1 cells producing IFNγ, a cytokine critical for inducing BAFF gene expression in astrocytes. Concurrently, elevated BAFF levels in the spinal cords of EAE mice were associated with increased expression of S-nitrosoglutathione (GSNO) reductase (GSNOR), the enzyme that degrades GSNO, a molecule known for its anti-inflammatory properties. Pharmacological inhibition of GSNOR using N6022, a reversible inhibitor, significantly reduced BAFF expression and decreased B cell accumulation in the CNS. In vitro, both GSNO and N6022 suppressed IFNγ- and TNFα-induced BAFF expression in cultured astrocytes by inhibiting STAT1 and NF-κB activation. Furthermore, co-culture of B cells with IFNγ/TNFα-stimulated astrocytes, or exposure to their conditioned media, resulted in an increased number of B cells and enhanced IL-6 production. These effects were attenuated either by pretreating astrocytes with GSNO or by applying BAFF-neutralizing antibodies to B cells. Collectively, these findings suggest that GSNOR modulates astrocytic BAFF expression, thereby influencing B cell activation and their IL-6-mediated functions in EAE. Modulation of this pathway may represent a promising avenue for future investigation into immune regulation in MS and related autoimmune conditions.</div></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"412 ","pages":"Article 578847"},"PeriodicalIF":2.5,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145882028","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Immune profiling unveils the systemic cytokine milieu associated with acute reversible encephalopathy with cytotoxic lesions of the corpus callosum (CLOCCs)","authors":"Silvia Sperandei ,&nbsp;Lorenzo Gaetani ,&nbsp;Giorgia Manni ,&nbsp;Marco Gargaro ,&nbsp;Giuseppe Vittorio De Socio ,&nbsp;Maria Cristina Gallina ,&nbsp;Andrea Fiacca ,&nbsp;Cinzia Costa ,&nbsp;Andrea Mancini ,&nbsp;Lucilla Parnetti ,&nbsp;Francesca Fallarino ,&nbsp;Massimiliano Di Filippo","doi":"10.1016/j.jneuroim.2025.578834","DOIUrl":"10.1016/j.jneuroim.2025.578834","url":null,"abstract":"<div><div>Encephalopathy with cytotoxic lesions of the corpus callosum (CLOCCs) represents a clinical-radiological entity characterized by MRI evidence of lesions involving the splenium of the corpus callosum and a collection of neurological symptoms ranging from consciousness impairment to seizures and focal neurological signs. The most widely accepted pathophysiological mechanism underlying CLOCCs is thought to be represented by a phenomenon of cytokine-induced cytotoxic edema but its exact immune pathogenesis is still unravelled and targeted treatments are lacking.</div><div>Here, we report the case of a 18-year-old male with CLOCCs associated with acute Epstein-Barr virus (EBV) infection, in whom the systemic immune response across acute and post-acute phases was characterized for the first time through longitudinal cytokine profiling. The obtained data pave the way to a more precise comprehension of CLOCCs pathogenesis and an individualized treatment of this potentially severe condition.</div></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"412 ","pages":"Article 578834"},"PeriodicalIF":2.5,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145771843","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Immature ovarian teratomas in anti-NMDA receptor encephalitis","authors":"Kristen Simone ,&nbsp;Nabeela Nathoo ,&nbsp;Allyssa Hooper ,&nbsp;Albert Aboseif ,&nbsp;Andrew Blake Buletko ,&nbsp;Niranjala Satkunam ,&nbsp;Jennifer A. McCombe ,&nbsp;Sophia Pin","doi":"10.1016/j.jneuroim.2025.578850","DOIUrl":"10.1016/j.jneuroim.2025.578850","url":null,"abstract":"<div><div>Anti-<em>N</em>-methyl-<span>d</span>-aspartate receptor autoimmune encephalitis (NMDAR-AIE) is an immune-mediated neurological disease with manifestations including altered mental status, neuropsychiatric disturbance, movement disorders, seizure, and dysautonomia. NMDAR-AIE is associated with ovarian teratoma in half of cases, typically mature teratoma. NMDAR-AIE is rarely associated with immature ovarian teratomas. Here, we describe three cases of NMDAR-AIE associated with immature ovarian teratomas. We review diagnosis and management of immature ovarian teratomas in NMDAR-AIE as a collaboration between gynecology and neurology, with multi-disciplinary recommendations provided regarding workup and management.</div></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"412 ","pages":"Article 578850"},"PeriodicalIF":2.5,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145849394","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Mitochondrial dysregulation as a central mechanism in autism spectrum disorder pathogenesis","authors":"Xiaoli Liao ,&nbsp;Jingqi Shao ,&nbsp;Zhihui Chen","doi":"10.1016/j.jneuroim.2025.578851","DOIUrl":"10.1016/j.jneuroim.2025.578851","url":null,"abstract":"<div><div>Autism spectrum disorder (ASD) is a complex neurodevelopmental disorder influenced by both genetic predispositions and environmental insults. However, the precise molecular mechanisms linking prenatal environmental perturbations to neurodevelopmental impairments remain poorly defined. This study investigates the role of mitochondrial dysfunction and metabolic disturbances in ASD pathogenesis using various preclinical models, including the maternal immune activation (MIA) and ASD high-risk gene knockout models. We performed transcriptomic profiling on mouse brain tissues to identify differentially expressed genes (DEGs) associated with mitochondrial and metabolic pathways. Functional enrichment analyses, including Gene Ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG), revealed significant disruptions in pathways such as oxidative phosphorylation, tricarboxylic acid, and energy metabolism. These findings point to mitochondrial dysfunction as a central mechanism contributing to metabolic imbalances in ASD. Comparative analysis with publicly available RNA-seq datasets from PTEN knockout model revealed both shared and unique metabolic signatures. Single-cell RNA-seq data from the MIA model further identified cell-type-specific metabolic alterations in distinct neuronal and glial populations. Additionally, analysis of the Human Fetal Single-Cell Atlas highlighted the relevance of these metabolic pathways in human brain development. Collectively, these results emphasize mitochondrial metabolism as a potential therapeutic target for ASD, offering insights into the molecular basis of this disorder.</div></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"412 ","pages":"Article 578851"},"PeriodicalIF":2.5,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145912017","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Efficacy and tolerability of metformin as an adjuvant therapy in patients with relapse-remitting multiple sclerosis receiving interferon Beta 1a: A randomized pilot trial","authors":"Mohamed Y. Abdelgaied ,&nbsp;Omar Abdelgawad ,&nbsp;Mohamed Hamed Rashad ,&nbsp;Mohamed H. Solayman ,&nbsp;Hend M. El-Tayebi","doi":"10.1016/j.jneuroim.2025.578852","DOIUrl":"10.1016/j.jneuroim.2025.578852","url":null,"abstract":"<div><h3>Background</h3><div>Neurodegeneration and inflammation can accelerate the demyelination process in multiple sclerosis (MS). We aimed to investigate the efficacy and tolerability of metformin as an add-on treatment to interferon Beta 1a (IFNβ-1a) in Egyptian patients with relapsing-remitting multiple sclerosis (RRMS).</div></div><div><h3>Method</h3><div>Thirty RRMS patients were divided into two groups: the experimental arm receiving IFNβ-1a plus 2 g of metformin once daily and the control arm receiving IFNβ-1a alone. Tolerance to metformin was measured for the intervention group. Following 6 months period, serum neurofilament light chain (sNFL) and nuclear factor Kappa B (NF-κB), T2 lesions in magnetic resonance imaging (MRI), and expanded disability status scale (EDSS) were measured.</div></div><div><h3>Results</h3><div>There were no statistically significant differences between the two groups in the change in the median (interquartile range) of the blood biomarkers (sNFL; −32.8 (21) vs −32.8 (13.4), <em>p</em> = 0.99) and NF-κB; −64.9 (35.1) vs −61.6 (35.7), <em>p</em> = 0.8, respectively). In clinical outcomes, there was no statistically significant in the mean (standard deviation) change of EDSS (0 vs 0, <em>p</em> = 1)<strong>.</strong> For MRI results, 11 patients had a stationary and regressive course 1 patient had a progressive course in the metformin group vs 6 patients had a stationary and regressive course and 2 had a progressive course in the control group, <em>p</em> = 0.23. All outcomes were measured after 6-month follow-ups. The most common side effects of metformin were diarrhea and abdominal pain without incidence of lactic acidosis.</div></div><div><h3>Conclusion</h3><div>Receiving metformin as an add-on therapy to IFNβ-1a did not result in a significant improvement in neurodegeneration and inflammation blood biomarkers and clinical outcomes. A high dose of metformin (2 g/day) is safe and well tolerated in patients with MS. Additional studies involving larger populations are necessary to confirm or disprove these findings.</div></div><div><h3>Trial registration</h3><div><span><span>ClinicalTrials.gov</span><svg><path></path></svg></span> ID: <span><span>NCT06812585</span><svg><path></path></svg></span></div></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"412 ","pages":"Article 578852"},"PeriodicalIF":2.5,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145882029","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A case of Homer-3 IgG cerebellar ataxia & literature review of 15 reported cases","authors":"Diana Anissian ,&nbsp;Divyanshu Dubey ,&nbsp;Nisa Vorasoot ,&nbsp;Ramona Miske ,&nbsp;Anastasia Zekeridou ,&nbsp;Andrew McKeon","doi":"10.1016/j.jneuroim.2025.578846","DOIUrl":"10.1016/j.jneuroim.2025.578846","url":null,"abstract":"<div><div>Homer-3-IgG autoimmunity is a rare diagnosis typically presenting with subacute cerebellar ataxia. Here, we report a patient presenting insidiously with cerebellar ataxia and mild cognitive impairment, referred to the Mayo Clinic for a second opinion of her initial diagnosis of parkinsonism. Considering autoimmune cerebellar ataxia as the correct diagnosis, the patient's cerebrospinal fluid (CSF) and serum samples were sent for Movement Disorders Autoimmune/Paraneoplastic Evaluation neural antibody testing. Murine tissue-based indirect immunofluorescence assay of serum and CSF revealed a unique cerebellar molecular layer-predominant ‘medusa head’ staining pattern. Protein microarray disclosed Homer-3 as high ranking hits. Serological diagnosis was confirmed by Homer-3-specific line blot assay. The patient was treated with a 12-week regimen of intravenous methylprednisolone. She had limited improvement, though at 6-month follow-up she had not progressed further off treatment. This patient is the third detected case with Homer-3-IgG in the Neuroimmunology laboratory of the Mayo Clinic, and the only one clinically evaluated at Mayo Clinic. Histories for the two other cases were unavailable. This case adds to the limited number of reports on Homer-3-IgG autoimmunity, with our literature review identifying 15 other cases reported to date. Neurological presentations include cerebellar ataxia (15/16), cognitive impairment (4/16), REM sleep behavior disorder (2/16), seizures (2/16), myeloradiculopathy (1/16), radiculoneuropathy (1/16), and psychosis (1/16). The most common treatment used was corticosteroids (15/16), followed by IVIg (7/16), mycophenolate mofetil (MMF) (4/16), plasma exchange (3/16), and rituximab (1/16). Reported outcomes have varied, with partial improvement being most common (7/15).</div></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"412 ","pages":"Article 578846"},"PeriodicalIF":2.5,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145839091","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}