Ten children with IgG-associated primary diffuse mesangial proliferative glomerulonephritis are presented. All cases showed diffuse global IgG deposits in the mesangium. IgG was the sole immunoglobulin deposited in the mesangium in 8 patients and was the predominant immunoglobulin in 2. C3 was also present in 8 patients. Light microscopy revealed variable diffuse and global mesangial proliferation in all cases, with additional focal segmental glomerular sclerosis in one. On electron microscopy, electron-dense deposits in the mesangium were identified in all patients. Clinically, 4 patients presented with the steroid-resistant nephrotic syndrome, and 6 had proteinuria and hematuria. At the latest follow-up, renal function was normal in all patients, 6 showed clinical remission and 4 had slight proteinuria and/or hematuria. These data suggest that there is a clinicopathologic entity, IgG-associated glomerulonephritis, characterized by diffuse mesangial proliferation, predominant mesangial IgG deposition, variable clinical presentation, and a relatively benign course.
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J Neurol
November 2024
Department of Neurology, St. Josef-Hospital, Ruhr-University Bochum, Gudrunstr. 56, 44791, Bochum, Germany.
Background: Optic neuritis (ON) is a common manifestation of multiple sclerosis (MS) and myelin-oligodendrocyte-glycoprotein IgG-associated disease (MOGAD). This study evaluated the applicability of optical coherence tomography (OCT) for differentiating between both diseases in two independent cohorts.
Methods: One hundred sixty two patients from seven sites underwent standard OCT and high-contrast visual acuity (HCVA) testing at least 6 months after first ON.
Neurol Neuroimmunol Neuroinflamm
September 2024
From the Department of Neurology (K.K., H.K., Y.M., N.S., N. Yamazaki, N. Yamamoto, S.U., C.N., H.O., Y.T., T.T., K.F., T.M., M.A.), Tohoku University Graduate School of Medicine, Sendai, Japan; Multiple Sclerosis and Neuromyelitis Optica Center (H.K., K.F.), Southern TOHOKU Research Institute for Neuroscience, Koriyama; Department of Multiple Sclerosis Therapeutics (H.K., K.F.), Fukushima Medical University, Fukushima, Japan; Department of Neurology (T.T.), NHO Yonezawa National Hospital, Yamagata, Japan; Division of Neurology (J.F., I.N.), Tohoku Medical and Pharmaceutical University, Sendai, Japan; Department of Neurology (Y.H.), Japanese Redcross Maebashi Hospital; Department of Neurology (Y.H.), Mihara Memorial Hospital, Isesaki, Japan; and Center for Brain Research (H.L.), Medical University of Vienna, Austria.
Objectives: In myelin oligodendrocyte glycoprotein IgG-associated disease (MOGAD) and aquaporin-4 IgG+ neuromyelitis optica spectrum disorder (AQP4+NMOSD), the autoantibodies are mainly composed of IgG1, and complement-dependent cytotoxicity is a primary pathomechanism in AQP4+NMOSD. We aimed to evaluate the CSF complement activation in MOGAD.
Methods: CSF-C3a, CSF-C4a, CSF-C5a, and CSF-C5b-9 levels during the acute phase before treatment in patients with MOGAD (n = 12), AQP4+NMOSD (n = 11), multiple sclerosis (MS) (n = 5), and noninflammatory neurologic disease (n = 2) were measured.
Int J Mol Sci
July 2024
Department of Immunology, NSW Health Pathology-ICPMR, Westmead Hospital, Sydney, NSW 2145, Australia.
Anti-IgLON5 (IgLON5-IgG)-associated disease is a newly defined clinical entity. This literature review aims to evaluate its pathogenesis, which remains a pivotal question. Features that favour a primary neurodegenerative mechanism include the non-inflammatory tauopathy neuropathological signature and overrepresentation of microtubule-associated protein tau () H1/H1 genotype as seen in other sporadic tauopathies.
View Article and Find Full Text PDFHeliyon
June 2024
Department of Neurology, Beijing Tongren Hospital, Capital Medical University, Beijing, 100176, China.
J Pers Med
June 2024
Syreon Research Institute, 1142 Budapest, Hungary.
Primary demyelinating disorders of the central nervous system (CNS) include multiple sclerosis and the orphan conditions neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein IgG-associated disease (MOGAD). Curative technologies under development aim to selectively block autoimmune reactions against specific autoantigens while preserving the responsiveness of the immune system to other antigens. Our analysis focused on target patient selection for such developments, carefully considering the relevant clinical, regulatory, and market-related aspects.
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