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Survey of Ophthalmology 2020A 51-year-old woman with a previous history of rheumatoid arthritis experienced painless progressive visual loss in the left eye for 3 weeks. Fundus examination revealed...
A 51-year-old woman with a previous history of rheumatoid arthritis experienced painless progressive visual loss in the left eye for 3 weeks. Fundus examination revealed optic disk pallor in her left eye. Magnetic resonance imaging of the brain and orbits showed enhancement of the pachymeninges and hypersignal at the left optic nerve. Meningeal biopsy was performed. Immunohistochemical staining for IgG4 revealed several IgG4-positive plasma cells, which in some areas reached the number of 50 cells/high-power field. In this case, the clinical and histopathological features of the patient met diagnostic criteria for rheumatoid arthritis and IgG4-related disease, respectively. Rheumatoid arthritis sometimes occurs with abundant IgG4 plasma cells and fulfills the histological diagnostic criteria for IgG4-related disease. This case demonstrates that overlapping features of IgG4-RD and rheumatoid arthritis may present in a single patient.
Topics: Arthritis, Rheumatoid; Biopsy; Brain; Diagnosis, Differential; Female; Humans; Magnetic Resonance Imaging; Meningitis; Middle Aged; Optic Nerve; Optic Nerve Diseases; Tomography, Optical Coherence
PubMed: 30521818
DOI: 10.1016/j.survophthal.2018.11.004 -
Nature Neuroscience Jul 2022The meninges, comprising the leptomeninges (pia and arachnoid layers) and the pachymeninx (dura layer), participate in central nervous system (CNS) autoimmunity, but...
The meninges, comprising the leptomeninges (pia and arachnoid layers) and the pachymeninx (dura layer), participate in central nervous system (CNS) autoimmunity, but their relative contributions remain unclear. Here we report on findings in animal models of CNS autoimmunity and in patients with multiple sclerosis, where, in acute and chronic disease, the leptomeninges were highly inflamed and showed structural changes, while the dura mater was only marginally affected. Although dural vessels were leakier than leptomeningeal vessels, effector T cells adhered more weakly to the dural endothelium. Furthermore, local antigen-presenting cells presented myelin and neuronal autoantigens less efficiently, and the activation of autoreactive T cells was lower in dural than leptomeningeal layers, preventing local inflammatory processes. Direct antigen application was required to evoke a local inflammatory response in the dura. Together, our data demonstrate an uneven involvement of the meningeal layers in CNS autoimmunity, in which effector T cell trafficking and activation are functionally confined to the leptomeninges, while the dura remains largely excluded from CNS autoimmune processes.
Topics: Animals; Arachnoid; Autoimmunity; Central Nervous System; Dura Mater; Humans; Meninges; Multiple Sclerosis
PubMed: 35773544
DOI: 10.1038/s41593-022-01108-3 -
Lancet (London, England) Apr 2020
Topics: Humans; Meningitis; Meningococcal Vaccines
PubMed: 32305081
DOI: 10.1016/S0140-6736(20)30865-5 -
Journal of Neuroinflammation May 2023The meninges, membranes surrounding the central nervous system (CNS) boundary, harbor a diverse array of immunocompetent immune cells, and therefore, serve as an... (Review)
Review
The meninges, membranes surrounding the central nervous system (CNS) boundary, harbor a diverse array of immunocompetent immune cells, and therefore, serve as an immunologically active site. Meningeal immunity has emerged as a key factor in modulating proper brain function and social behavior, performing constant immune surveillance of the CNS, and participating in several neurological diseases. However, it remains to be determined how meningeal immunity contributes to CNS physiology and pathophysiology. With the advances in single-cell omics, new approaches, such as single-cell technologies, unveiled the details of cellular and molecular mechanisms underlying meningeal immunity in CNS homeostasis and dysfunction. These new findings contradict some previous dogmas and shed new light on new possible therapeutic targets. In this review, we focus on the complicated multi-components, powerful meningeal immunosurveillance capability, and its crucial involvement in physiological and neuropathological conditions, as recently revealed by single-cell technologies.
Topics: Humans; Meninges; Central Nervous System; Nervous System Diseases
PubMed: 37231449
DOI: 10.1186/s12974-023-02803-z -
Medicina Clinica Dec 2021
Topics: Gemella; Gram-Positive Bacterial Infections; Humans; Meningitis
PubMed: 33972101
DOI: 10.1016/j.medcli.2021.02.018 -
Paediatrics and International Child... Nov 2021
Topics: Child; Humans; Meningitis; Tuberculosis, Meningeal
PubMed: 34783305
DOI: 10.1080/20469047.2021.1952818 -
Neurological Sciences : Official... Oct 2023
Topics: Humans; Meningitis; Meningitis, Aseptic; Syndrome
PubMed: 37219645
DOI: 10.1007/s10072-023-06872-z -
Immune-Mediated Hypertrophic Pachymeningitis and its Mimickers: Magnetic Resonance Imaging Findings.Academic Radiology Nov 2023Hypertrophic pachymeningitis (HP) is a rare and chronic inflammatory disorder presenting as localized or diffuse thickening of the dura mater. It can be idiopathic or an... (Review)
Review
Hypertrophic pachymeningitis (HP) is a rare and chronic inflammatory disorder presenting as localized or diffuse thickening of the dura mater. It can be idiopathic or an unusual manifestation of immune-mediated, infectious, and neoplastic conditions. Although some cases may remain asymptomatic, HP can lead to progressive headaches, cranial nerve palsies, hydrocephalus, and other neurological complications, which makes its recognition a fundamental step for prompt treatment. Regarding the diagnosis workup, enhanced MRI is the most useful imaging method to evaluate dural thickening. This article addresses the MR imaging patterns of immune-mediated HP, including immunoglobulin G4-related disease, neurosarcoidosis, granulomatosis with polyangiitis, rheumatoid pachymeningitis, and idiopathic HP. The main infectious and neoplastic mimicking entities are also discussed with reference to conventional and advanced MR sequences.
Topics: Humans; Diagnosis, Differential; Dura Mater; Hypertrophy; Magnetic Resonance Imaging; Meningitis
PubMed: 36882352
DOI: 10.1016/j.acra.2023.01.017 -
Development (Cambridge, England) Oct 2023Perivascular fibroblasts (PVFs) are a fibroblast-like cell type that reside on large-diameter blood vessels in the adult meninges and central nervous system (CNS). PVFs...
Perivascular fibroblasts (PVFs) are a fibroblast-like cell type that reside on large-diameter blood vessels in the adult meninges and central nervous system (CNS). PVFs contribute to fibrosis following injury but their homeostatic functions are not defined. PVFs were previously shown to be absent from most brain regions at birth and are only detected postnatally within the cerebral cortex. However, the origin, timing and cellular mechanisms of PVF development are not known. We used Col1a1-GFP and Col1a2-CreERT2 transgenic mice to track PVF development postnatally. Using lineage tracing and in vivo imaging we show that brain PVFs originate from the meninges and are first seen on parenchymal cerebrovasculature at postnatal day (P) 5. After P5, PVF coverage of the cerebrovasculature expands via local cell proliferation and migration from the meninges. Finally, we show that PVFs and perivascular macrophages develop concurrently. These findings provide the first complete timeline for PVF development in the brain, enabling future work into how PVF development is coordinated with cell types and structures in and around the perivascular spaces to support normal CNS vascular function.
Topics: Animals; Meninges; Fibroblasts; Mice, Transgenic; Mice; Cerebral Cortex; Cell Movement; Cell Proliferation; Macrophages; Brain
PubMed: 37756588
DOI: 10.1242/dev.201805 -
Current Opinion in Immunology Jun 2022The meninges encase the brain and spinal cord and house a variety of immune cells, including developing and mature B cells, and antibody-secreting plasma cells. In... (Review)
Review
The meninges encase the brain and spinal cord and house a variety of immune cells, including developing and mature B cells, and antibody-secreting plasma cells. In homeostasis, these cells localize around the dural venous sinuses, providing a defense 'zone' to protect the brain and spinal cord from blood-borne pathogens. Dural plasma cells predominantly secrete IgA antibodies, and some originate from the gastrointestinal tract, with the number and antibody isotype shaped by the gut microbiome. For developing B cells arriving from the adjacent bone marrow, the dura provides a site to tolerize against central nervous system antigens. In this review, we will discuss our current understanding of meningeal humoral immunity in homeostasis.
Topics: Brain; Dura Mater; Homeostasis; Humans; Immunity, Humoral; Meninges
PubMed: 35569417
DOI: 10.1016/j.coi.2022.102188