Figure 4 Neuropathology of MOG and AQP4 antibody–associated demyelinating lesions in the brain The biopsy specimen revealed a small actively demyelinating.

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Patrick Van Der Voorn, Janneke Tekstra, Rob H. J. Beelen, Cornelis P

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Figure Model contrasting the potential role of antibodies to myelin oligodendrocyte glycoprotein (MOG) or aquaporin-4 (AQP4) in opticospinal inflammationMOG-specific.

Figure 3 Brain MRI findings in patients with MOG-Ab Extensive brain lesions with large diameter (A and B), posterior reversible encephalopathy–like lesions.

Figure 1 Muscle biopsy from a patient with a slowly progressive (24 years) HMGCR antibody–associated myopathy syndrome (A) Hematoxylin & eosin stain, (B)

Histopathologic features (all images at ×10 magnification).

Figure 2 Orbital MRI findings One-third of myelin oligodendrocyte glycoprotein antibody–positive patients revealed extensive enhancement patterns that.

Figure Vertebral artery angiogram and tissue pathology

Figure 3 Antibodies to MOG using different secondary antibodies: Anti-human IgG (H + L), IgG1, or IgM(A) Comparison of binding to full-length myelin oligodendrocyte.

Figure Neuroimaging and pathology

Figure 3 Immunohistochemical analyses of positive and negative Epstein-Barr virus (EBV) control tissues using immunostaining Immunohistochemical analyses.

Figure 1 Percent positivity by clinical feature Overall, 6

Figure 2 Temporal distribution of MOG antibody in serum of 2 relapsing patients with demyelinating diseases Temporal distribution of MOG antibody in serum.

Figure 1 Treg percentage and suppressive function increased during each round of Treg infusions Treg percentage and suppressive function increased during.

Figure 2 Immunopathologic analysis of all 3 Rasmussen encephalitis cases Immunopathologic analysis of all 3 Rasmussen encephalitis cases (A) Perivascular.

Figure Muscle biopsy of the left biceps showing the characteristic pathologic findings in BCIM Muscle biopsy of the left biceps showing the characteristic.

Figure 1 Histopathologic features of a chronic active and a chronic plaque in the MS brain Histopathologic features of a chronic active and a chronic plaque.

Figure 1 Muscle biopsy images demonstrating a pauci-immune necrotizing autoimmune myopathy in illustrative cases 1 and 2 (A–D) Case 1 deltoid muscle. Muscle.

Figure Clinical presentation of daclizumab side effects with skin rash and meningoencephalomyelitis Clinical presentation of daclizumab side effects with.

Figure 1 Flow diagram of the assays and the samples that were evaluatedA total of 1,109 samples were initially screened at a serum dilution of 1:20 for.

Figure Brain MRI and biopsy specimens from the pontine lesion

Figure 2 Brain-infiltrating immune cells mainly consist of CD8+ memory T cells Immunofluorescence staining of brain-infiltrating immune cells. Brain-infiltrating.

Figure 1 Integrative model of NMO/SD pathogenesis

Figure 2 Brain biopsy Brain biopsy (A) Double staining with anti-aquaporin-4 (AQP4) antibody (dark green) and Luxol fast blue (blue) is shown. Loss of.

Figure Nuclear Nrf2 expression after fumarate therapy A new left occipital fluid-attenuated inversion recovery hyperintense (A), T1 hypointense (B), and.

Figure 1 Histopathologic features of case 1 (A–G) and case 2 (H–L)‏

Figure 2 Neuropathologic examination for lymphoma etiology (patients 1–4)‏ Neuropathologic examination for lymphoma etiology (patients 1–4) Immunohistochemical.

Figure 1 Cerebral MRI during the disease course Cerebral MRI with multiple cerebral supratentorial lesions during the disease course: periventricular lesions.

Figure 5 Increased frequency of parenchymal CD138- and LMP-1–positive cells in MS Increased frequency of parenchymal CD138- and LMP-1–positive cells in.

Figure 4 Comparison of 7.0T and 3.0T MRI (patients 5 and 6)‏

Figure 1 Neuropathologic examination of brain areas with normal MRI appearance and with gadolinium enhancement (patient 1)‏ Neuropathologic examination.

Figure 2 Neuropathology of PML lesions in a patient with MS treated with fingolimod Neuropathology of PML lesions in a patient with MS treated with fingolimod.

Figure 2 APCs from laquinimod-treated mice inhibit differentiation of Tfh cells APCs from laquinimod-treated mice inhibit differentiation of Tfh cells.

Figure Four months after symptom onset(A) Two fluorodeoxyglucose positive left inguinal lymph nodes. Four months after symptom onset(A) Two fluorodeoxyglucose.

Figure 2 Histochemical and immunohistochemical staining and electron microscopic examination of structures in the brain biopsy Hematoxylin & eosin staining.

Figure 2 Histopathologic findings of patients with both inflammatory myopathy and myasthenia gravis Histopathologic findings of patients with both inflammatory.

Figure 2 Overview of the patient's history and immunofluorescence pattern of patient CSF IgG Overview of the patient's history and immunofluorescence pattern.

Diseases Involving Myelin

Figure MRI and neuropathologic characteristics of the tumefactive demyelinating lesion in our patient MRI and neuropathologic characteristics of the tumefactive.

Figure MRI and histology of demyelinating lesion(A) Symmetric T2 hyperintensity in the midbrain with relative sparing of cerebral peduncles. MRI and histology.

Figure 3. Time curves for GFAP, S100B, and tTau release in CSF

Figure Radiologic and pathologic findings Fluid-attenuated inversion recovery (FLAIR) sequence with a single large T2-hyperintense signal involving the.

Figure 2 Representative brain MRIs from patients with neuromyelitis optica Lesions are localized at sites of high aquaporin-4 expression (white dots).

Figure 2 CD4+ and CD8+ T cells accumulate in the CSF in GABAB receptor antibody–associated LE CD4+ and CD8+ T cells accumulate in the CSF in GABAB receptor.

Figure 1 Schematic overview of flow cytometry Schematic overview on the analysis of peripheral immune cells by flow cytometry. Schematic overview of flow.

Figure 4 Pattern of relapse in patients with MOG-Ab Five myelin oligodendrocyte glycoprotein antibody (MOG-Ab)–positive patients experienced a relapse,

Figure 2 Cerebral and spinal MRI (A) Restricted diffusion of both optic nerves (arrows) on diffusion-weighted and apparent diffusion coefficient imaging.

Figure 2. Neuropathologic diagnosis of Creutzfeldt-Jakob disease (CJD) at postmortem Neuropathologic diagnosis of Creutzfeldt-Jakob disease (CJD) at postmortem.

Figure 4 Aquaporin-4 immunoglobulin G (AQP4-IgG) index in time-matched paired serum-CSF specimens: 3 attack/preattack pairs and 7 bridge/remission pairs.

Figure 2 Pathologic diagnosis of CAA-related vascular inflammation Hematoxylin & eosin staining (A) revealed focal intramural inflammation including lymphocytes,

Figure 4 Confirmatory cohorts to assess MOG-IgG1 assay(A) All 81 aquaporin-4 (AQP4)- seropositive patients (blue) from the Oxford National neuromyelitis.

Figure 1 Annual trend in specimen type submitted as first sample for aquaporin-4 immunoglobulin G testing (serum only vs CSF only vs both) from 101,065.

Figure 1 B cells and plasma cells accumulate in the CSF in GABAB receptor antibody–associated LE B cells and plasma cells accumulate in the CSF in GABAB.

Figure 4 Autopsy immunochemistry results

Figure Disease course and neuropathology of CASPR2 encephalitis(A) Summary of the most important changes during the disease course. Disease course and.

Figure Overview of patients with demyelinating diseases, presence of clinical symptoms frequently associated with NMDAR encephalitis, and antibody status.

Figure 2 Histopathological features in CIDP

Figure 1 Full-length MOG cell-based assay using a serum dilution of 1:160 as a cutoff for positivity (red line in both plots)(A) Myelin olidgodendrocyte.

Luxol fast blue–stained section from the brain slice imaged in figure 1. Luxol fast blue–stained section from the brain slice imaged in figure 1. The outlined.

Figure MRI brain comparison prior and after treatment and brain biopsy findings MRI brain comparison prior and after treatment and brain biopsy findings.

Figure 1 Peripheral blood lymphocyte counts during dose titrationB-lymphocyte (CD19+; A) and total lymphocyte (CD45+; B) counts (cells/µL) in peripheral.

Figure 2 Brain biopsy of 2 patients with anti-MOG encephalitis initially misdiagnosed with small vessel CNS vasculitis Brain biopsy of 2 patients with.

Figure 3 Muscle biopsy showing myofiber atrophy and degeneration

Figure 2 B-cell very late antigen-4 (VLA-4) deficiency reduced CNS accumulation of B cells, but not proinflammatory or regulatory T cells (Treg), in myelin.

Figure 2 Detection of atypical anti-neuronal antibodies Immunohistofluorescence assay on rat brain sagittal slices incubated with the patient's CSF and.

Figure 6 P2Y12 is highly expressed in CD68+ and CD163+ cells during parasitic brain infectionIn a case of Schistosoma mekongi infection, hematoxylin and.

Figure 1 Imaging and histopathologic characteristics of patients with CNS-FHL Imaging and histopathologic characteristics of patients with CNS-FHL FLAIR.

Figure 3 C5B3 blocked MAC formation

Figure 2 Antibodies to MOG detected with anti-human IgG (H + L) as the secondary antibody(A) Schematic of the human MOG proteins tested. Antibodies to.

Figure 2 Nonhuman primate brain immunohistochemistry

Figure 4 C5B3 decreased NMOSD mouse model lesions in vivo

Presentation transcript:

Figure 4 Neuropathology of MOG and AQP4 antibody–associated demyelinating lesions in the brain The biopsy specimen revealed a small actively demyelinating lesion (A, arrow in the magnified section indicates macrophages containing LFB-positive myelin degradation products) and inflammatory infiltrates composed of CD68-positive macrophages (B), and CD8-positive T cells (C). Neuropathology of MOG and AQP4 antibody–associated demyelinating lesions in the brain The biopsy specimen revealed a small actively demyelinating lesion (A, arrow in the magnified section indicates macrophages containing LFB-positive myelin degradation products) and inflammatory infiltrates composed of CD68-positive macrophages (B), and CD8-positive T cells (C). One vessel showed perivascular deposits of activated complement complex C9neo (D, arrows). The autopsy tissue showed confluent demyelinating lesions in the brain that were immunohistochemically characterized by loss of MBP (E, rectangle enlarged in H) but contained large preoligodendrocytes that strongly labeled for CNPase (F, rectangle enlarged in I) while MOG was almost negative (G, rectangle enlarged in J; lesion borders highlighted with dotted lines). The inflammatory infiltrates mainly contained CD3-positive (K) and CD8-positive (L) T cells and perivascular CD79a-positive B cells (M). The lesion in the optic chiasm showed perivascular deposits of activated complement complex C9neo (N, arrows) and was characterized by a destructive tissue injury with loss of astrocytes in the anti-AQP4 (O), AQP1 (P), and GFAP staining (Q). One plaque in the medulla oblongata showed a selective loss of AQP4 (R; lesion border highlighted with dotted lines) while AQP1 (S) and GFAP (T) were still preserved. The astrocytes in this lesion showed clasmatodendrosis with beading or loss of processes resulting in rounded astrocytes (T, magnified sections). Magnification: E–G: ×40; A and O–T: ×100; B–D: 200×; H–N and magnified section in T: 400×; magnified section in A: 600×. AQP = aquaporin; CNPase = 2',3'-cyclic-nucleotide 3'-phosphodiesterase; GFAP = glial fibrillary acid protein; LFB = Luxol fast blue; MBP = myelin basic protein; MOG = myelin oligodendrocyte glycoprotein. Franziska Di Pauli et al. Neurol Neuroimmunol Neuroinflamm 2015;2:e175 © 2015 American Academy of Neurology