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December 2015; 2 (6) ArticleOpen Access

Fulminant demyelinating encephalomyelitis

Insights from antibody studies and neuropathology

Franziska Di Pauli, Romana Höftberger, Markus Reindl, Ronny Beer, Paul Rhomberg, Kathrin Schanda, Douglas Sato, Kazuo Fujihara, Hans Lassmann, Erich Schmutzhard, Thomas Berger
First published November 4, 2015, DOI: https://doi.org/10.1212/NXI.0000000000000175
Franziska Di Pauli
From the Clinical Department of Neurology (F.D.P., M.R., R.B., K.S., E.S., T.B.) and Department of Neuroradiology (P.R.), Medical University of Innsbruck; Institute of Neurology (R.H.) and Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Departments of Neurology and Multiple Sclerosis Therapeutics (D.S., K.F.), Tohoku University Graduate School of Medicine, Sendai, Japan.
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Romana Höftberger
From the Clinical Department of Neurology (F.D.P., M.R., R.B., K.S., E.S., T.B.) and Department of Neuroradiology (P.R.), Medical University of Innsbruck; Institute of Neurology (R.H.) and Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Departments of Neurology and Multiple Sclerosis Therapeutics (D.S., K.F.), Tohoku University Graduate School of Medicine, Sendai, Japan.
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Markus Reindl
From the Clinical Department of Neurology (F.D.P., M.R., R.B., K.S., E.S., T.B.) and Department of Neuroradiology (P.R.), Medical University of Innsbruck; Institute of Neurology (R.H.) and Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Departments of Neurology and Multiple Sclerosis Therapeutics (D.S., K.F.), Tohoku University Graduate School of Medicine, Sendai, Japan.
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Ronny Beer
From the Clinical Department of Neurology (F.D.P., M.R., R.B., K.S., E.S., T.B.) and Department of Neuroradiology (P.R.), Medical University of Innsbruck; Institute of Neurology (R.H.) and Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Departments of Neurology and Multiple Sclerosis Therapeutics (D.S., K.F.), Tohoku University Graduate School of Medicine, Sendai, Japan.
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Paul Rhomberg
From the Clinical Department of Neurology (F.D.P., M.R., R.B., K.S., E.S., T.B.) and Department of Neuroradiology (P.R.), Medical University of Innsbruck; Institute of Neurology (R.H.) and Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Departments of Neurology and Multiple Sclerosis Therapeutics (D.S., K.F.), Tohoku University Graduate School of Medicine, Sendai, Japan.
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Kathrin Schanda
From the Clinical Department of Neurology (F.D.P., M.R., R.B., K.S., E.S., T.B.) and Department of Neuroradiology (P.R.), Medical University of Innsbruck; Institute of Neurology (R.H.) and Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Departments of Neurology and Multiple Sclerosis Therapeutics (D.S., K.F.), Tohoku University Graduate School of Medicine, Sendai, Japan.
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Douglas Sato
From the Clinical Department of Neurology (F.D.P., M.R., R.B., K.S., E.S., T.B.) and Department of Neuroradiology (P.R.), Medical University of Innsbruck; Institute of Neurology (R.H.) and Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Departments of Neurology and Multiple Sclerosis Therapeutics (D.S., K.F.), Tohoku University Graduate School of Medicine, Sendai, Japan.
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Kazuo Fujihara
From the Clinical Department of Neurology (F.D.P., M.R., R.B., K.S., E.S., T.B.) and Department of Neuroradiology (P.R.), Medical University of Innsbruck; Institute of Neurology (R.H.) and Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Departments of Neurology and Multiple Sclerosis Therapeutics (D.S., K.F.), Tohoku University Graduate School of Medicine, Sendai, Japan.
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Hans Lassmann
From the Clinical Department of Neurology (F.D.P., M.R., R.B., K.S., E.S., T.B.) and Department of Neuroradiology (P.R.), Medical University of Innsbruck; Institute of Neurology (R.H.) and Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Departments of Neurology and Multiple Sclerosis Therapeutics (D.S., K.F.), Tohoku University Graduate School of Medicine, Sendai, Japan.
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Erich Schmutzhard
From the Clinical Department of Neurology (F.D.P., M.R., R.B., K.S., E.S., T.B.) and Department of Neuroradiology (P.R.), Medical University of Innsbruck; Institute of Neurology (R.H.) and Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Departments of Neurology and Multiple Sclerosis Therapeutics (D.S., K.F.), Tohoku University Graduate School of Medicine, Sendai, Japan.
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Thomas Berger
From the Clinical Department of Neurology (F.D.P., M.R., R.B., K.S., E.S., T.B.) and Department of Neuroradiology (P.R.), Medical University of Innsbruck; Institute of Neurology (R.H.) and Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Departments of Neurology and Multiple Sclerosis Therapeutics (D.S., K.F.), Tohoku University Graduate School of Medicine, Sendai, Japan.
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Citation
Fulminant demyelinating encephalomyelitis
Insights from antibody studies and neuropathology
Franziska Di Pauli, Romana Höftberger, Markus Reindl, Ronny Beer, Paul Rhomberg, Kathrin Schanda, Douglas Sato, Kazuo Fujihara, Hans Lassmann, Erich Schmutzhard, Thomas Berger
Neurol Neuroimmunol Neuroinflamm Dec 2015, 2 (6) e175; DOI: 10.1212/NXI.0000000000000175

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    Figure 1 Cerebral MRI during the disease course

    Cerebral MRI with multiple cerebral supratentorial lesions during the disease course: periventricular lesions with diffusion restriction, only slight hyperintensity on T2-weighted images (A). Progressive diffusion restriction and now clearly hyperintense lesions on T2-weighted images 2 weeks after disease onset (B) and 4 weeks after disease onset (C). ADC = apparent diffusion coefficient.

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    Figure 2 Cerebral and spinal MRI

    (A) Restricted diffusion of both optic nerves (arrows) on diffusion-weighted and apparent diffusion coefficient imaging. (B) Intramedullary lesions. (C) Marginal contrast enhancement at disease onset (arrows). (D) Brainstem lesions (arrows).

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    Figure 3 Antibody titers during the disease course

    Temporal dynamic of MOG and AQP4 antibodies and time points of MRI and CSF sampling. AQP4 = aquaporin-4; MOG = myelin oligodendrocyte glycoprotein.

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    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). 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.

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