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Use of Advanced Magnetic Resonance Imaging Techniques in Neuromyelitis Optica Spectrum Disorder

Stephane Kremer, MD, PhD1,2; Felix Renard, PhD3; Sophie Achard, PhD3; Marco A. Lana-Peixoto, MD, PhD4; Jacqueline Palace, DM5; Nasrin Asgari, MD, PhD6,7; Eric C. Klawiter, MD8; Silvia N. Tenembaum, MD9; Brenda Banwell, MD10,11; Benjamin M. Greenberg, MD12,13; Jeffrey L. Bennett, MD, PhD14,15; Michael Levy, MD, PhD16; Pablo Villoslada, MD17; Albert Saiz, MD17; Kazuo Fujihara, MD, PhD18; Koon Ho Chan, MD, PhD, FRCP19; Sven Schippling, MD20,21,22,23; Friedemann Paul, MD24,25,26; Ho Jin Kim, MD, PhD27,28; Jerome de Seze, MD, PhD29,30,31; Jens T. Wuerfel, MD24,25,26,32 ; and the Guthy-Jackson Charitable Foundation (GJCF) Neuromyelitis Optica (NMO) International Clinical Consortium and Biorepository
[+] Author Affiliations
1ICube (UMR 7357, UdS, Centre National de la Recherche Scientifique), Fédération de médecine translationelle de Strasbourg, Université de Strasbourg, Strasbourg, France
2Department of Radiology, Hôpitaux Universitaires de Strasbourg, Strasbourg, France
3Centre National de la Recherche Scientifique, Grenoble Image Parole Signal Automatique, Grenoble, France
4CIEM MS Research Center, University of Minas Gerais, Minas Gerais, Brazil
5Department of Neurology, Oxford University Hospital Trust, Oxford, England
6Department of Neurobiology, Institute of Molecular Medicine, University of Southern Denmark, Odense
7Department of Neurology, Vejle Hospital, Vejle, Denmark
8Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston
9Department of Neurology and Neurophysiology, National Pediatric Hospital Dr Juan P. Garrahan, Buenos Aires, Argentina
10Department of Neurology, University of Pennsylvania, Philadelphia
11Division of Child Neurology, Department of Pediatrics, Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania
12Department of Neurology and Neurotherapeutics, University of Texas Southwestern Medical Center, Dallas
13Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas
14Department of Neurology, University of Colorado Denver, Aurora
15Department of Ophthalmology, University of Colorado Denver, Aurora
16Department of Neurology, Johns Hopkins University, Baltimore, Maryland
17Institute of Biomedical Research August Pi Sunyer–Hospital Clínic de Barcelona, Barcelona, Spain
18Department of Multiple Sclerosis Therapeutics, Tohoku University Graduate School of Medicine, Sendai, Japan
19University Department of Medicine, Research Center of Heart, Brain, Hormone and Healthy Aging, Li Ka Shing Faculty of Medicine, University of Hong Kong, Hong Kong, People’s Republic of China
20Neuroimmunology and Multiple Sclerosis Research Section, University Hospital Zurich, Zurich, Switzerland
21Department of Neurology, University Hospital Zurich, Zurich, Switzerland
22Neuroscience Center Zurich, Federal Technical High School Zurich, Zurich, Switzerland
23University of Zurich, Zurich, Switzerland
24NeuroCure Clinical Research Center, Charité University Medicine, Berlin, Germany
25Clinical and Experimental Multiple Sclerosis Research Center, Charité University Medicine, Berlin, Germany
26Department of Neurology, Charité University Medicine, Berlin, Germany
27Department of Neurology, Research Institute, Goyang, Korea
28Hospital of National Cancer Center, Goyang, Korea
29Neurology Department, Hôpitaux Universitaires de Strasbourg, Strasbourg, France
30Clinical Investigation Center (INSERM 1434), Hôpitaux Universitaires de Strasbourg, Strasbourg, France
31UMR INSERM 1119 and Fédération de médecine translationelle, Strasbourg, France
32Institute of Neuroradiology, University Medicine Göttingen, Göttingen, Germany
JAMA Neurol. 2015;72(7):815-822. doi:10.1001/jamaneurol.2015.0248.
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Brain parenchymal lesions are frequently observed on conventional magnetic resonance imaging (MRI) scans of patients with neuromyelitis optica (NMO) spectrum disorder, but the specific morphological and temporal patterns distinguishing them unequivocally from lesions caused by other disorders have not been identified. This literature review summarizes the literature on advanced quantitative imaging measures reported for patients with NMO spectrum disorder, including proton MR spectroscopy, diffusion tensor imaging, magnetization transfer imaging, quantitative MR volumetry, and ultrahigh-field strength MRI. It was undertaken to consider the advanced MRI techniques used for patients with NMO by different specialists in the field. Although quantitative measures such as proton MR spectroscopy or magnetization transfer imaging have not reproducibly revealed diffuse brain injury, preliminary data from diffusion-weighted imaging and brain tissue volumetry indicate greater white matter than gray matter degradation. These findings could be confirmed by ultrahigh-field MRI. The use of nonconventional MRI techniques may further our understanding of the pathogenic processes in NMO spectrum disorders and may help us identify the distinct radiographic features corresponding to specific phenotypic manifestations of this disease.

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Figure 1.
Diffusion Tensor Imaging

A, Fiber bundles are composed of axons with myelinated sheaths. B, The corresponding diffusion tensor is modeled by an ellipsoid. Parallel diffusivity (Dpar) corresponds to the diffusivity in the main direction of the fiber bundle (reflecting axonal integrity), and perpendicular diffusivity (Dper) is related to the diffusivity orthogonal to this direction (reflecting the myelination).

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Figure 2.
Resting-State Functional Magnetic Resonance Imaging

A, Axial (left) and sagittal (right) views of the brain functional network. Nodes are located toward the coordinates of the regional centroids of the automated anatomical labeling template. Short-distance connections corresponding to the red edges are predominantly in the posterior cortex, whereas the long-distance connections shown in blue are between the frontal cortex and the regions of the parietal and temporal association cortex. B, Expanded Disability Status Scale (EDSS) as a function of the hub disruption index. A hub disruption index of 0 corresponds to a normal network. The farther the index deviates from 0, the more significant the reorganization of the network (in terms of topology). A correlation score highlights the fact that the reorganization of the brain network is a marker of the severity of the disease. The solid line represents the linear regression fit across all participants.

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Figure 3.
Optic Radiations

Optic radiation tractography was performed using a diffusion tensor imaging/magnetic resonance imaging (MRI) scan (Siemens Avanto 1.5-T MRI scanner, with 30 directions). Two seed points (the brightly colored fiber bundles) have been defined, the first one in the lateral geniculate body and the second one in the white matter at the posterior part of the occipital horn of the lateral ventricle. The fiber bundles are color coded according to their directions of impulse transmission.

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Figure 4.
Magnetic Resonance Imaging Scans of Neuromyelitis Optica (NMO) and Multiple Sclerosis (MS) Lesions at 7 T

Multiple sclerosis lesions are characteristically centered on a small vein in T2*-weighted sequences (blue arrowheads pointing to lesion surface and yellow arrowheads pointing to central intralesional vein) (A), a finding not present in 7-T magnetic resonance imaging scans of patients with NMO spectrum disorder who have brain parenchymal lesions (blue arrowheads) (B).

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