Contents I
MEDICAL RADIOLOGY Diagnostic Imaging
Editors:
A. L. Baert, Leuven K. Sartor, Heidelberg
Contents III
M. Filippi · N. De Stefano · V. Dousset J.C. McGowan (Eds.)
MR Imaging in
White Matter Diseases of the Brain
and Spinal Cord
With Contributions by
R. Bammer · A. Bertolino · A. Bizzi · L. R. Caplan · M. Castillo · N. De Stefano · V. Dousset B. J. Emmer · F. Fazekas · M. Filippi · K. W. Fishbein · G. B. Frisoni · J. L. Go · J. A. Gomes S. J. Hickman · M. A. Horsfi eld · T. W. J. Huizinga · P. Jezzard · A. Kangarlu · P. E. Kim
B. K. Kleinschmidt-DeMasters · M. Knauth · I. Kovanlikaya · D. L. Kraitchman · R. Lenkinski D. K. B. Li · C. Marras · M. Maya · M. Mascalchi · J. C. McGowan · D. H. Miller · S. Mori
M. Mortilla · L. Nagae-Poetscher · D. T. Okuda · D. W. Paty · B. Pollo · C. Raybaud · M. A. Rocca M. Rovaris · F. Salvi · F. Sambataro · S. Schwarz · J. H. Simon · R. G. Spencer · S. Strasser-Fuchs S. D. Swanson · A. Toosy · A. Traboulsee · M. A. van Buchem · P. C. M. van Zijl · T. L. Vollmer G. Zhao · C-S. Zee
Foreword by
K. Sartor
With 247 Figures in 601 Separate Illustrations, 41 in Color and 23 Tables
123
IV Contents
Medical Radiology · Diagnostic Imaging and Radiation Oncology Series Editors: A. L. Baert · L. W. Brady · H.-P. Heilmann · M. Molls · K. Sartor Continuation of Handbuch der medizinischen Radiologie
Encyclopedia of Medical Radiology
Library of Congress Control Number: 2004103353
ISBN 3-540-40230-6 Springer-Verlag Berlin Heidelberg New York
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Massimo Filippi, MD
Director, Neuroimaging Research Unit Department of Neurology
Scientifi c Institute and
University Ospedale San Raffaele Via Olgettina, 60
20132 Milan Italy
Nicola De Stefano, MD, PhD Associate Professor
Department of Neurology & Behavioral Sciences University of Siena
Viale Bracci 2 53100 Siena Italy
Vincent Dousset, MD, PhD Professor, Service de Neuroradiologie Diagnostique et Thérapeutique
CHU Bordeaux Pellegrin and Laboratoire de Neurobiologie des Affections de la Myéline Université Victor Segalen Bourdeaux 2 33076 Bordeaux
France
Joseph C. McGowan, PhD
Associate Professor of Electrical Engineering Department of Electrical Engineering Maury Hall 227
United States Naval Academy Annapolis, MD 21402-5025 USA
Contents V
When magnetic resonance imaging (MRI) started to be used clinically in the early 1980s it rapidly became clear how little information had been provided hitherto by computed tomog- raphy (CT) on the various diseases that involve the cerebral (and spinal) white matter. For the fi rst time many white matter abnormalities with too little effect on tissue electron density to render them visible on CT could be shown in vivo. Also, as there was no ionizing radia- tion involved, these abnormalities could be studied over time (“monitored”) with essentially no risk to the patient. Along with the evolution of the new imaging modality, such as the steady improvement of MRI hard- and software and the increasing usage of paramagnetic agents to enhance tissue contrast, radiologists and their clinical colleagues gained insight into both the natural history and the course under therapy of many white matter diseases.
It was soon recognized, though, that some white matter diseases had a fairly characteristic or even diagnostic pattern on MRI, while others did not; morphologically there was much overlap between diseases differing in etiology and pathogenesis. Despite this limitation of standard structural (conventional) imaging techniques, MRI eventually gained a decisive role in clinical diagnosis and research of the most important white matter disease, multiple sclerosis (MS).
As the quest for advanced MRI methods to obtain more fundamental information on the various disease processes and to improve differential diagnosis continued, any promis- ing new imaging or measuring technique was tested for its usefulness to study normal and abnormal white matter. Some of the presently available techniques allow us to study crucial aspects of neurometabolism in a quantitative way (magnetization transfer MRI, diffusion- weighted MRI, proton MR spectroscopy), while others provide insight into neurovascular physiology and brain and spinal cord function (perfusion-weighted MRI, functional MRI).
The ultimate aim, however, is to develop methods suitable to study white matter structure, metabolism, physiology and function at the cellular and even molecular level, an endeavor likely to be successful only at fi eld strengths above 1.5 T.
In this book, conceived and edited by M. Filippi, N. De Stefano, V. Dousset and J. C. McGowan, an impressive number of world-renowned experts have set new standards of compact infor- mation regarding white matter disease. The book deals fi rst with the principles of pertinent modern MRI techniques and then covers in depth the disorders of myelination, including normal brain development, demyelinating diseases, immune-mediated disorders of white matter including vasculitides, white matter disorders related to aging, and white matter dis- orders secondary to other pathologic conditions.
In this state-of-the-art compendium, I am quite sure, interested (neuro)radiologists, neu- roclinicians, and neuroresearchers can fi nd practically everything worthwhile knowing on the in vivo imaging of white matter diseases of the brain and spinal cord. Convinced that the book will be a success, I wish to laud the editors and authors for their joint effort and timely work.
Heidelberg K. Sartor
Foreword
Contents VII
Preface
The application of magnetic resonance imaging (MRI) to the study of the central nervous system (CNS) has greatly improved our ability to diagnose numerous pathological condi- tions affecting the brain and the spinal cord, as well as to monitor their evolution. This is particularly true for multiple sclerosis (MS), where the sensitivity of T2-weighted MRI in the detection of white matter lesions, together with the ability of post-contrast T1-weighted images to refl ect the presence of acute infl ammatory activity, may allow us to demonstrate the dissemination of MS pathology in space and time earlier than the clinical assessment, thus leading to an earlier and more confi dent diagnosis. However, the whole spectrum of white matter diseases, ranging from inherited and acquired disorders of myelination to neurodegenerative conditions related to aging, has been the focus of many MRI studies since the earliest clinical application of this technology.
In the past few years, in parallel with the advancement of MRI technology, the many limi- tations of conventional MRI have become evident, both in the diagnostic work-up and in the research setting. Conventional MRI patterns of white matter pathology may, on the one hand, overlap among different CNS diseases, while, on the other, they provide only limited pieces of information on the underlying pathological changes in terms of both accuracy and specifi city. As regards this latter issue, conventional MRI has three major limitations. First, T2-weighted signal abnormalities just refl ect the presence of increased water content, which may range from transient edema to irreversible demyelination and axonal loss. Secondly, the presence of contrast enhancement indicates that blood-brain barrier permeability is increased and associated with ongoing infl ammation, but it does not provide any informa- tion about the nature and extent of associated tissue damage. Thirdly, conventional MRI is unable to detect and quantify the presence of damage occurring in the normal-appearing CNS tissues, which have been shown to be diffusely and sometimes severely damaged in many white matter disorders.
Structural and metabolic quantitative MRI techniques, such as magnetization transfer (MT) MRI, diffusion-weighted (DW) MRI and proton MR spectroscopy (1H-MRS), are increasingly being applied to the study of white matter diseases. Other non-conventional MRI techniques, such as functional MRI (fMRI), cell-specifi c MRI, perfusion MRI, molecular MRI and microscopic imaging with ultra-high-fi eld MRI, are emerging as additional prom- ising tools for improving our understanding of many of these conditions. These MRI tech- niques represent an extraordinary set of powerful instruments to gain in vivo fundamental insights into the pathogenesis and evolution of white matter damage. MT MRI and DW MRI enable us to quantify the extent of structural changes occurring in T2-visible lesions and in the white matter that appears normal on conventional MR images. 1H-MRS can add informa- tion on the biochemical nature of white matter changes, with the potential to improve sig- nifi cantly our ability to monitor infl ammatory demyelination and axonal injury. Structural and metabolic MR-based quantitative techniques are also contributing to the understanding of the reparative mechanisms occurring after injury to the CNS. This latter aspect is likely to have a central role in determining the fi nal clinical outcome of all neurological condi-
VIII Contents
tions. In this context, fMRI holds substantial promise to elucidate the mechanisms of cortical adaptive reorganization following brain injury, and eventually to achieve a more accurate picture of the balance between tissue damage and repair in various CNS conditions. Cellular MRI, molecular MRI and perfusion MRI have the potential to provide additional pieces of information on the heterogeneous aspects of white matter damage, which might be central for the understanding of the pathogenesis of new lesion formation and evolution. High-fi eld MRI will affect dramatically anatomical visualization, proton and nonproton MRS, fMRI and nonproton imaging and, as a consequence, our ability to image the critical components of white matter diseases. This aspect of MRI technology is progressing rapidly, and the time for a more extensive clinical application of high-fi eld MRI will probably come soon.
The present book aims at providing a complete and updated review of the “state of the art” of the application of conventional, quantitative and functional MRI techniques to the study of white matter disorders of the brain and spinal cord. In the fi rst, extensive section, each chapter examines in details the basic principles, advantages and disadvantages of all the aforementioned MRI techniques. The subsequent sections are clinically driven and focused on the various disorders that can affect the human CNS white matter. The role of MRI in the diagnosis and in monitoring the effi cacy of experimental treatment is reviewed extensively, as well as the novel insights provided by quantitative MR techniques into the pathophysiol- ogy of all these conditions. We hope that this book will represent a valuable tool for clinicians and researchers who wish to gain a deeper understanding of the complex issues related to diagnosis, work-up and treatment of patients with diseased white matter, as well as a reser- voir for new ideas and a stimulus for further investigations.
Milan Massimo Filippi
Siena Nicola De Stefano
Bordeaux Vincent Dousset
Annapolis Joseph C. McGowan
Contents IX
MR Techniques: Principles . . . 1 1 Basis of MR Contrast
Mark A. Horsfi eld . . . 3 2 Hardware for Magnetic Resonance Imaging
Kenneth W. Fishbein, Joseph C. McGowan, and Richard G. Spencer . . . 13 3 Spin- and Gradient Echo Imaging
Dara L. Kraitchman . . . 29 4 Fast Imaging with an Introduction to k-Space
Joseph C. McGowan . . . 41 5 Magnetization Transfer
Joseph C. McGowan . . . 57 6 Quantitative Diffusion Imaging
Peter C.M. van Zijl, Lidia Nagae-Poetscher and Susumu Mori . . . 63 7 MR Methods to Measure Cerebral Perfusion
Scott D. Swanson . . . 83 8 Functional MRI
Peter Jezzard and Ahmed Toosy . . . 93 9 MR Spectroscopy
Robert E. Lenkinski . . . 115 10 Molecular Imaging and High-Field MRI in Multiple Sclerosis
Alayar Kangarlu . . . 129
Disorders of Myelination . . . 149 11 MR Imaging of Brain Development
Charles Raybaud . . . 151 12 Imaging of Inherited and Acquired Metabolic Brain Disorders
Mauricio Castillo . . . 177 13 Proton MR Spectroscopy in Metabolic Disorders of the Central Nervous System
Nicola De Stefano and Marzia Mortilla . . . 195
Contents
X Contents
Demyelinating Diseases . . . 209 14 Conventional MRI Techniques in Multiple Sclerosis
Anthony Traboulsee, David K.B. Li, Guojun Zhao, and Donald W. Paty . . . 211 15 Multiple Sclerosis: Other MR Techniques
Massimo Filippi and Maria A. Rocca . . . 225 16 Variants of Multiple Sclerosis
Jack H. Simon and Bette K. Kleinschmidt-DeMasters . . . 241 17 Acute Disseminated Encephalomyelitis
Stefan Schwarz and Michael Knauth . . . 255 18 Demyelinating Diseases of the Spinal Cord
Roland Bammer, Franz Fazekas, and Siegrid Strasser-Fuchs . . . 269 19 Demyelinating Diseases of the Optic Nerve
Simon J. Hickman and David H. Miller . . . 279
Immune-Mediated Disorders . . . 291 20 Primary Angiitis of the Central Nervous System
Darin T. Okuda and Timothy L. Vollmer . . . 293 21 Neuro-Psychiatric Systemic Lupus Erythematosus
Bart J. Emmer, Tom W. J. Huizinga, and Mark A. van Buchem . . . 311 22 Non-MS Infl ammatory Diseases of the CNS:
MR Features in Addition to the White Matter
Mario Mascalchi and Fabrizio Salvi . . . 331 23 White Matter Pathology in Systemic Immune-Mediated Diseases
Marco Rovaris and Massimo Filippi . . . 343
White Matter Disorders Related with Aging . . . 353 24 Neuroimaging of Normal Brain Aging
Giovanni B. Frisoni . . . 355 25 White Matter Abnormalities in Patients with Cerebrovascular Disease
Joao A. Gomes and Louis R. Caplan . . . 363 26 Neurodegenerative Diseases with Associated White Matter Pathology
Mario Mascalchi . . . 377
Contents XI
White Matter Changes Secondary to Other Conditions . . . 389
27 Viral and Non-Viral Infections in Immunocompetent and Immunocompromised Patients
Vincent Dousset . . . 391 28 Neoplastic Disorders
Alberto Bizzi, Bianca Pollo, and Carlo Marras . . . 411 29 Head Trauma
Chi-Shing Zee, Marcel Maya, John L. Go, Paul E. Kim,
and Ilhami Kovanlikaya . . . 441 29 Psychiatric Disorders
Fabio Sambataro and Alessandro Bertolino . . . 453
Subject Index . . . 465 List of Contributors . . . 475
Basis of MRI Contrast 1
MR Techniques: Principles