Our thanks go to our colleagues at the VU Univer- Preface to the Third Edition sity Medical Center and to those in other hospitals Reading through the prefaces of the two previous edi- who referred their patients to us. We are indebted to tions,we can say that much of what was said there still all colleagues who allowed us to use their MR images, holds. At the same time,however,much has changed. published or unpublished,making it possible for us to There has been immense progress in the technical present illustrations of nearly all known white matter possibilities of magnetic resonance and in the know- disorders. Two colleagues were particularly helpful ledge of genetic defects, biochemical abnormalities, and provided us with essential and unpublished f- and cellular processes underlying myelin disorders. ures: our friends Susan Blaser,from the Hospital for This immense progress has prompted us to embark Sick Children in Toronto,and Zoltán Patay,from the upon the enormous task of rewriting the previous King Faisal Hospital in Riyadh. edition and adding 40 chapters. In doing so we have Many people at the VU University Medical Center tried to cover most white matter disorders,hereditary have been of great technical help to us in producing and acquired,and to present a collection of images to high quality images and in providing secretarial illustrate the field to the fullest possible extent. This assistance. The contributions of these people are edition will therefore be more complete than the pre- mentioned separately in the acknowledgements.

Myelin and White Matter

Classification of Myelin Disorders
Selective Vulnerability
Myelination and Retarded Myelination
Lysosomes and Lysosomal Disorders
Metachromatic Leukodystrophy
Multiple Sulfatase Deficiency
Globoid Cell Leukodystrophy: Krabbe Disease
GM1 Gangliosidosis
GM2 Gangliosidosis
Fabry Disease
Fucosidosis
Mucoplysaccharidoses
Free Sialic Acid Storage Disorder
Neuronal Ceroid Lipofuscinoses
Adult Polyglucosan Body Disease
Peroxisomes and Peroxisomal Disorders
Peroxisome Biogenesis Defects
Peroxisomal D-Bifunctional Protein Deficiency
Peroxisomal Acyl-CoA Oxidase Deficiency
X-Linked Adrenoleukodystrophy
Refsum Disease
Mitochondria and Mitochondrial Disorders
Mitochondrial Encephalopathy with Lactic Acidosis and Stroke-like Episodes
Leber Hereditary Optic Neuropathy
Kearns-Sayre Syndrome
Mitochondrial Neurogastrointestinal Encephalomyopathy
Leigh Syndrome and Mitochondrial Leukoencephalopathies
Pyruvate Carboxylase Deficiency
Multiple Carboxylase Deficiency
Cerebrotendinous Xanthomatosis
Cockayne Syndrome
Trichothiodystrophy with Photosensitivity
Pelizaeus-Merzbacher Disease and X-linked Spastic Paraplegia Type 2
18q? Syndrome
Phenylketonuria
Glutaric Aciduria Type 1
Propionic Acidemia
Nonketotic Hyperglycinemia
Maple Syrup Urine Disease
3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency
Canavan Disease
L-2-Hydroxyglutaric Aciduria
D-2-Hydroxyglutaric Aciduria
Hyperhomocysteinemias
Urea Cycle Defects
Serine Synthesis Defect Caused by 3-Phosphoglycerate Dehydrogenase Deficiency
Molybdenum Cofactor Deficiency and Isolated Sulfite Oxidase Deficiency
Galactosemia
Sjögren-Larsson Syndrome
Lowe Syndrome
Wilson Disease
Menkes Disease
Fragile X Premutation.-Hypomelanosis of Ito
Incontinentia Pigmenti
Alexander Disease
Giant Axonal Neuropathy
Megalencephalic Leukoencephalopathy with Subcortical Cysts
Congenital Muscular Dystrophies
Myotonic Dystrophy Type 1
Myotonic Dystrophy Type 2
X-linked Charcot-Marie-Tooth Disease
Oculodentodigital Dysplasia
Leukoencephalopathy with Vanishing White Matter
Aicardi-Goutières Syndrome
Leukoencephalopathy with Calcifications and Cysts
Leukoencephalopathy with Brain Stem and Spinal Cord Involvement and Elevated White Matter Lactate
Hypomyelination with Atrophy of the Basal Ganglia and Cerebellum
Hereditary Diffuse Leukoencephalopathy with Neuroaxonal Spheroids
Dentatorubropallidoluysian Atrophy
Cerebral Amyloid Angiopathy
Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy
Cerebral Autosomal Recessive Arteriopathy with Subcortical Infarcts and Leukoencephalopathy
Polycystic Lipomembranous Osteodysplasia with Sclerosing Leukoencephalopathy (Nasu-Hakola Disease)
Pigmentary Orthochromatic Leukodystrophy
Adult-Onset Autosomal Dominant Leukoencephalopathies
Inflammatory and Infectious Disorders
Multiple Sclerosis
Acute Disseminated Encephalomyelitis and Acute Hemorrhagic Encephalomyelitis
Acquired Immunodeficiency Syndrome
Progressive Multifocal Leukoencephalopathy
Brucellosis
Subacute Sclerosing Panencephalitis
Congenital and Perinatal Cytomegalovirus Infection
Whipple Disease
Toxic Encephalopathies
Iatrogenic Toxic Encephalopathies
Central Pontine and Extrapontine Myelinolysis
Hypernatremia
Marchiafava-Bignami Syndrome
Posterior Reversible Encephalopathy Syndrome
Langerhans Cell Histiocytosis
Post-Hypoxic-Ischemic Damage
Post-Hypoxic-Ischemic Encephalopathy of Neonates.-Neonatal Hypoglycemia
Delayed Posthypoxic Leukoencephalopathy
White Matter Lesions of the Elderly
Subcortical Arteriosclerotic Encephalopathy
Vasculitis
Leukoencephalopathy and Dural Arteriovenous Fistulas
Leukoencephalopathy After Radiotherapy and Chemotherapy
Gliomatosis Cerebri
Diffuse Axonal Injury
Wallerian Degeneration and Myelin Loss Secondary to Neuronal and Axonal Degeneration
Diffusion-Weighted Imaging
Magnetization Transfer Imaging
Magnetic Resonance Spectroscopy: Basic Principles and Application in White Matter Disorders
Pattern Recognition in White Matter Disorders.