CONTENTS -- AUTHORS' APPOINTMENTS -- FOREWORD -- 1 LEUKODYSTROPHY AND MYELIN -- Introduction -- Definition of leukodystrophies -- 2 MYELINATION IN HEALTH AND DISEASE -- Introduction -- Oligodendrocytes have a highly polarized shape -- Molecular composition of myelin and oligodendrocyte membranes -- Molecular mechanisms of myelin membrane formation -- Reciprocal interactions between axons and oligodendrocytes -- Summary and conclusions -- 3 THE ROLE OF ASTROCYTES IN WHITE MATTER DISEASE -- Introduction -- Cellular lineage systems in the mammalian brain -- Astrocytes in development and adult life -- Leukodystrophies with distinct astrocyte pathology -- Possible mechanisms of white matter damage secondary to astrocyte dysfunction -- Summary and future perspectives -- 4 MICROGLIA AND LEUKODYSTROPHIES -- Introduction -- Macrophage populations in the central nervous system -- Defining microglial activation -- Microglia in development and repair -- Brain colonization, self-renewal, and post-lesional recruitment of microglia -- Microglia in the pathogenesis of leukodystrophies -- Adrenoleukodystrophy -- Does VLCFA accumulation cause microglial activation? -- How does microglial activation relate to demyelination in adrenoleukodystrophy? -- Metachromatic leukodystrophy -- Microglia in globoid cell leukodystrophy (Krabbe disease) -- The role of microglia in transplantation -- Concluding remarks -- 5 X-LINKED ADRENOLEUKODYSTROPHY -- Introduction -- Biochemical and molecular basis -- Clinical features -- Diagnosis -- Animal models -- Pathogenesis -- Therapy in adrenoleukodystrophy -- Expanded screening for asymptomatic individuals -- 6 KRABBE DISEASE (GLOBOID CELL LEUKODYSTROPHY) -- Introduction -- Clinical features -- Diagnostic evaluation -- Pathological findings -- Biochemical findings -- Molecular genetics -- Newborn screening -- Studies in animal models.

Therapy -- Conclusions -- 7 ALEXANDER DISEASE -- Introduction -- Clinical presentation -- MRI characteristics -- Pathology -- Diagnosis -- GFAP mutations -- Cases without GFAP mutations -- Recent cases -- Disease mechanisms -- Treatment -- Concluding remarks -- 8 METACHROMATIC LEUKODYSTROPHY -- Classification and definition -- Incidence and prevalence -- Genetics -- Biochemical background -- Pathophysiology -- Clinical features -- Laboratory and genetic diagnosis -- Therapy -- 9 CANAVAN DISEASE -- Introduction -- Molecular basis -- Clinical features -- Variant forms of the disease -- Differential diagnosis -- Pathogenesis and pathophysiology -- Prognosis -- Epidemiology -- Gene therapy -- Prevention -- 10 PELIZAEUS-MERZBACHER DISEASE: GENETIC MODELS AND MECHANISMS -- Involvement of myelin proteolipid protein -- The PLP1 gene -- Genetics of PMD/SPG-2 -- Genetic models -- Subcellular mechanisms of disease development -- Pelizaeus-Merzbacher-like disease -- Conclusion -- 11 THERAPEUTIC APPROACHES IN LEUKODYSTROPHIES -- Introduction -- Cell-based therapies -- Enzyme replacement therapy -- Gene therapy -- Other therapeutic approaches -- Future development -- 12 CLINICAL APPROACH TO IDENTIFICATION OF LEUKOENCEPHALOPATHIES -- Introduction -- Clinical features -- Metabolic changes -- Magnetic resonance imaging -- Calcifications -- Cystic lesions -- Conclusion -- INDEX.