Biology of Brain Dysfunction: Volume 1

'1 Genetic Disorders of Mucopolysaccharide Metabolism.- I. Introduction.- II. Classification of the Disorders.- A. Mucopolysaccharidosis I-the Hurler Syndrome.- B. Mucopolysaccharidosis II-the Hunter Syndrome.- C. Mucopolysaccharidosis III-the Sanfilippo Syndrome.- D. Mucopolysaccharidosis IV-the Morquio Syndrome.- E. Mucopolysaccharidosis V-the Scheie Syndrome.- F. Mucopolysaccharidosis VI-the Maroteaux-Lamy Syndrome.- G. Other Mucopolysaccharide Disorders.- III. Genetics.- IV. Chemical Pathology.- A. Storage.- B. Excretion of Mucopolysaccharides.- V. Chemistry of the Sulfated Mucopolysaccharides.- VI. The Biochemical Defect.- VII. Some Practical Applications of Recent Biochemical Findings.- VIII. Summary.- Note Added in Proof.- References.- 2 Galactosemia: Biochemistry, Genetics, Pathophysiology, and Developmental Aspects.- I. Introduction.- A. Evolutionary and Developmental Aspects.- B. Galactose Uptake.- II. Galactose Metabolism.- A. Galactokinase.- B. Galactose-1-Phosphate Uridylyl Transferase.- C. UDP-Galactose-4-Epimerase.- D. UDP-Glucose Pyrophosphorylase.- E. UDP-Glucose Dehydrogenase.- F. UDP-Galactose Galactosyl Transferase.- G. Galactose Dehydrogenase.- H. Galactose Reductase.- III. Hereditary Enzymatic Deficiencies in Galactose Metabolism.- A. Galactokinase Deficiency.- B. Galactose-1-Phosphate Uridylyl Transferase Deficiency.- C. Pedigree Studies.- D. Variations in Galactosemia.- E. Detection of Galactosemia.- IV. The Pathophysiology of Galactosemia.- A. Accumulation of Galactitol in Tissues.- B. Biochemical Basis for the Pathophysiology of Galactose-1-Phosphate Accumulation.- V. Treatment.- A. Dietary Control.- B. Outcome of Treatment.- References.- 3 Cytogenetic Aspects of Brain Dysfunction.- I. Introduction.- II. Incidence of Human Chromosome Anomalies.- III. Syndromes Related to Autosomal Abnormalities.- A. Numerical Aberrations.- B. Structural Aberrations.- IV. Syndromes Related to Sex Chromosome Abnormalities.- A. XXY-Klinefelter's Syndrome.- B. XXXXY Syndrome.- C. XXX, XXXX, and XXXXX Females.- D. XYY and XXYY Males.- E. X0-Turner's Syndrome.- V. Syndromes Related to Chromosome Breakages.- VI. Detection of Individuals with Abnormal Chromosomes.- VII. Prenatal Diagnosis of Chromosomal Abnormalities.- VIII. Concluding Remarks.- References.- 4 Disorders of Glucose Metabolism in Brain Dysfunction.- I. Introduction.- II. Systemic Disorders of Glucose Metabolism.- A. Hypoglycemia.- B. Systemic Disorders of Glucose Metabolism in Mental Disease.- III. Newer Concepts of Cerebral Glucose Metabolism in Vivo.- A. Human Subjects Studied with Arteriovenous Technique.- B. Animal Experiments on Metabolic Compartmentation in Brain.- C. Comments and Summary.- IV. Disorders of Glucose Metabolism in Brain.- A. Arteriovenous Studies with Chronic Mental Patients.- B. Convulsive Agents.- C. Hepatic Encephalopathy and Coma.- D. Hypoxia, Anoxia, and Hypocarbia.- E. Cerebral Vascular Disease and Cerebral Arteriosclerosis.- F. Other Conditions Causing Disorders in Cerebral Glucose Metabolism.- G. Comments and Summary.- References.- 5 Effects of Viral Infections on the Brain.- I. Introduction.- II. Methods.- A. Histological Techniques.- B. Histochemical Techniques.- C. Immunofluorescent Methods.- D. Electron Microscopy.- E. Cell and Tissue Culture Methods.- F. Virological Techniques.- G. Biochemical Methods.- H. Immunological Methods.- III. Pathways of Viral Spread.- A. Spread of Viruses to the Brain.- B. Dissemination of Viruses Within the Brain.- IV. Cellular Response to Viral Infection.- A. Susceptibility of Brain Cells to Viral Infection.- B. Cytopathic Effects.- C. Cell Proliferation.- D. Malignant Transformation.- E. Cellular Changes Resembling the Effect of Aging.- F. Infection of Cells Without Morphological Changes.- G. Inflammatory Reaction.- V. Biochemical Changes.- A. Nucleic Acids, Proteins, and Lipids.- B. Interferon.- VI.