Biology of Brain Dysfunction, Softcover reprint of the original 1st ed. 1975
Volume 3

The growth of neurochemistry. molecular biology, and biochemical genetics has led to a burgeoning of new information relevant to the pathogenesis of brain dysfunction. This explosion of exciting new information is crying out for collation and meaningful synthesis. In its totality, it defies systematic summa­ tion, and, of course, no one author can cope. Thus invitations for contributions were given to various experts in areas which are under active investigation, of current neurological interest, and pregnant. Although this project is relatively comprehensive, by dint of size. other topics might have been included; the selection was solely my responsibility. I believe systematic summation a virtual impossibility-indeed, hardly worth the effort. The attempt to assemble all of the sections involved in a large treatise with multiple authors inevitably results in untoward delays due to the difference in the rate at which various authors work. Therefore, the following strategy has been adopted: multiple small volumes and a relatively flexible format, with publication in order of receipt and as soon as enough chapters are assembled to make publication practical and economical. In this way, the time lag between the ideas and their emergence in print is the shortest.

1 Pathophysiology of Perinatal Hypoxic-Ischemic Brain Damage.- I. Introduction.- II. Human Perinatal Hypoxia-Ischemia.- A. Definitions.- B. Predisposing Factors.- C. Perinatal Mortality and Morbidity.- III. Neuropathology of Perinatal Hypoxia-Ischemia.- IV. Perinatal Central Nervous System Development.- A. Cerebrovascular Development.- B. Cerebral Energy Transformations in Developing Brain.- V. Experimental Approaches to Perinatal Hypoxia-Ischemia.- A. Perinatal Resistance in Hypoxia and Ischemia.- B. Methods of Producing Hypoxia and Cerebral Ischemia.- C. Effects of Hypoxia and Ischemia on the Brain.- D. Effects of Hypoxia on the Heart.- E. Factors Modifying the Response to Hypoxia.- VI. Conclusions.- References.- 2 Pathogenesis of Brain Dysfunction in Inborn Errors of Amino Acid Metabolism.- I. Introduction.- A. The Metabolic Sequelae of an Inborn Error of Amino Acid Metabolism.- B. Lines of Evidence Bearing on the Neurological Effects of These Metabolic Sequelae.- C. The Free Amino Acid Pools of the Nervous System.- D. Abnormalities in the Concentrations of Free Amino Acids and Metabolites in the Brain of Patients with Inborn Errors of Amino Acid Metabolism.- II. Effects of Metabolic Sequelae on the Fluxes and Transport of Amino Acids.- A. Amino Acid Fluxes.- B. Alterations of Amino Acid Transport.- III. Effects of Metabolic Sequelae on Synthesis of Protein in Brain.- IV. Effects of Metabolie Sequelae on Substances Affecting Neurotransmission in Brain.- A. Metabolism of Putative Amino Acid Transmitters.- B. Metabolism of Catecholamines.- C. Metabolism of 5-Hydroxytryptamine.- V. Effects of Metabolie Sequelae on Carbohydrate Metabolism and Energy Utilization.- VI. Effects of Metabolie Sequelae on Synthesis of Lipids and Myelin.- VII. Comments and Conclusions.- A. Evidence for Theories of Pathogenesis.- B. The Threshold for Neurological Deficit.- References.- 3 Disorders of Organic Acid Metabolism.- I. Introduction.- II. Inborn Errors of Leucine Metabolism.- A. Isovaleric Acidemia.- B. ?-Methylcrotonyl CoA Carboxylase Deficiency.- III. Inborn Errors of Isoleucine and Valine Metabolism.- A. ?-Methylacetoacetyl CoA Thiolase Deficiency.- B. Propionic Acidemia.- C. Methylmalonic Acidemia.- IV. Inborn Errors of Metabolism of Other Organic Acids.- A. Pyroglutamic Aciduria.- B. Congenital Medium-Chain Dicarboxylic Aciduria.- C. Glutaric Acidemia.- V. Inborn Errors of Pyruvate Metabolism.- A. Pyruvate Metabolism and Its Metabolie Regulations.- B. Pyruvate Carboxylase Deficiency.- C. Pyruvate Decarboxylase Deficiency.- VI. Disorders of Organic Acid Metabolism Induced by Natural Toxin.- A. Jamaican Vomiting Sickness.- References.- 4 Biological Aspects of Affective Psychoses.- I. Introduction.- II. Clinical Features.- III. The Biogenic Amine Hypothesis.- IV. Indoleamines in Affective Disorders.- A. Urinary Studies.- B. Cerebrospinal Fluid Studies.- C. 5-HIAA and 5-HT in the Brains of Depressed Suicidal Patients.- D. The Effects of Tryptophan and 5-HT Antagonists in Affective Disorders.- V. Lithium and Affective Disorders.- VI. Catecholamines and Affective Disorders.- A. Urinary Studies.- B. Cerebrospinal Fluid Studies.- C. The Effect of l-Dopa on Mood.- D. Effects of ?-Methylparatyrosine.- E. Enzymatic Studies.- F. Electroconvulsive Therapy (ECT).- G. Cholinergic-Adrenergic Interactions.- VII. Neuroendocrine Function in Affective Disorders.- A. Growth Hormone.- B. Prolactin.- C. ACTH and Cortisol.- D. Thyroid.- E. Sex Hormones.- VIII. Conclusions.- References.- 5 Wilson’s Disease.- I. Copper Metabolism.- II. Copper Toxicity.- III. An Inherited Abnormality of Copper Metabolism: Wilson’s Disease.- A. Pathology.- B. Pathological Physiology.- C. Clinical Aspects of Wilson’s Disease in the Central Nervous System.- D. Treatment.- E. Assessment of Current Knowledge of the Biochemical Lesion in Wilson’s Disease.- References.- 6 Pathogenesis of Slow Infections of the Central Nervous System.- I. Introduction.- II. Slow Virus Subacute Spongiform Encephalopathies.- A. General.- B. A Model System for Slow Virus Subacute Spongiform Encephalopathies: Scrapie.- C. Other Subacute Spongiform Encephalopathies.- D. Conclusion.- III. Slow Viral Encephalomyelitides.- A. General.- B. Subacute Sclerosing Panencephalities.- C. Other Slow Encephalomyelitides.- D. Conclusion.- References.- 7 Pathogenesis of Intrauterine Infections of the Brain.- I. Introduction.- II. Factors Required for the Initiation of Intrauterine Brain Infection.- A. Maternal Infection with Fetal Transmission.- B. Susceptible Stage of Gestation.- III. Infections Affecting the Fetal or Newborn Central Nervous System.- A. Naturally Occurring Infections in Man.- B. Naturally Occurring and Experimentally Induced Infections in Lower Animals.- IV. Possible Effector Mechanisms of Virus-Mediated Damage to the Developing Brain.- A. Generalized Placental and Fetal Infection.- B. Vasculitis.- C. Predilection for Discreet CNS Cell Populations of Either Immature or Differentiated Cell Types.- D. Chromosomal Injury.- E. Immunological Mechanisms.- F. Indirect Injury.- V. Fetal Immunological Response.- VI. Interferons.- VII. Discussion.- References.- 8 Ionizing Radiations and the Nervous System.- I. Introduction.- II. Some Radiobiological Considerations.- III. Developing Organisms.- A. Experimental Studies.- B. Effects in Man.- IV. The Adult.- A. Structural Changes.- B. Functional Changes.- References.- 9 Brain Dysfunction in Congenital Malformations of the Nervous System.- I. Introduction.- II. Dysraphic States.- A. Anencephaly.- B. Cranial Dysraphism Other Than Anencephaly.- III. Cerebral Ageneses and Hypoplasias.- A. Hypoplasia of the Cerebral Hemispheres.- B. Arhinencephaly.- C. Absence of the Corpus Callosum.- D. Agenesis of the Septum Pellucidum.- E. Absence or Hypoplasia of the Cerebellum.- F. Agenesis or Hypoplasia of Cranial Nerve Nuclei.- IV. Dysgenesis of the Cerebral Structures.- A. Lyssencephaly or Agyria.- B. Cerebral Polymicrogyria.- C. Cerebellar Polymicrogyria.- D. Heterotopia.- E. Minor Architectural Anomalies of the Cerebral Cortex.- F. Congenital Hyperplasia of the Brain, or Megalencephaly.- V. Developmental Cyst Formation.- A. Cavum Septi Pellucidi.- B. Developmental Porencephaly.- VI. Congenital Hydrocephalus and Hydranencephaly.- A. Congenital Hydrocephalus.- B. Hydranencephaly.- VII. Synopsis.- References.- 10 Pathogenesis of Brain Dysfunction in Deficiency of Thiamine, Riboflavin, Pantothenic Acid, or Vitamin B6.- I. Introduction.- II. Thiamine Deficiency.- A. Introduction.- B. Manifestations of Thiamine Deficiency.- C. Biochemical Changes in Thiamine Deficiency.- D. Summary.- III. Riboflavin Deficiency.- A. Introduction.- B. Manifestations of Riboflavin Deficiency.- C. Biochemical Changes in Riboflavin Deficiency.- D. Summary.- IV. Pantothenic Acid Deficiency.- A. Introduction.- B. Manifestations of Pantothenic Acid Deficiency.- C. Biochemical Changes in Pantothenic Acid Deficiency.- D. Summary.- V. Vitamin B6 Deficiency.- A. Introduction.- B. Manifestations of Vitamin B6 Deficiency.- C. Biochemical Changes in Vitamin B6 Deficiency.- D. Summary.- References.