Handbook of Neurochemistry : Volume VII Pathological Chemistry of the Nervous System

Anyone who has any contact with mental patients, old or young, or their families, or just visits a mental hospital or school for the retarded, is aware of the tremendous suffering caused by malfunctioning of the brain. The func­ tion of no other organ is so crucial for our everyday life, our proper func­ tioning, indeed our happiness, and no other illness causes as much anguish to patients or their families as mental illness. It is surprising and sad, therefore, how little effort has been devoted to research in this area; more so because such research is the only hope to ameliorate this suffering, or, to speak in the language of politics or economics, to decrease the enormous sums that we spend on trying to help our patients, with what is must generally be agreed are the most primitive and inadequate methods of treatment. Clearly, since functions of the brain are vital not only in illness, but in health, pathology is not the only area of concern to neurochemists, but it is an area that urgently needs neurochemical contributions. Progress in this field has been slower than in other areas of neurochemistry, and it seems that solutions in this field are very elusive. The reason for this is that the experimental approach is especially difficult in conditions specific for humans, or specific for complex behavior. Front Matter....Pages i-xxiii Disorders of Glycogen and Related Macromolecules in the Nervous System....Pages 1-15 Neurochemical Aspects of Mucopolysaccharidoses....Pages 17-32 Lipidoses....Pages 33-46 Biochemical Aspects of Multiple Sclerosis....Pages 47-91 Lipid Metabolism in Wallerian Degeneration....Pages 93-106 Basic Protein of Myelin and Its Role in Experimental Allergic Encephalomyelitis and Multiple Sclerosis....Pages 107-129 Pathological Cytosomes....Pages 131-142 Brain Damage in the Aminoacidurias....Pages 143-167 Abnormal Metabolism of Sulfur-Containing Amino Acids Associated with Brain Dysfunction....Pages 169-190 Amino Acid and Protein Metabolism in Wallerian Degeneration....Pages 191-219 Changes in Metabolic Processes During Pathogenesis of Experimental Allergic Encephalomyelitis....Pages 221-234 Proteins of the Central Nervous System in Pathology....Pages 235-254 Biochemical Effects of Adrenocortical Steroids on the Central Nervous System....Pages 255-287 Quantitative Neurochemical Histology....Pages 289-327 Biochemistry of Middle and Late Life Dementias....Pages 329-369 The Biochemistry of Affective Disorders....Pages 371-416 Alteration of the Brain Barrier System in Pathological States....Pages 417-427 Chemical Physiopathology of the Cerebrospinal Fluid....Pages 429-464 Neurochemistry of Parkinsonism....Pages 465-501 Biochemistry of Brain Tumors....Pages 503-542 Amino Acid Transport....Pages 543-572 Back Matter....Pages 573-675 1 Disorders of Glycogen and Related Macromolecules in the Nervous System.- I. The Normal Metabolism of Glycogen.- II. The Normal Distribution of Glycogen in the Nervous System.- III. Glycogen Deposition in Reactive Astrocytosis.- IV. Glycogen Deposition in Astrocytes after Irradiation.- V. Glycogen Deposition in Astrocytes in Severe Liver Disease.- VI. Glycogen Accumulation in Neurons and Schwann Cells.- VII. Increase of CNS Glycogen by Barbiturates.- VIII. The Effects of Certain Other Drugs and Altered Physiologic States on Glycogen in Cerebral Cortex.- IX. The Effect of Diabetes on CNS Glyco