Cerebral Sphingolipidoses

Cerebral Sphingolipidoses: A Symposium on Tay-Sach's Disease and Allied Disorders is a collection of papers presented at the 1961 Symposium on the Cerebral Sphingolipidoses, held in Isaac Albert Research Institute of the Jewish Chronic Disease Hospital and the Downstate Medical Center of the State University of New York. The contributors demonstrate the significance attached to an intensive study of Tay-Sach's and allied diseases. A unique feature of the Symposium was the constant awareness of the clinical implications of the fundamental studies presented. This book is organized into three sections encompassing 30 chapters. The first part deals with the morphological aspects of cerebral lipidoses, including the diagnosis, biopsy, pathology, and clinical features of these diseases. This part also provides case reports of specific cerebral sphingolipidoses. The second part highlights the biochemical aspects of Tay-Sach's and allied disorders. This part specifically looks into the mechanism of abnormal lipid metabolism, chemical pathology of lipids, and quantitative fractionation of complex lipid mixtures. This part also considers the characterization of accumulated gangliosides in brain, which is one the most striking features in infantile amaurotic idiocy, a type of Tay-Sach's disease. The third part explores the genetic and therapeutic aspects of the sphingolipidoses. This book will be of value to lipid chemists, geneticists, biochemists, neuropathologists, and clinicians.

Contributors and ParticipantsPrefaceAcknowledgmentsMorphologic Studies 1. Biopsy Diagnosis of Neurologic Diseases in Children with Emphasis on the Lipidoses 2. Pathogenesis of White Matter Changes in Tay-Sachs' Disease 3. On the Pigment Variant of Amaurotic Idiocy 4. Electron Microscopy of the Cerebrum in Tay-Sachs' Disease 5. Comparative Histologic, Histochemical and Electron Microscopy Studies of Rectal Biopsies in a Case of Adult Hereditary Cerebromacular Degeneration 6. The Cerebral Pathology of Infantile Gaucher's Disease 7. Cerebral Lipidosis of Unclear Nature 8. The Sphingolipids from the Spleen of a Case of Lipidosis 9. Lipid Deposits in the Brain in Nonspecific Conditions Histochemically like Those in the Lipidoses 10. Vacuolized Blood Lymphocytes in the Lipidoses and Other Central Nervous System Diseases with Special Reference to Histochemical Studies 11. Concepts of DysmyelinationBiochemical Studies 12. Alkali-Stable Choline Phospholipids ("Sphingomyelin") in Tissue of Amaurotic Idiocies 13. Abnormalities in Lipid Metabolism in Two Members of a Family with Niemann-Pick Disease 14. The Chemical Pathology of Krabbe Disease and Metachromatic Leucodystrophy 15. Determination of Alkali-Stable Phospholipids, Plasmalogens and Phosphatidyl Compounds in Tissues by Phosphorus Analysis 16. Quantitative Fractionation of Complex Lipid Mixtures 17. Chemical Pathology of Glycolipids in Brain Tissue of Tay-Sachs' Disease 18. Aminoglycolipids and Glycoproteins of Human Brain: New Methods for their Extraction and Further Study in the Sphingolipidoses 19. Physiochemical Factors Which Influence the Cationic Interactions of Strandin 20. The Fractionation of Beef Brain Ganglioside into Several Components with Thin-Layer and Column Silica Gel Chromatography 21. The Structure of the Gangliosides 22. The Membranous Granules in Toy-Sachs' Disease 23. A Study of Lipids in Cerebrospinal Fluid (and Serum). VII. In Several Sphingolipidoses (Tay-Sachs' Disease, Metachromatic Leucodystrophy, and Niemann-Pick Disease)Genetic and Therapeutic Aspects of the Sphingolipidoses 24. The Genetic Mechanism of Gaucher's Disease 25. Tangier Disease (Familial Cholesterolosis with High Density Lipoprotein Deficiency) 26. Some Epidemiologic and Genetic Aspects of Tay-Sachs' Disease 27. Genetic and Demographic Considerations Concerning Tay-Sachs' Disease 28. Genetics of the Sphingolipidoses 29. Principles and Practices on a Ward for Children with Tay-Sachs' Disease 30. Therapeutic Approaches to the LipidosesAuthor IndexSubject Index

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