Cerebral sphingolipidoses ; a symposium on Tay-Sachs' disease and allied disorders

Content: Front Matter , Page iii Copyright , Page iv CONTRIBUTORS AND PARTICIPANTS , Pages v-x PREFACE , Pages xi-xii , HEINRICH WAELSCH ACKNOWLEDGMENTS , Page xiii Biopsy Diagnosis of Neurologic Diseases in Children, with Emphasis on the Lipidoses , Pages 1-13 , BENJAMIN H. LANDING, JACK H. RUBINSTEIN Pathogenesis of White Matter Changes in Tay-Sachs' Disease , Pages 15-28 , STANLEY M. ARONSON, BRUNO W. VOLK On the Pigment Variant of Amaurotic Idiocy , Pages 29-47 , FRANZ SEITELBERGER, KASPAR SIMMA Electron Microscopy of the Cerebrum in Tay-Sachs' Disease , Pages 49-56 , ROBERT D. TERRY, SAUL R. KOREY, MARTIN WEISS Comparative Histologic, Histochemical, and Electron Microscope Studies of Rectal Biopsies in a Case of Adult Hereditary Cerebromacular Degeneration , Pages 57-72 , L. ROIZIN, W. SLADE, H. HERMIDA, H. ASAO The Cerebral Pathology of Infantile Gaucher's Disease , Pages 73-99 , BETTY Q. BANKER, JAMES Q. MILLER, ALLEN C. CROCKER Cerebral Lipidosis of Unclear Nature , Pages 101-118 , GEORGE JERVIS, RUTH C. HARRIS, JOHN H. MENKES The Sphingolipids from the Spleen of a Case of Lipidosis , Pages 119-123 , ABRAHAM ROSENBERG Lipid Deposits in the Brain in Nonspecific Conditions Histochemically Like Those in the Lipidoses , Pages 125-128 , IRWIN FEIGIN, ILONA BUBELIS Vacuolized Blood Lymphocytes in the Lipidoses and Other Central Nervous System Diseases with Special Reference to Histochemical Studies , Pages 129-140 , MONA SPIEGEL-ADOLF, H.W. BAIRD III, H.S. COLEMAN, E.G. SZEKELY Concepts of Dysmyelination , Pages 141-164 , CHARLES M. POSER Alkali-Stable Choline Phospholipids (“Sphingomyelin”) in Tissue of Amaurotic Idiocies , Pages 165-170 , G.W.F. EDGAR Abnormalities in Lipid Metabolism in Two Members of a Family with Niemann-Pick Disease , Pages 171-178 , J.N. CUMINGS The Chemical Pathology of Krabbe Disease and Metachromatic Leucodystrophy , Pages 179-195 , MARJORIE B. LEES, HUGO W. MOSER Determination of Alkali-Stable Phospholipids, Plasmalogens, and Phosphatidyl Compounds in Tissues by Phosphorus Analysis , Pages 197-214 , GERHARD SCHMIDT, LOUIS H. FINGERMAN, S.J. THANNHAUSER Quantitative Fractionation of Complex Lipid Mixtures , Pages 215-235 , GEORGE ROUSER Chemical Pathology of Glycolipids in Brain Tissue of Tay-Sachs' Disease , Pages 237-248 , E.R. BERMAN, S. GATT Aminoglycolipids and Glycoproteins of Human Brain: New Methods for Their Extraction and Further Study in the Sphingolipidoses , Pages 249-270 , SAMUEL BOGOCH Physiochemical Factors Which Influence the Cationic Interactions of Strandin , Pages 271-288 , ALFRED F. HARRIS, ABRAHAM SAIFER The Fractionation of Beef Brain Ganglioside into Several Components with Thin-Layer and Column Silica Gel Chromatography , Pages 289-299 , JOEL A. DAIN, H. WEICKER, GERHARD SCHMIDT, S.J. THANNHAUSER The Structure of the Gangliosides , Pages 301-307 , E. KLENK, W. GIELEN, G. PADBERG The Membranous Granules in Tay-Sachs' Disease , Pages 309-315 , S. SAMUELS, S.R. KOREY, J. GONATAS, R. TERRY, M. WEISS A Study of Lipids in Cerebrospinal Fluid (and Serum). VII. In Several Sphingolipidoses (Tay-Sachs' Disease, Metachromatic Leucodystrophy, and Niemann-Pick Disease) , Pages 317-326 , WALLACE W. TOURTELLOTTE, RICHARD J. ALLEN, RUSSELL N. DEJONG The Genetic Mechanism of Gaucher's Disease, , Pages 327-342 , DAVID YI-YUNG HSIA, JEAN NAYLOR, JOHN A. BIGLER Tangier Disease (Familial Cholesterolosis with High-Density Lipoprotein Deficiency) , Pages 343-357 , DONALD S. FREDRICKSON, PAUL H. ALTROCCHI Some Epidemiologic and Genetic Aspects of Tay-Sachs' Disease , Pages 359-374 , NTINOS C. MYRIANTHOPOULOS Genetic and Demographic Considerations Concerning Tay-Sachs' Disease , Pages 375-394 , STANLEY M. ARONSON, BRUNO W. VOLK Genetics of the Sphingolipidoses , Pages 395-411 , ALFRED G. KNUDSON JR., WILLIAM D. KAPLAN Principles and Practices on a Ward for Children with Tay-Sachs' Disease , Pages 413-420 , ABRAM KANOF, SAMUEL DUNKELL, IRMA ABRAMSON Therapeutic Approaches to the Lipidoses , Pages 421-440 , ALLEN C. CROCKER, SIDNEY FARBER AUTHOR INDEX , Pages 441-451 SUBJECT INDEX , Pages 452-456 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 sec