Foreword. List of contributors. 1. A neuropathologic overview of the neurodystrophies and neurolipidoses (J.M. Powers). 2. Biochemistry of lipids (P. Morell, A.D. Toews). 3. Enzymology (I. Mononen). 4. Molecular biology (S. Byravan, A.T. Campagnoni). 5. Bone marrow transplantation treatment for globoid cell leukodystrophy, metachromatic leukodystrophy, adrenoleukodystrophy, and Hurler syndrome (W. Krivit,E.G. Shapiro, L.A. Lockman, M. Balthazor, J. Wagner, C. Peters, J. Anderson, D. Loes). 6. Metabolic manipulation therapy of the neurolipidoses (G. Grabowski, N.D. Leslie). 7. Prospects for gene therapy in the lysosomal storage disorders (E.M. Kaye). 8. Gaucher disease (R.O. Brady). 9. Niemann-Pick diseases (M.T. Vanier, K. Suzuki). 10. Metachromatic leukodystrophies (M. Philippart). 11. Globoid leukodystrophy (E.H. Kolodny). 12. Farber disease (lipogranulomatosis) (H.W. Moser). 13. Fabry disease:?-galactosidase A deficiency (C.M. Eng, R.J. Desnick). 14. The gangliosidoses (K. Suzuki, K. Suzuki). 15. The mucopolysaccharidoses (C.B. Whitley). 16. The oligosaccharidoses: mannosidosis, fucosidosis and aspartylglucosaminuria (G. Dawson). 17. Schindler disease: deficient ?-N-acetylgalactosaminidase activity (R.J. Desnick, D. Schindler). 18. Sialidoses (W.A. Gahl, D.M. Krasnewich, J.C. Williams). 19. The mucolipidoses (including I-cell disease)(J.G. Leroy). 20. Mitochondrial disorders (D.C. De Vivo, M. Hirano, S. DiMauro). 21. X-linked adrenoleukodystrophy (P. Aubourg). 22. Heredopathia atactica polyneuritiformis (Refsum's disease) (O.H. Skjeldal). 23. Generalized peroxisomal disorders and disorders of peroxisomal fatty acid oxidation (R.J.A. Wanders, H.S.A. Heymans, R.B.H. Schutgens, P.G. Barth). 24. Rhizomelic chondrodysplasia punctata (H.S.A. Heymans, R.J.A. Wanders). 25. Disorders of lipoprotein metabolism (S.D. Kafonek, H.W. Moser). 26. Pelizaeus-Merzbacher disease (F. Seitelberger, S. Urbanits, K.-A. Nave). 27. Smith-Lemli-Opitz syndrome (R.I. Kelley). 28. Cerebrotendinous xanthomatosis (A. Federico, M.T. Dotti). 29. Sjoegren-Larsson syndrome (W.B. Rizzo). 30. Carbohydrate-deficient glycoprotein syndromes (B.A. Hagberg, G. Blennow, B. Kristiansson, H. Stibler). 31. Disorders of organic acid metabolism (G.F. Hoffmann, K.M. Gibson). 32. Canavan disease (R. Matalon, K. Michals, R. Kaul). 33. Batten disease or neuronal ceroid lipofuscinosis (R.-M. Boustany). 34. Alexander disease (A.B. Johnson). 35. Hallervorden-Spatz syndrome (K.F. Swaiman). 36. Leukoencephalopathies due to exogenous factors with features of leukodystrophy: central nervous system damage caused by exposure to solvent vapors (toluene) (M. Kornfeld). Index.
