Ceroid-lipofuscinosis (Batten's Disease)

This book includes presentations from the Proceedings of the International Symposium on Human and Animal Models held in Roros, Norway, in June, 1980. The introduction includes the source of funds to study this disease and the initial description by Otto Christian Stengel. A section describes the clinical course of this disease and its variations, and the tests used in diagnosis, viz, the electroretinogram, electro-oculogram, visual evoked potential, color vision tests, and fluorescein angiography. The pathology, with well-reproduced electron micrographs, depicts the stages of degeneration within central and peripheral nerves resulting from deposition of ceroid-lipofuscin. The study of peripheral blood lymphocytes with the electron microscope is advocated as an easy procedure to establish the diagnosis of juvenile ceroidlipofuscinosis. In the canine model, peroxides increase along with the accumulation of lipopigments in cerebral neurons and glial cells. The isolation, identification, and quantitation of ceroid-lipofuscin pigment is described. Animal models include the English