CEROID‐LIPOFUSCINOSIS (BATTEN'S DISEASE): PATHOGENESIS OF BLINDNESS IN THE OVINE MODEL
- 1 July 1985
- journal article
- research article
- Published by Wiley in Neuropathology and Applied Neurobiology
- Vol. 11 (4), 273-290
- https://doi.org/10.1111/j.1365-2990.1985.tb00025.x
Abstract
Blindness is a feature of the group of storage diseases of children known as the ceroid-lipofuscinoses. Sequential studies in the ovine model, which most resembles the juvenile form of human disease, showed clearly that blindness had two components, a central and a peripheral. Whereas the central component, attributable to neuronal death and atrophy of the cerebral cortex, was responsible for early loss of vision, retinal atrophy was also extremely advanced in terminal stages of the disease. The primary retinal change was one of dystrophy of photoreceptor outer segments which preceded degeneration and necrosis of the photoreceptros cells themselves. Electroretinography showed that there was a progressive loss of a- and b-waves during the course of the disease, but this was preceded by a diminsihed c-wave which was eventually replaced by a negative potential. However, the pigment epithelium remained functionally (azide responsive) and ultrastructurally intact throughout the study. Loss of brain weight with selective cerebral atrophy also correlated with abnormal behaviour and facial manoeuvres that were intrepreted as partial seizures that did not become generalized.This publication has 14 references indexed in Scilit:
- Biosynthesis and Morphogenesis of Outer Segment Membranes in Vertebrate Photoreceptor CellsPublished by Elsevier ,1982
- Canine Hereditary Ceroid LipofuscinosisEuropean Neurology, 1982
- Jansky-Bielschowsky Form of Neuronal Ceroid-LipofuscinosisArchives of Ophthalmology (1950), 1980
- OVINE CEROID‐LIPOFUSCINOSIS: A MODEL OF BATTEN'S DISEASENeuropathology and Applied Neurobiology, 1980
- Ultrastructure of the Retina in Canine Neuronal Ceroid LipofuscinosisOphthalmic Research, 1979
- CHANGES IN ULTRASTRUCTURE AND FUNCTION OF THE SHEEP PIGMENT EPITHELIUM AND RETINA INDUCED BY SODIUM IODATE1Acta Ophthalmologica, 1977
- CHANGES IN ULTRASTRUCTURE AND FUNCTION OF THE SHEEP PIGMENT EPITHELIUM AND RETINA INDUCED BY SODIUM IODATE1Acta Ophthalmologica, 1977
- An Ultrastructural Study of The Retina in the Jansky-Bielschowsky Type of Neuronal Ceroid-LipofuscinosisAmerican Journal of Ophthalmology, 1977
- The Fine Structure of the Retina in Neuronal Ceroid-LipofuscinosisAmerican Journal of Ophthalmology, 1974
- Cellular Physiology of the Retina*†Journal of the Optical Society of America, 1963