Summary
Ultrastructural studies of the retinae in two NCL-affected Dalmatian dogs revealed ubiquitous accumulation of lipopigments in numerous cell types of the retina, the fine structure of which closely resembled that seen in NCL-affected English setters. Photoreceptors and other retinal cell types were largely intact. These findings show that the retinal involvement in NCL of our Dalmatian dogs is identical to that of NCL-affected English setters. It also shows that in canine NCL a severe retinopathy, regularly encountered in human childhood NCL, does not develop. Thus, the NCL of Dalmatian dogs —and English setters — represents a reliable model to study human NCL, but for human retinopathia pigmentosa perhaps only at its earliest stage.
Similar content being viewed by others
References
Appleby EC, Longstaffe JA, Bell FR (1982) Ceroid-lipofuscinosis in two Saluki dogs. J Comp Pathol 92:375–380
Armstrong D, Koppang N, Nilsson SE (1982) Canine hereditary ceroid-lipofuscinosis. Eur Neurol 21:147–156
Bichsel P, Vandevelde M (1982) Un cas de lipofuscinose céroide chez un berger de Yougoslavie. Schweiz Arch Tierheilkd 124:413–418
Cummings JF, de Lahunta A (1977) An adult case of canine neuronal ceroid-lipofuscinosis. Acta Neuropathol (Berl) 39:43–51
Dahme E, Christomanou H, Bilzer T, Anzil AP (1983) Ceroidlipofuscinosis in the Dalmatian dog. J Meet Scand German Neuropathologists, Turku/Finland, June 3–4, 1983
Goebel HH, Fix JD, Zeman W (1974) The fine structure of the retina in neuronal ceroid-lipofuscinosis. Am J Ophthalmol 77:25–39
Goebel HH, Zeman W, Damaske E (1977) An ultrastructural study of the retina in the Jansky-Bielschowsky type of neuronal ceroid-lipofuscinosis. Am J Ophthalmol 83:70–79
Goebel HH, Koppang N, Zeman W (1979) Ultrastructure of the retina in canine neuronal ceroid-lipofuscinosis. Ophthalmic Res 11:65–72
Koppang N (1973/74) Canine ceroid-lipofuscinosis — A model for human neuronal ceroid-lipofuscinosis and aging. Mech Ageing Dev 2:421–445
Malkusch F (1985) Beitrag zur bioptischen Diagnose der neuronalen Ceroid-Lipofuscinose (NCL) des Hundes. Vet Med Diss (Thesis), Munich/FRG
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Goebel, H.H., Dahme, E. Retinal ultrastructure of neuronal ceroid-lipofuscinosis in the Dalmatian dog. Acta Neuropathol 68, 224–229 (1985). https://doi.org/10.1007/BF00690199
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00690199