Acta Neuropathologica

, Volume 68, Issue 3, pp 224–229 | Cite as

Retinal ultrastructure of neuronal ceroid-lipofuscinosis in the Dalmatian dog

  • H. H. Goebel
  • E. Dahme
Original Works

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.

Key words

Dalmatian dogs Lipopigments Retina Retinopathy Ultrastructure Ceroidlipofuscinosis 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Appleby EC, Longstaffe JA, Bell FR (1982) Ceroid-lipofuscinosis in two Saluki dogs. J Comp Pathol 92:375–380Google Scholar
  2. Armstrong D, Koppang N, Nilsson SE (1982) Canine hereditary ceroid-lipofuscinosis. Eur Neurol 21:147–156Google Scholar
  3. Bichsel P, Vandevelde M (1982) Un cas de lipofuscinose céroide chez un berger de Yougoslavie. Schweiz Arch Tierheilkd 124:413–418Google Scholar
  4. Cummings JF, de Lahunta A (1977) An adult case of canine neuronal ceroid-lipofuscinosis. Acta Neuropathol (Berl) 39:43–51Google Scholar
  5. Dahme E, Christomanou H, Bilzer T, Anzil AP (1983) Ceroidlipofuscinosis in the Dalmatian dog. J Meet Scand German Neuropathologists, Turku/Finland, June 3–4, 1983Google Scholar
  6. Goebel HH, Fix JD, Zeman W (1974) The fine structure of the retina in neuronal ceroid-lipofuscinosis. Am J Ophthalmol 77:25–39Google Scholar
  7. 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 83:70–79Google Scholar
  8. Goebel HH, Koppang N, Zeman W (1979) Ultrastructure of the retina in canine neuronal ceroid-lipofuscinosis. Ophthalmic Res 11:65–72Google Scholar
  9. Koppang N (1973/74) Canine ceroid-lipofuscinosis — A model for human neuronal ceroid-lipofuscinosis and aging. Mech Ageing Dev 2:421–445Google Scholar
  10. Malkusch F (1985) Beitrag zur bioptischen Diagnose der neuronalen Ceroid-Lipofuscinose (NCL) des Hundes. Vet Med Diss (Thesis), Munich/FRGGoogle Scholar

Copyright information

© Springer-Verlag 1985

Authors and Affiliations

  • H. H. Goebel
    • 1
  • E. Dahme
    • 2
  1. 1.Division of NeuropathologyUniversity of MainzMainzFederal Republic of Germany
  2. 2.Dept. of Veterinary Pathology (General Pathology and Neuropathology)University of MunichMunichFederal Republic of Germany

Personalised recommendations