Summary
Ten Devon cattle from a single property were affected with blindness from 14 months of age. Severe retinal degeneration progressing to atrophy was associated with widespread intracellular accumulation of pigment in the retinal ganglion cells, central nervous system and major organs. The pigment was consistent with ceroid-lipofuscin granules on histological, histochemical and ultrastructural examination. Although a familial relationship existed between affected individuals, a pattern of inheritance could not be established by examination of available breeding records. The disease is compared to similar disorders reported in man and other species.
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References
Appleby EC, Longstaff JA, Bell FR (1982) Ceroid-lipofuscinosis in two Saluki dogs. J Comp Pathol 92:375–380
Armstrong D, Koppang N, Jolly RD (1980) Ceroid-lipofuscinosis. Comp Pathol Bull 21:2–4
Boellaard JW, Schlote W (1986) Ultrastructural heterogeneity of neuronal lipofuscin in the normal human cerebral cortex. Acta Neuropathol (Berl) 71:285–294
Cho DY, Leipold HW, Rudolph R (1986) Neuronal ceroidosis (ceroid-lipofuscinosis) in a Blue Heeler dog. Acta Neuropathol (Berl) 69:161–164
Cummings JF, De Lahunta A (1977) An adult case of canine neuronal ceroid-lipofuscinosis. Acta Neuropathol (Berl) 39:43–51
Donnelly WJC, Sheahan BJ, Rogers TA (1973) GMI gangliosidosis in Friesian calves. J Pathol 111:173–179
Dorling PR, Huxtable CR, Vogel P (1978) Lysosomal storage in Swainsona spp. toxicosis: an induced mannosidosis. Neuropathol Appl Neurobiol 4:285–295
Edwards JR, Richards RB (1979) Bovine generalised glycogenesis type II: a clinico-pathological study. Br Vet J 135:338–348
Goebel HH, Dahme E (1985) Retinal ultrastructure of neuronal ceroid-lipofuscinosis in the Dalmation dog. Acta Neuropathol (Berl) 68:224–229
Green PD, Little PB (1974) Neuronal ceroid-lipofuscin storage in Siamese cats. Can J Comp Med 38:207–212
Hanichen T, Puschner H (1971) Generalised lipofuscinosis with neural complications in a dog. Vet Med Rev 171:27–39
Hartley WJ, Webb RF (1982) A suspected new storage disease in cattle. Vet Pathol 19:616–622
Healy PJ (1982) Lysosomal hydrolase activity in leucocytes from cattle, sheep, goats, horses and pigs. Res Vet Sci 33:275–279
Hoover DM, Little PB, Cole WD (1984) Neuronal ceroid-lipofuscinosis in a mature dog. Vet Pathol 21:359–361
Huxtable CR, Chapman HM, Main DC, Vass D, Pearse BHG, Hilbert BJ (1987) Neurological disease and lipofuscinosis in horses and sheep grazingTrachyandra divaricata (branched onion weed) in South Western Australia. Aust Vet J 64:105–108
Jolly RD, Hartley WJ (1977) Storage diseases of domestic animals. Aust Vet J 53:1–8
Jolly RD, Janmaat A, West DM, Morrison I (1980) Ovine ceroid-lipofuscinosis: a model of Batten's disease. Neuropathol Appl Neurobiol 6:195–209
Koppang N (1970) Neuronal ceroid-lipofuscinosis in English setters. J Small Anim Pract 10:639–644
Koppang N (1974) Canine ceroid-lipofuscinosis. A model for human neuronal ceroid-lipofuscinosis and aging. Mech Aging Dev 2:421–445
Lake BO (1984) Lysosomal enzyme deficiencies. In: Adams JH, Corsellis JA, Duchen LW (eds) Greenfield's neuropathology, 4th edn. Edward Arnold, London, pp 503–513
Mayhew IG, Jolly RD, Pickett BT, Slack PM (1985) Ceroid-lipofuscinosis (Batten's disease): pathogenesis of blindness in the ovine model. Neuropathol Appl Neurobiol 11:273–290
Nimmo Wilkie JS, Hudson EB (1982) Neuronal and generalised ceroid-lipofuscinosis in a Cocker spaniel. Vet Pathol 19:623–628
O'Sullivan B, Healy PJ, Fraser IR, Neiper RE, Whittle AJ, Sewell CA (1981) Generalised glycogenesis in Brahman cattle. Aust Vet J 57:227–229
Rac R, Giesecke PR (1975) Lysosomal storage disease in Chihuahuas. Aust Vet J 51:403–404
Read WK, Bridges CH (1968) Neuronal lipodystrophy. Occurrence in an inbred strain of cattle. Pathol Vet 6:235–243
Taylor R, Farrow BRH (1988) Ceroid-lipofuscinosis in Border Collie dogs. Acta Neuropathol (Berl) 75:627–631
Vandevelde M, Fatzer R (1980) Neuronal ceroid-lipofuscinosis in older dachshunds. Vet Pathol 17:686–692
Wolfe LS, Ng Ying Kin NMK, Baker RR, Carpenter S, Anderman F (1976) Identification of retinoyl complexes as the autofluorescent component of neuronal storage material in Batten Disease. Science 195:1360–1362
Wolfe LS, Ivy GO, Witkop CJ (1987) Dolichols, lysosomal membrane turnover and relationships to the accumulation of ceroid and lipofuscin in inherited diseases, Alzheimer's disease and aging. Chem Scr 27:79–84
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Supported in part by a Wool Research Trust Fund scholarship (P. A. W. H.)
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Harper, P.A.W., Walker, K.H., Healy, P.J. et al. Neurovisceral ceroid-lipofuscinosis in blind Devon cattle. Acta Neuropathol 75, 632–636 (1988). https://doi.org/10.1007/BF00686210
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DOI: https://doi.org/10.1007/BF00686210