An 8-month-old Sydney Silky dog that was ataxic and hyperkinetic was found to have a neurovisceral storage disease. Typical Gaucher cells were seen in the liver, lymph nodes and cerebellum, but not in the spleen. Ultrastructurally, the storage bodies in Gaucher cells contained tubular structures, and many neurones contained laminated cytosomes. accumulations of a ‘wispy’ material and rarely tubular material. Chemically, the liver and brain contained glucocerebroside.
References
1.
AdachiM.WallaceB.J.SchnechL.VolkB.W.:
Fine structure of central nervous system in early infantile Gaucher's disease.
Arch. Path.83:
513–526
(1967).
2.
AdornatoB.LampertP.:
Status spongiosus of nervous tissue, Electron microscopic studies.
Acta neuropath.19:
271–289
(1971).
3.
AleuF.P.TerryR.D.ZellwegerH.:
Electron microscopy of two cerebral biopsies in Gargoylism.
J. Neuropath. exp. Neurol.24:
304–317
(1965).
4.
BradyR.O.:
The sphingolipidoses.
New Engl. J. Med.275:
312–318
(1966).
5.
CancillaP. A.BarlowR. M.:
Morphologic abnormalities of myelin in border disease of lambs; in
Zimmerman Progress in neuropathology,
vol. 1,
pp. 76–83 (
Heineman,
London1971).
6.
CullingC.F.A.:
Handbook of histopathological techniques (Butterworths,
London1963).
7.
HibbsR.G.FerransV.J.CriprianoP.R.TardifK.J.:
A histochemical and electron microscopic study of Gaucher cells.
Arch. Path.89:
137–153
(1970).
8.
JollyR. D.BlakemoreW. F.:
Inherited lysosomal storage diseases.
An essay in comparative medicine. Vet. Rec.92:
391–400,
(1973).
9.
LawsL.SaalJ. R.:
Lipidosis of the hepatic reticulo-endothelial cells in a sheep.
Austr. vet. J.44:
416–417,
(1968).
10.
NormanR.M.:
Gaucher's disease; in Vinken and Bruyn Handbook of clinical neurology.
vol. 10.
pp. 509–531 (
North-Holland,
Amsterdam1970).
11.
NormanR.M.UrichH.LloydO.C.:
The neuropathology of infantile Gaucher's disease.
J. Path. Bact.62:
121–131
(1956).
12.
SandisonA.J.AndersonL.J.:
Histiocytosis in two pigs and a cow. Conditions resembling lipid storage disorders in man.
J. Path.100:
207–210
(1970).
13.
SuzukiK.SuzukiY.FletcherT.F.:
Further studies on galactocerebrosidase β-galactosidase in globoid cell leukodystrophy; in
Volk and Aranson Sphingolipids. sphingolipidases and allied disorders,
pp. 487–498 (
Plenum Press,
New York1972).
14.
SvennerholmL.:
The metabolism of gangliosides in cerebral lipidoses; in
Aranson and Volk Inborn disorders of sphingolipid metabolism,
pp. 169–186 (
Pergamon Press.
Oxford1967).
