Spontaneous Gangliosidoses in Animals

  • Masazumi Adachi


During the past two decades, an increasing number of reports have appeared in the literature which have dealt with diseases considered to be the counterpart of human gangliosidoses. Thus for the first time, in 1953, Hagen1 described clinical and pathological features of two cases of “amaurotic idiocy” in English setters. Histochemically, however, the cytoplasmic inclusion material seemed to be mainly phosphatides. Koppang2 used animals for the same breeding stock of English setters and obtained, by planned mating, dogs of both sexes affected with the disease. Fourteen of them developed slowly progressive clinical signs at one year of age which consisted of incoordination and decreased vision. Later the dogs displayed various mental disturbances and died at about two years of age. He considered the clinical course to be similar to that seen in the juvenile form of familial amaurotic idiocy, but could not reach any conclusions regarding the genetic pattern of transmission. Grossly, the brain showed edematous leptomeninges and atrophic changes, especially in the cerebellum. Microscopically, the neurons of the brain exhibited a cytoplasmic granular material which displayed histochemical characteristics of glycolipids.


Sialic Acid Glycosidase Activity Total Sialic Acid Total Ganglioside Cocker Spaniel 
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Copyright information

© Plenum Press, New York 1975

Authors and Affiliations

  • Masazumi Adachi
    • 1
    • 2
  1. 1.Isaac Albert Research InstituteKingsbrook Jewish Medical CenterUSA
  2. 2.Downstate Medical CenterState University of New YorkBrooklynUSA

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