Abstract
Four cases of a generalized form of glycogenosis occurring in German Shepherd dogs, all females, are described. Symptoms could be noticed as early as the age of two months and progressed slowly for months. They appeared as dizziness, muscular weakness, and in two of the cases as poor nutritional state. The abdomen became gradually distended. The main lesion seen at postmortem was a greatly increased liver size with some moderate liver fibrosis. Heavy deposits of a granular substance behaving as glycogen in histochemical tests and at electron microscopy were found in the hepatic cells, muscle fibres of the heart, skeletal and smooth muscles, and in nerve and glial cells of the central nervous system. The substance was lying freely dispersed in the cell cytoplasm without any indication of lysosomal storage. The disease of dogs does not seem to be fully comparable with any of the types observed in man, but is probably much related to Type III or Cori's Disease. Structure analysis of the deposits and enzyme investigations have been done and are published (Čeh et al. 1976).
Keywords: storage disease, glycogen, pathology, dog
Sammendrag
Det er beskrevet 4 tilfeller av en generalisert form for patologisk glykogenavleiring i organene hos hund. Alle var tisper og av Schäfer rase. Symptomene ble observert allerede fra ca. 2 måneders alder og tiltok i løpet av måneder. De besto i muskelsvekkelse, slapphet, tiltagende bukomfång og hos to av dyrene dårlig matlyst og nedsatt tilvekst. Ved obduksjon fantes en meget sterkt forstørret og svakt fibrotisk lever. Unormalt store mengder av en kornet substans som histokjemisk forholdt seg som glykogen fantes avleiret i cellenes cytoplasma i lever, all tverrstripet og glatt muskulatur, nerveceller og gliaceller. Substansen lå fordelt i cytoplasma uten tegn til lysosomal leiring og fantes i monogranulær form og i rosetter. Lidelsen hos hund synes ikke å være helt lik noen av de beskrevne typer av glykogenose hos menneske, men synes å stå nær Type III eller Cori’s sykdom. Strukturanalyse av den kornede substans og enzymatiske undersøkelser er utført av Ceh et al. (1976).
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Acknowledgment
We gratefully acknowledge the help given by Dr. Per Nafstad in the electron microscopy work.
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