Polyglucosan body disease in a mixed-breed dog

Authors: Mehrtens GN, Hill FI, Hill JA, Davey PM, Jolly RD, Hopcroft DH
Publication: New Zealand Veterinary Journal, Volume 50, Issue 1, pp 32-35, Feb 2002
Publisher: Taylor and Francis

Animal type: Companion animal, Dog
Subject Terms: Clinical pathology, Diagnostic procedures, Genetics, Liver/hepatic disease, Locomotor, Disease/defect, Metabolic disease, Nervous system/neurology, Pathology
Article class: Clinical Communication

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Abstract: AIM: To describe the histopathology of a previously unrecorded canine disease and deduce the cause of the lesions.
METHODS: Formalin-fixed tissues were processed into paraffin wax and epoxy resin for light and electron microscopy of variously stained sections of liver, brain, heart muscle and kidney.
RESULTS: Periodic acid Schiff (PAS) -positive bodies in liver and myocardium were typical of a polyglucosan body disease. Neurons contained coarse granular material that stained similarly to the polyglucosan bodies.
CONCLUSION: The nature, distribution and histochemistry of lesions observed are consistent with a putative diagnosis of Glycogen storage disease type IV, an inherited metabolic defect associated with a deficiency of glycogen-branching enzyme not previously reported in dogs.
KEY WORDS: Polyglucosan body, glycogen storage, type IV, dog.

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