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Multisystem

This is a Multisystem condition.

GM1 Gangliosidosis

What is GM1?

An early onset form of lysosomal storage disease, this can cause affected dogs to display neurologic signs as puppies or young adults. These include partial or total vision loss, behavior changes, abnormal gait, and seizures. Lysosomal storage diseases are a class of disease stemming from inborn errors of metabolism. Lysosomes are one of many cellular structures with a highly specialized function: their job is to break down and recycle cellular waste, which includes engulfed molecules or microorganisms, byproducts of cellular metabolism, and old or degrading organelles. Lysosomes are known to contain over 50 enzymes (the proteins that do the digesting); lysosomal storage disease arises when there is a malfunction in any of these enzymes. The hallmark of lysosomal storage disease is the microscopic appearance of the lysosomes: they appear enlarged and swollen with undigested byproducts. The buildup of undigested byproducts can eventually lead to lysosomal, cell, tissue, and organ malfunction. Gangliosidosis in particular is caused by buildup of a fatty substance known as ganglioside, especially in cells of the nervous system.

What are the signs & symptoms that develop in affected dogs?

Signs include partial or total vision loss, behavior changes, abnormal gait, and seizures.

When do signs and symptoms develop?

This is an early onset form of lysosomal storage disease, this can cause affected dogs to display neurologic signs as puppies or young adults.

How do vets diagnose this condition?

Genetic and laboratory testing used to diagnose this disease. Advanced imaging (MRI/CT) may be used to exclude other similar disease processes.

How is this condition treated?

There is no treatment except supportive care for the clinical signs the affected dog develops.

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