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Glycogen Storage Disease in Dogs

Glycogenosis in Dogs

Glycogen storage disease, also termed glycogenosis, manifests as a deficiency or malfunction in the enzymes responsible for glycogen metabolism within the body. This rare hereditary condition encompasses various types, all of which result in the accumulation of glycogen, the primary carbohydrate reserve material in the body. Glycogen aids in short-term energy storage by converting to glucose as the body requires it for metabolic functions. The abnormal buildup of glycogen in tissues can lead to the enlargement and dysfunction of various organs, including the liver, heart, and kidneys.

There are four distinct types of glycogenoses known to affect dogs, with certain breeds being more susceptible to specific types. Type I-a, commonly referred to as von Gierke’s disease, predominantly affects Maltese puppies. Type II, Pompe’s disease, is prevalent in Lapland dogs and typically manifests around six months of age. Type III, Cori’s disease, occurs in young female German Shepherds. Finally, Type VII predominantly affects English Springer Spaniels aged between two to nine years old.

Symptoms and Types

Symptoms and types of glycogenosis vary depending on the breed and type of the disease.

Type I-a, commonly seen in Maltese puppies, may lead to failure to thrive, mental depression, hypoglycemia (low blood sugar), and potential death or euthanasia by the age of sixty days to alleviate symptoms.

Type II, prevalent in Lapland dogs, is marked by symptoms such as vomiting, progressive muscle weakness, and cardiac abnormalities. Typically, affected dogs succumb to the disease before reaching two years of age.

Type III, often observed in German Shepherds, manifests with symptoms including depression, weakness, failure to thrive, and mild hypoglycemia.

Type IV, found in English Springer Spaniels, results in hemolytic anemia, where red blood cells are destroyed, and hemoglobinuria, a condition characterized by abnormally high concentrations of hemoglobin in the urine.


The various forms of glycogenoses stem from deficiencies in glucose-metabolizing enzymes within the body. These types are differentiated by the specific enzyme deficiency present. In dogs, Type I-a arises from a deficiency of glucose-6-phosphatase, Type II from a deficiency of acid glucosidase, Type III from a deficiency of amylo-1 and 6-glucosidase, and Type VII from a deficiency of phosphofructokinase. Type IV, which affects cats, results from a deficiency of the glycogen branching enzyme.


The diagnosis of glycogen storage disease depends on the symptoms and the suspected type of the condition. A definitive diagnosis can be made through tissue enzyme analysis and the determination of glycogen levels. Additional diagnostic tests may include urine analysis, genetic testing, and electrocardiography (ECG) to assess changes in the heart’s electrical output.


Treatment approaches depend on the diagnosed type of glycogen storage disease and the extent of symptoms. For dogs diagnosed with Types I-a and III, intravenous (IV) dextrose may be necessary to address acute episodes of severely low blood sugar. However, long-term management of these conditions typically yields limited success. To regulate associated hypoglycemia, dietary adjustments may involve frequent feedings of a high-carbohydrate diet.

Living and Management

Following diagnosis, your dog requires ongoing monitoring and treatment for hypoglycemia. Unfortunately, there are limited options for reversing this condition. Due to the progressive decline in physical health, most animals affected by glycogenosis are euthanized.


Prevention of glycogen storage disease involves refraining from breeding animals affected by the condition. Additionally, the parents of such animals should not be bred together to mitigate the risk of future occurrences.

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