Neurovisceral Glucocerebrosidosis in Dogs

Neurovisceral glucocerebroside or Gaucher's Disease as it is often referred to, is a rare hereditary storage lysosomal disease that is caused by an autosomal recessive gene. Dogs with the condition are seen to have an acid beta-glucosidase and GBA deficiency which happens when their glucosylceramidases do not work as they should. The result is that enzymes start to build up intracellularly which causes all the damage that’s associated with neurovisceral glucocerebosidosis.

Breeds Most at Risk

Studies have established that certain breeds are more predisposed to inheriting the disorder than others which includes the following:

• Australian Silky Terrier

Symptoms to Watch Out For

When dogs develop the condition, there is a dramatic accumulation of glucocerebrosides in most areas of many of their organs which includes in the following:

• Brain
• Liver
• Peripheral lymph nodes

Symptoms Associated with the Condition

The onset of symptoms can be quite rapid and dogs typically start to show signs of there being something wrong with them when they are anything from 6 to 8 months old. Symptoms to watch out for usually include the following:

• Low, poor weight gain
• Lymphadenopathy
• Progressive neurological issues
• Uncoordinated gait - general ataxia
• A wide stance and gait
• Trembling
• Hyperkinesis
• Hypermetria
• Exaggerated limb reflexes but normal sensory reflexes

Diagnosing the Condition

A vet would need to thoroughly examine a dog suspected of suffering from the condition and they would also typically recommend carrying out the following tests which would help confirm a diagnosis:

• MRI scan which would help rule out any other health issue that may be causing the symptoms

Testing for Neurovisceral Glucocerebrosidosis

Unfortunately, there are no DNA tests available for this disease and as time passes, the symptoms tend to worsen with dogs usually succumbing to the disorder four to six months after the first symptoms first manifested themselves. It is also worth noting that the younger a dog is when they first start to show clinical signs of there being something wrong with them, the quicker the symptoms progress and because of the aggressive nature of the disease, it is much kinder to put a dog to sleep rather than let them suffer.