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This is a hereditary neurological disease  that is one of the lysosomal storage diseases.  The membranes that coat neurons in the Central Nervous System and work as insulators for each neuron are not properly recycled through the normal cell cycle.  As the membrane degrades and is replaced by the cell, it should be broken down in the lysosomes of the cells.  The lysosomes breakdown worn out parts of the cell and prepare them to be reused but in this condition, the lysosomes lack the proper enzymes to break the used parts of the cells.  Because of this, there cannot be a reconstitution of these integral parts of the neurons.

The neurological deterioration can be confused with several other conditions and is difficult to diagnose directly.  The deterioration begins between four to six months old and will progress slowly until approximately a year and a half.  The disorder will become progressively worse at this point and will lead to a decision about the quality of life for the dog.  Certain breeds are more prone to this; Alaskan Malamute, Beagle, Golden Retriever, Japanese Chin, Portuguese Water Dog, Shiba Enu, and Toy Poodle.  Symptoms can include:

  • Mental Dullness
  • Non-Responsiveness
  • Exaggerated Startled Response
  • Forgetting to Go Outside to Go to the Bathroom
  • Goose Stepping
  • Lameness
  • Balance Issues
  • Head Shaking
  • Corneal Clouding
  • Weakness
  • Lethargy
  • Emotional Imbalances
  • Lack of Awareness of Surroundings
  • Death