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Multisystem

This is a Multisystem condition.

Neuronal Ceroid Lipofuscinosis 10, NCL 10

What is NCL 10?

A lysosome is a structure within the cell that digests and removes waste. When the lysosome cannot recycle waste properly, the waste accumulates and causes the cell to die. This form of lysosomal storage disease causes juvenile to adult onset neurologic signs.

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

Common symptoms reflect central nervous system malfunction and include partial or total vision loss, behavior changes, abnormal gait, and seizures. Symptoms usually progress slowly over time.

When do signs and symptoms develop?

Bulldogs homozygous for this mutation typically present between 1 and 3 years of age and the symptoms progress slowly over 2 to 4 years.

How do vets diagnose this condition?

NCL is definitively diagnosed through genetic testing or examination of central nervous system (CNS) tissues after the affected dog is deceased. Clinical signs of this disease may mimic many other CNS diseases, so examination by a veterinarian or veterinary neurologist is required. Advanced imaging (CT or MRI scan) and/or spinal taps may be performed to rule out other diseases.

How is this condition treated?

Currently, there is no treatment for NCL. While gene therapy trials in mouse models have proven promising, there is much more research required before it can be used in veterinary hospitals.

What actions should I take if my dog is affected?

  • Keeping your affected dog comfortable at home is the best therapy you can provide.
  • Slings can be used to assist with their mobility.
  • Blind dogs benefit from leaving your furniture in the same place and being put on leashes in unfamiliar territory.
  • Please give your dog any medication as prescribed by your veterinarian.