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Multisystem

This is a Multisystem condition.

Neuronal Ceroid Lipofuscinosis 8, NCL 8

What is NCL 8?

Neuronal ceroid lipofuscinoses (NCLs) are lysosomal storage diseases that cause the accumulation of ceroid-lipofuscin in multiple areas of the brain and retinas. 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.

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

Common symptoms reflect central nervous system (CNS) malfunction and progress from anxiety, noise sensitivity, and restlessness to impaired vision and hearing. Epileptic-like seizures, loss of house training skills, and difficulties walking may develop later in the course of the disease, which typically leads to severe quality of life issues around two years of age.

When do signs and symptoms develop?

In Salukis, NCL 8 has been observed with signs beginning at around one year of age.

How do vets diagnose this condition?

Diagnosis is made based on clinical history, physical examination, genetic testing, and ruling out other causes of similar symptoms. Histopathology of the brain may offer a definitive diagnosis after the affected dog is deceased. Clinical signs of NCL 8 may mimic many other CNS diseases, so examination by a veterinarian or veterinary neurologist is required. Advanced imaging (CT or MRI scan) and/or a spinal tap may be performed to rule out other diseases.

How is this condition treated?

There is currently no treatment for NCL 8. Gene therapy is an evolving field but requires more research to be available clinically. Some medications aimed at controlling seizures may offer a temporary reduction in their frequency, however, the medications will eventually stop controlling the seizures.

What actions should I take if my dog is affected?

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