Neuronal Ceroid Lipofuscinosis 8, NCL 8

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.

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

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.

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.

Talk to your vet about your dog’s NCL result so you can work together to plan ongoing care and monitoring. Note any changes in their coordination, mobility, or behavior.

Keep your dog’s environment calm and consistent. Use slings, harnesses, or supportive devices as needed to help with mobility, and place rugs or mats on slippery floors to prevent falls.

Encourage gentle, food-based activities such as treat searches or puzzle toys to help your dog stay mentally engaged without overexertion.

Help your dog feel secure by keeping furniture in familiar places, using non-slip mats, and maintaining predictable daily routines.

CLN8 belongs to a family of proteins known to function in lipid synthesis and transport. Unlike many proteins that cause NCL, CLN8 is found in the endoplasmic reticulum and likely participates in the shuttling of lipoproteins out of the endoplasmic reticulum.

Penetrance: Neuronal Ceroid Lipofuscinosis 8 is recessive, that is, a dog requires two copies of the variant to show signs of the disease from the variant. This variant was first described in Saluki.

Gene

CLN8

Inheritance type

recessive

Clinical category

Multisystem

Citations

1. Lingaas et al 2018

• Saluki