Neuronal Ceroid Lipofuscinosis 5 in Dogs

Category: Nervous System & Behavioral
Inheritance: Autosomal recessive
Breeds documented: 3
Published sources: 6

Overview

Changes in the CLN5 gene cause a form of neuronal ceroid lipofuscinosis called NCL5. Abnormal material builds up in nerve cells, leading to progressive damage in the brain and spinal cord. Affected dogs may become anxious, lose familiar skills, and have trouble coordinating their movements. Vision can deteriorate, and tremors or seizures may develop as the disease advances. Different CLN5 variants are responsible in different dog families.

Signs to watch for

Clumsy movements and difficulty using stairs
Anxiety, agitation, or persistent pacing
Less response to familiar commands or loss of learned habits
Worsening vision in bright or dim light
Trembling or seizures
Repetitive circling or unusually fixed behavior

Signs usually begin in young dogs, but timing differs among reported forms.

When to get help Book a veterinary visit soon for worsening coordination, vision, or learned behavior.

Diagnosis and tests

DNA tests look for the CLN5 variant associated with the dog's ancestry. The Golden Retriever test detects a different change from the one reported in Border Collies and Australian Cattle Dogs. A vet interprets the result alongside neurological and eye examinations.

Breeds with documented risk

3 breeds

By breed size

2 Medium
1 Large

Golden RetrieverLarge · Sporting Group
Border CollieMedium · Herding Group
Australian Cattle DogMedium · Herding Group

Inheritance

Autosomal recessive
A dog is affected only if it inherits two copies of the gene variant, one from each parent. A dog with one copy is a carrier: usually healthy, but able to pass the variant on. When two carriers are bred, on average one puppy in four is affected. A DNA test can tell clear, carrier and at-risk dogs apart.
As stated by OMIA

We've gathered what published sources say about this condition, so you can talk it through with your vet. It isn't veterinary advice: you and your vet know your dog best.