Neuronal ceroid lipofuscinosis of the dog was first described more than 20 years ago, yet this disease is still known to very few veterinarians and dog owners. In this monogenic autosomal recessive inherited disease, ceroid lipofuscin, a degradation product of the cells, is deposited mainly in the nerve cells of the retina and brain, causing them to perish. The most common symptoms observed in this disease are: Behavioral changes (aggressiveness, anxiety, restlessness, sudden housebreaking), impaired vision up to complete blindness, seizures and changes in gait (stumbling, ataxia) CCL disease has been detected in several breeds of dogs so far.

We need your active help in the research for the clarification of Canine Ceroid Lipofuscinosis, should your dog, the symptoms described in the respective breeds, then contact us or send us an EDTA blood sample of your dog. If possible, EDTA blood samples from the parents and littermates of the affected dog should also be sent in, as these samples greatly facilitate the clarification of CCL.

The processing fee is 50 € per sample sent in from a Tibetan Terrier/American Bulldog and 42 € for English Setters.

You can download the required sample submission form here on this page. Furthermore we would like to ask you to fill in the questionnaire about the symptoms of your dog completely and to send it in as well.

The symptoms described on this page are not sufficient to diagnose CCL. If you suspect your dog has CCL based on the symptoms described, please consult your veterinarian or be referred to a veterinary neurologist or eye specialist.

If you, as a veterinarian, have a patient in your practice who has the symptoms described or who you suspect may have CCL, please make the owner(s) aware of participation in our research project.

In collaboration with the Clinic for Small Animals of the University of Veterinary Medicine Hanover Foundation, a study is taking place to investigate CCL using MRI. Interested owners of dogs suffering from CCL are welcome to contact us for this purpose.

We kindly ask you to send us EDTA blood samples of the following dog breeds

  •     American Staffordshire Terrier
  •     Australian Cattle Dog
  •     Australian Shepherd
  •     Border Collie
  •     Chihuahua
  •     Cocker Spaniel
  •     Dalmatian
  •     Dachshund
  •     Badger spaniel
  •     Dalmatian
  •     English Setter
  •     Golden Retriever
  •     Japanese Retriever
  •     Labrador Retriever
  •     Pit Bull Terrier
  •     Saluki
  •     Sarplaninac
  •     Welsh Corgi
  •     Miniature Schnauzer

CCL in different breeds

American Bulldog

Genetic test available (available at the Institute for Animal Breeding and Genetics of the University of Veterinary Medicine Hanover Foundation; to the submission form)

American Staffordshire Terrier

Age at the time of the disease: 3-5 years
Clinical signs: mainly ataxia (disturbed coordination of movements) with balance disorders up to the inability to walk without falling down.
Genetic test available (available at ANTAGENE, France)

Australian Cattle Dog

Age at the time of disease: approx. 12 months
Time of euthanasia: approximately 1-2 years later, depending on the progression of the disease.
Clinical signs:
Changes in gait: Progressive ataxia (impaired movement coordination) as the disease progresses.
Eyes: Decrease in vision of the dogs up to complete blindness.
Behavioral changes: Behavioral changes occur.
The causative gene is not yet known.

Australian Shepherd

Age at the time of the disease: < 2 years
Clinical signs:
Changes in gait: Progressive problems walking, weakness and uncoordinated movements.
Eyes: Blindness may occur.
Change in behavior: dogs become aggressive, show dementia symptoms and confusion.
Seizure occurrence: Seizures have been described in some cases.
The causative gene is known.

Border Collie

Genetic test available (available at Laboklin)

Chihuahua

Age at the time of disease: 12 -20 months
Time of euthanasia: about 2 years, depending on the progression of the disease.
Clinical signs:
Changes in gait: walking in circles.
Eyes: Impairment of vision.
Behavioral changes: dogs become aggressive, lose sense of smell.
The causative gene is not yet known.

Cocker Spaniel

Age at the time of the disease: 18 months to 6 years
Time of euthanasia: Depending on the progression of the disease.
Clinical symptoms:
Changes in gait: Progressive problems with walking, weakness, and uncoordinated movements.
Eyes: Blindness may occur.
Change in behavior: dogs become aggressive, show dementia symptoms and confusion.
Seizure occurrence: Seizures have been described in some cases.
Furthermore, jaw spasms and emaciation may occur.

Dackel

In the Dachshund must be distinguished between two different forms of CCL. On the one hand there is an adult form and a juvenile form which affects young dogs.
For the juvenile form a genetic test is available (University of Missouri).
Age at time of disease: juvenile form approximately 9 months; adult form approximately 4.5 -6.5 years.
Time of euthanasia: juvenile form at approximately 12 months; adult form at 4.5 -7 years, depending on disease progression.
Clinical signs:
Changes in gait: pass gait, ataxia (impaired coordination of movement), goose step.
Eyes: Impaired vision in the juvenile form, have not been described in the adult form.
Alteration of behavior: dogs become aggressive, compulsive actions, running in circles, loss of housetraining, unable to perform previously mastered commands.
Seizure occurrence: Seizures occur.
In the adult form, excessive hunger (polyphagia) and increased fluid intake (polydipsia) may occur.
In the juvenile form, vomiting has been observed.
The causative gene for the adult form is not yet known.
In the juvenile form, the causative mutation is in the canine genes CLN1 (dwarf) and CLN2 (long coat).

Dachsbracke

Age at the time of the illness: approx. 2 years.
Time of euthanasia: about 4 years, depending on the progression of the disease.
Clinical symptoms:
Eyes: Not described to date.
Change in behavior: apathy, disorientation, running into obstacles, anxiety especially with auditory stimuli, stereotyped forced movements, house mess, leaning against walls.
Seizure occurrence: Not described to date.
Changes in gait: Ataxia (impaired coordination of movement), spreading of forelimbs and hindlimbs, stepping through.
Polydipsia (increased intake of fluid) and frequent passing of small amounts of urine (polyuria) may occur during the course of the disease.
The causative gene is not yet known.

Dalmatiner

Age at the time of the illness: 0.5-1 year
Time of euthanasia: about 2-7 years later, depending on the progression of the disease.
Clinical symptoms:
Eyes: The first symptoms of the disease in Dalmatians are a decrease in the dogs' vision, which is expressed by affected dogs running into objects. As the disease progresses, complete blindness occurs.
Change in behavior: aggressiveness towards people, affected bitches show cannibalism towards their puppies. Affected dogs show tendencies of autoaggression, extreme paw nibbling may occur. Agitated pacing.
Changes in gait: tremor and ataxia (impaired coordination of movement) occur from 15-22 months of age. Increased stumbling and running into objects may be observed from about 2 years of age.
Seizures also occur from 15-22 months.
The causative gene is not yet known.

English Setter

Age at the time of the disease: 12-14 months
Change of behavior: Confusion and dementia symptoms
Eyes: The first symptoms of the disease are a decrease in the dogs' vision, which is expressed in the fact that affected dogs run against objects. As the disease progresses, complete blindness occurs.
Changes in gait: ataxia (disturbed coordination of movement), spreading of the forelimbs and hind limbs.
Epileptic seizures are particularly common in this breed and worsen as the disease progresses.
Genetic test available (available at the University of Missouri).

Golden Retriever / Labrador Retriever

Age at the time of the illness: 6-7 years
Time of euthanasia: about 1-2 years later, depending on the progression of the disease.
Clinical symptoms:
Seizure history: Convulsions, characterized by twitching of the face and ears, have been observed as the first symptoms of the disease. As the disease progresses, tonic-clonic seizures may occur.
Changes in gait: Ataxia (impaired coordination of movement) occurs as the disease progresses.
In addition, dysphagia (difficulty swallowing) may occur as the disease progresses.
The causative gene is not yet known.

Japanese Retriever

Age at the time of the disease: from about 3 years.
Clinical symptoms:
Seizure occurrence: Convulsions have been observed as the first symptoms of the disease.
In addition, polydipsia (increased thirst) may occur during the course of the disease.
The causative gene is not yet known.

Pitt Bull Terrier

Clinical symptoms: mainly ataxia (disturbed coordination of movement) with balance disorders.
Genetic test available (available at ANTAGENE, France)

Polish Owczarek Nizinny (PON)

Age at the time of the illness: of approx. 0,5-4,5 years
Time of euthanasia: about 2-4 years later, depending on the progression of the disease.
Clinical signs:
Change in behavior: a common observation is aggressiveness toward familiar people. Increasing jumpiness (especially at unusual noises), anxiety, confusion.
Eyes: decrease in vision, initially especially at dusk or dark. No longer follows movements properly with eyes.
Changes in gait: Progressive changes in locomotion, especially in hind limbs. With occasional crossing of limbs and stumbling. As the disease progresses, animals may not want to move and may lean against walls.
Skin problems may occur in affected dogs.
The causative gene is not yet known.

Salukis

The causative gene is not yet known.

Tibet Terrier

Age at the time of the disease: 4 to 6 years.
Clinical symptoms:
Changes in gait: Progressive problems walking, weakness and uncoordinated movements, problems climbing stairs.
Eyes: Night blindness at onset, which may worsen to complete blindness.
Change in behavior: dogs become aggressive and nervous, show dementia symptoms and confusion. Problems with eating are also common.
Seizure occurrence: Seizures have been described in some cases.
Genetic test available (available at the Institute for Animal Breeding and Genetics of the University of Veterinary Medicine Hanover Foundation; to the submission form).

Welsh Corgi

Age at the time of the disease: 6 -8 years.
Time of euthanasia: 6 -9 years, depending on the progression of the disease.
Clinical signs:
Eyes: Impairment of vision occurs.
Behavioral changes: behavioral changes have been observed.
Seizure occurrence: Seizures occur.
The causative gene is not yet known.

Zwergschnauzer

Age at the time of the illness: 2-4 years
Time of euthanasia: about 2-4 years later, depending on the progression of the disease.
Clinical symptoms:
Eyes: The first symptoms of the disease in the Miniature Schnauzer are visual problems. Affected dogs can no longer find their way around unfamiliar environments, run into objects, do not want to climb stairs. The blindness progresses very quickly, often the dogs are completely blind 5 months after the first symptoms.
Change in behavior: The dogs appear confused, are no longer aware of their surroundings. Can no longer perform commands that are otherwise safely followed.
Seizures in the form of persistent tremors may occur.
The causative gene is not yet known.