Juvenile Laryngeal Paralysis & Polyneuropathy (JLPP)
The first time I ever heard of this was at a dog show and a breeder asked me if I'd had any experience with it because she had lost a few puppies to it. I had never heard of it....several years ago. Then I heard more talk about it last year and at that time affected only 2 litters in the US. My initial thought was that the breed had a lot bigger problems than something that had only affected 2 known litters. Granted, that this disease is particularly cruel in that it is 100% fatal to those puppies that contract it, and that it affects only very young dogs; but with such a small number affected I thought we had bigger fish to fry.
I now believe that this is the absolute most important use of any resources we can throw at it. The cases seem to tie back to a very popular European Stud dog who is now very prevalent in both the European and US gene pool. It is of utmost importance to have a means to identify carriers as soon as possible, given the statistical odds of producing carriers in an autosomal ressessive disease, there could be many, many carriers now coming of breeding age and this problem will spread through the breed like a time bomb if not identified. I hope you will support the JLPP research project http://www.brtca.org/juvenile-laryngeal-paralysis-and-polyneurpathy.html
I now believe that this is the absolute most important use of any resources we can throw at it. The cases seem to tie back to a very popular European Stud dog who is now very prevalent in both the European and US gene pool. It is of utmost importance to have a means to identify carriers as soon as possible, given the statistical odds of producing carriers in an autosomal ressessive disease, there could be many, many carriers now coming of breeding age and this problem will spread through the breed like a time bomb if not identified. I hope you will support the JLPP research project http://www.brtca.org/juvenile-laryngeal-paralysis-and-polyneurpathy.html
Through a BRTCA funded research grant, the genetic marker for JLPP has been discovered and the test is now offered through the OFFA to determine if BRTs are Clear or Carriers. https://secure.offa.org/cart.html While Carriers can be used in a responsible breeding program, it is imperative that any breeding not be between two carriers, since all dogs receiving two copies of the gene will die of JLPP.
Juvenile Laryngeal Paralysis & Polyneuropathy (JLPP) in Black Russian Terriers
Recently, Dr. Nicolas Granger reported a syndrome in Black Russian Terrier pups in Europe characterized by difficulties breathing and weakness shortly after weaning age. We have now seen affected dogs in the USA. The purpose of this article is to provide information about the condition so that breeders and veterinarians can be alert to any future cases and help us
find the gene responsible.
What is Juvenile Laryngeal Paralysis & Polyneuropathy?
The brain controls muscles via signals that travel through nerves. A disease that affects the nerves is called a polyneuropathy: poly- (many), neuro- (nerves), -pathy (a disease). Due to a quirk in the way an embryo develops, one of the longest nerves in the body supplies the muscles of the voice box (larynx). The vocal folds vibrate as air moves over them allowing a dog to bark. When they breathe in, muscles in the larynx pull the vocal folds aside so that air can move easily into their lungs. These nerves also help to
close the larynx when the dog swallows so they do not choke on their food.
If nerves are unable to convey that message properly, the muscles become weak or paralyzed. The longest nerves are often affected first; hence laryngeal paralysis is the first symptom. The vocal folds cannot be pulled out of the way as the dog breaths in. They vibrate noisily and can obstruct the flow of air into the lungs particularly when the dog is exercised or hot. The dog may also choke on their food or water or regurgitate, which can result in pneumonia.
The next longest nerves in the body go to the back legs, thus they are affected next. The dogs have difficulty getting up and wobble as they walk. Eventually the front legs will also be affected. The symptoms do not occur until after weaning age, and thus the disease is called juvenile laryngeal paralysis/polyneuropathy or JLPP for short
If you look down the throat of a dog with laryngeal paralysis, the vocal folds (arrows) do not pull out of the way like they should as the dog inhales. The airway cannot then open up completely when the dog is breathing hard, and they have trouble getting
enough air.
What else can look like JLPP?
There are other, much more common diseases that can affect a pup’s ability to breath. The windpipe (trachea) is stiff to keep it open when the dog is breathing hard. In some dogs, particularly toy breeds, the trachea does not have the proper stiffness and it can collapse as the dog breathes producing a honking cough. This condition is called collapsing trachea. An infection of the trachea such as kennel cough can cause irritation to the trachea and a similar sounding cough. The major difference is that dogs
with tracheal disease cough when breathing out, while laryngeal paralysis produces noise when the dog breathes in.Infections can cause swelling of the tonsils & lymph nodes around the throat in a young pup (strangles) shich can make it difficutl for the pup to breathe. Finally, other diseases of the nervous system, such as distemper infections, can affect nerves producing signs
of weakness, sometimes with pneumonia. Laryngeal paralysis also occurs in older dogs, but JLPP is different because they develop paralysis at such a young age.
Is this a hereditary disease?
Further research is needed, but the pedigrees analyzed thus far suggest that JLPP is inherited as a recessive trait. In a recessive
disease, both parents of an affected pup are normal. All animals have two copies of each gene, one that is inherited from the mother and one inherited from the father. A dog that has one normal gene and one gene that causes the disease is a carrier of the trait. They show no symptoms because the one good gene is enough for their nerves to develop normally, but they will pass that
bad gene on to about half of their offspring. If a carrier dog is bred to another carrier, then some of the pups (25% on average) will get a bad gene from each parent. Without one good gene to carry the day, the nerves cannot function normally and the unlucky pup has JLPP.
How do we find the gene resonsible for JLPP?
Genes contain the genetic code that programs everything about an animal from the color of their coat to how their nerves develop.
Genetic disease occurs when a mutation interferes with the ability of a gene to function normally. Each dog has an estimated 20,000 individual genes, any one of which could contain a mutation that causes JLPP. We now have the tools, however, to find the mutation responsible for such diseases.
Using these tools, we have successfully found the gene responsible for other hereditary diseases such as neonatal encephalopathy in Standard Poodles and degenerative myelopathy in a number of breeds. The research to identify the cause of a genetic disease requires a long term commitment by the breeders involved. Once we have identified the gene responsible for the disease, we can develop a DNA test that will aid breeders in avoiding the disease in the future.
How can I help?
If you have a litter with a pup you believe might be affected, please contact us. We can help your veterinarian in determining
whether or not this is the problem in your pup.
Any information provided to us will be kept strictly confidential.
Thank you!
Recently, Dr. Nicolas Granger reported a syndrome in Black Russian Terrier pups in Europe characterized by difficulties breathing and weakness shortly after weaning age. We have now seen affected dogs in the USA. The purpose of this article is to provide information about the condition so that breeders and veterinarians can be alert to any future cases and help us
find the gene responsible.
What is Juvenile Laryngeal Paralysis & Polyneuropathy?
The brain controls muscles via signals that travel through nerves. A disease that affects the nerves is called a polyneuropathy: poly- (many), neuro- (nerves), -pathy (a disease). Due to a quirk in the way an embryo develops, one of the longest nerves in the body supplies the muscles of the voice box (larynx). The vocal folds vibrate as air moves over them allowing a dog to bark. When they breathe in, muscles in the larynx pull the vocal folds aside so that air can move easily into their lungs. These nerves also help to
close the larynx when the dog swallows so they do not choke on their food.
If nerves are unable to convey that message properly, the muscles become weak or paralyzed. The longest nerves are often affected first; hence laryngeal paralysis is the first symptom. The vocal folds cannot be pulled out of the way as the dog breaths in. They vibrate noisily and can obstruct the flow of air into the lungs particularly when the dog is exercised or hot. The dog may also choke on their food or water or regurgitate, which can result in pneumonia.
The next longest nerves in the body go to the back legs, thus they are affected next. The dogs have difficulty getting up and wobble as they walk. Eventually the front legs will also be affected. The symptoms do not occur until after weaning age, and thus the disease is called juvenile laryngeal paralysis/polyneuropathy or JLPP for short
If you look down the throat of a dog with laryngeal paralysis, the vocal folds (arrows) do not pull out of the way like they should as the dog inhales. The airway cannot then open up completely when the dog is breathing hard, and they have trouble getting
enough air.
What else can look like JLPP?
There are other, much more common diseases that can affect a pup’s ability to breath. The windpipe (trachea) is stiff to keep it open when the dog is breathing hard. In some dogs, particularly toy breeds, the trachea does not have the proper stiffness and it can collapse as the dog breathes producing a honking cough. This condition is called collapsing trachea. An infection of the trachea such as kennel cough can cause irritation to the trachea and a similar sounding cough. The major difference is that dogs
with tracheal disease cough when breathing out, while laryngeal paralysis produces noise when the dog breathes in.Infections can cause swelling of the tonsils & lymph nodes around the throat in a young pup (strangles) shich can make it difficutl for the pup to breathe. Finally, other diseases of the nervous system, such as distemper infections, can affect nerves producing signs
of weakness, sometimes with pneumonia. Laryngeal paralysis also occurs in older dogs, but JLPP is different because they develop paralysis at such a young age.
Is this a hereditary disease?
Further research is needed, but the pedigrees analyzed thus far suggest that JLPP is inherited as a recessive trait. In a recessive
disease, both parents of an affected pup are normal. All animals have two copies of each gene, one that is inherited from the mother and one inherited from the father. A dog that has one normal gene and one gene that causes the disease is a carrier of the trait. They show no symptoms because the one good gene is enough for their nerves to develop normally, but they will pass that
bad gene on to about half of their offspring. If a carrier dog is bred to another carrier, then some of the pups (25% on average) will get a bad gene from each parent. Without one good gene to carry the day, the nerves cannot function normally and the unlucky pup has JLPP.
How do we find the gene resonsible for JLPP?
Genes contain the genetic code that programs everything about an animal from the color of their coat to how their nerves develop.
Genetic disease occurs when a mutation interferes with the ability of a gene to function normally. Each dog has an estimated 20,000 individual genes, any one of which could contain a mutation that causes JLPP. We now have the tools, however, to find the mutation responsible for such diseases.
Using these tools, we have successfully found the gene responsible for other hereditary diseases such as neonatal encephalopathy in Standard Poodles and degenerative myelopathy in a number of breeds. The research to identify the cause of a genetic disease requires a long term commitment by the breeders involved. Once we have identified the gene responsible for the disease, we can develop a DNA test that will aid breeders in avoiding the disease in the future.
How can I help?
If you have a litter with a pup you believe might be affected, please contact us. We can help your veterinarian in determining
whether or not this is the problem in your pup.
Any information provided to us will be kept strictly confidential.
Thank you!