Camelot Pugs
Juergen "Jay" Ernst
659 Penn High Park Road
Jeannette, PA 15644
724 309 4317
Home of top quality, family raised AKC champion line and champion sired Pug and Havanese puppies

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PDE Research
Research on Brain Disease in Pugs Could Also Help Scientists Understand A Rare Form of Multiple Sclerosis
Toy dogs may be small and cute, but that doesn’t mean they can’t suffer from some very big and ugly health problems. There are a number of serious, and often fatal, inflammatory diseases of the brain and spinal cord seen in toy breeds. Although these conditions have been recognized as a problem for several decades, their causes have largely remained unexplained.
That may be changing. In recent years, there has been a growing body of evidence suggesting that many, or all, of this group of breed-specific degenerative nervous system conditions may be auto-immune in origin. In fact, there is a growing suspicion that these diseases could be related to the human disease known as multiple sclerosis (MS).
Thanks to a grant from the AKC Canine Health Foundation, scientists are now much closer to understanding how strong that similarity actually may be. A group of researchers looking at the genetic causes of one of these inflammatory brain conditions, necrotizing meningoencephalitis (NME), have found that disease risk in Pug Dogs is closely linked to the same group of genes that has been implicated in human risk for MS.
A whole genome scan of Pug dogs with and without NME found that the single genetic association for the disease was with the dog leukocyte antigen (DLA) complex – a group of genes analogous to the human leukocyte antigen (HLA) complex. However, the 2010 study, published by Dr. Kimberly Greer and her colleagues, didn’t stop there. It also delved further to discover that the specific sequence variants that were highly correlated to a Pug’s risk of developing NME were in the same genes that have been shown to be associated with human risk of MS.
Necrotizing meningoencephalitis, also known as Pug Dog Encephalitis (PDE), is an aggressive brain disease that can lead to death within months, weeks, or even days, of onset. Although the most common forms of MS progress far more slowly than NME, relapsing and remitting for years or even decades, there are certain acute forms of MS that behave more like the Pug condition. As with NME, these severe progressive forms of MS occur more often in females than in males and have a younger age of onset than the more commonly observed types. Thus, although NME isn’t a perfect model for MS, this naturally occurring dog disease could still provide scientists with important insights about how similar inflammatory brain diseases progress in humans.
The long term implications, at least for Pugs, remain mixed. It has been known for years that inbreeding dogs to bring out desirable "pure" breed traits can also amplify the presence of harmful conditions. That is certainly the case for NME in Pugs. In this study, 11 percent of dogs had two copies of the genetic sequence associated with the highest risk of NME and a full 29 percent had a single copy of the variant.
Although genetic testing could help breeders limit the number of puppies at the highest level of danger, those with two copies of the gene, it is impossible to even consider weeding the problematic mutation out of the Pug genome entirely. Pugs are already a relatively homogeneous group, with 87 percent of the breed traceable back to a single male ancestor. Trying to eliminate every copy of the high-risk variant would cause a further loss in genetic diversity, and there’s a real possibility that doing so could lead to more problems than it would solve.
This work was funded by AKC Canine Health Foundation Grant 640.
Scientific publication:
Greer, K.A., et al., Necrotizing meningoencephalitis of Pug Dogs associates with dog leukocyte antigen class II and resembles acute variant forms of multiple sclerosis. Tissue Antigens, 2010. 76(2): p. 110-118.
From the source, Dr. Kimberly Greer:
Thank you for your continued interest! I have moved the laboratory to Indiana, and would be most appreciative if you could post my newer contact information for your members. The address, office and email are below. My faculty page is: faculty web pages are a work in progress, but the basics are there! I hope to have a full research site very soon.

As for a research update, I am in the midst of submitting the most recent manuscript. It should be far enough through the process that I can give you a most up-to-date review in July. Please write me again with a reminder in July, and I can send you a sum of the work. 

For the time being, I am still accepting Pugs into the study. Our AKC grant has been approved for the next year so the research can move along. Our cremation and shipping donation fund is drained, however. Since I am not allowed to use the AKC grant money for cremation or for sending Pugs to cremation, I must find donations to provide these services for the Pugs and their owners. 

Basically, I am humbly asking Pug parents for donations to keep the PDE victims able to be involved in the studies. We all know that without having cremation and without the Pugs returning to their homes (where they belong!) with their owners, they cannot be a part of the ongoing research. The grant strictly prohibits payment of shipping the Pugs to the lab, cremation, or return shipment for them. Pug owners have graciously kept this rolling-fund (reserved for shipments and cremation of Pugs) just-filled-enough to keep the little guys "moving" for 6.5 years! Aren't Pug owners wonderful?! With the economy down, it's understandable that people are cutting back, but I must ask for people to remember the research if even just a little bit, so we can collectively help those who are currently suffering from PDE.

In the last two publications, which I will attach, we were able to show that the spinal tap does not reliably tell us whether a Pug has PDE or not. The spinal tap is no longer recommended for Pugs who are having encephalitis problems. We also know that the only medication helping the Pugs with PDE survive longer is the anticonvulsant. While many of them have prednisone and may also take other medications, it is the anti-seizure medication that is making the most difference for the Pugs with PDE. We have evaluated the seasonal effects and geographical effects as well, and neither of these are important for PDE. This means that no matter where you live or what type of climate you live in, it does not help or hurt your Pug in terms of getting PDE.

Thank you for the note, Amanda. I appreciate your organization of all the Pug parents. Please send my regards to all-

Kimberly A. Greer, Ph.D.
Assistant Professor
School of Natural Sciences and Mathematics
Indiana University East
2325 Chester Blvd.
Richmond, IN 47374
Office: 765-973-8445
Fax: 765-973-8430