Leaflet on ILPN - Inherited Leonberger Polyneuropathy

 

 

Definition
ILPN is a degenerative disease of the peripheral nervous system.  Affected are nervus peronaeus (hind leg), nervus recurrens (larynx) and musculus tibialisranialis (hind leg).

Symtomes

§  Paralysis of the vocal ligaments = larynx paralysis (LP)

§  Intense respiratory noise, coughing after eating or drinking, hoarse and changed barking, breathlessness.

§  PN (Polyneuropathia)

§  Uncoordinated hind leg, limping with hind legs with trailing of the toenails, cock's treadle, weak hint leg, amyotrophia.

Appearance

§  Before the first year of life = early ILPN, fast worsening

§  After five years = late ILPN, slowly worsening

Progress

Degeneration of the nervus peronaeus of the hint leg and caused by this the non adequate stimulation of the muscles which results in minimized muscles of the hint leg.  The dog looses power and agility, stumbles and buckles.  The dog does not suffer from pain, but from the lesser quality of life.  There is no possibility to cure.

The paralysis of the vocal cords embeds the danger of swallowing and as a consequence aspiration pneumonia shows up and finally suffocation!

Distribution

The Leonberger PN occurs in all countries.  Unfortunately the destructive genes are not yet known and thus they way of the inheritance still lays in the dark.  Countries with a tight genetic basis (inbreeding/line breeding), show more PN-cases.

Research project USA/CH

The USA started their breeding with some dogs from Europe.  Their genetic basis was not as wide spread as in Europe.  Thus, it was there when die inheritance of the disease was first detected.  In the year 2000 Ms. Dr. D. Shelton began her research on ILPN and published some articles.

2007 the Vetsuisse University of Berne started the research, mainly with diseased Swiss Leonberger.  Today the USA and Switzerland are jointly researching for the causal gene or genes.

The goal of their work is a DNA-test that enables dog owners and breeders to detect bearers or PN-free dogs.

Blood samples

The blood and the anamnesis of dogs with PN-symptoms are the basis for the research, but also the blood of healthy dogs is helpful.  The genome is analyzed by latest means.  Berne but also Minnesota very urgently need blood samples.  The addresses of Berne and the USA are stated in the attachment.

Biopsy

The most secure diagnosis is only possible from biopsies of the nervus peronaeus and the musculus tibialis cranialis.  These biopsies can be taken by experienced neurosurgeons from a vivid dog or post mortem (after the death of the Leo).  The neuropathological institutes of Prof. Dr. D. Shelton, San Diego and Prof. Dr. Th. Bilzer, Düsseldorf, can detect mutations of the sample.  For the purpose of comparison also samples of healthy dogs are very important.

Genetic test and breed planning

If  a DNA-test can be developed, we will be able to detect whether the Leo is a bearer or PN-free.  The moment we will have a reliable test, all dogs used for breeding will have to pass this test before used for breeding.  In order to gain control over this disease it is necessary that in the near future only PN-bearers and PN-free dogs may be mate.  PN-ill dogs have to be excluded.  The moment there will be enough PN-free Leos, the breeding with PN-bearers will be forbidden.  Genetisists say that this is the way to eliminate this disease within 2 - 3 generations.

Since 2009, the Swiss breeding regulations require blood samples from all stud dogs and their offsprings.  The time a reliable DNA-test will be available, all blood samples, stored in Berne, will be examined and the result announced to the dog owner.

More info: http://leonbergerunion.com