Polyneuropathy
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John Williams
Professor Shelton - UCLA
Myelos Corporation

   

 

Polyneuropathy (Harvey's Disease)

 

There are lots of good web sites around that will give you loads of information about Leo health issues. This page is simply designed to bring together information available on the web about a relatively newly described condition in Leos: Polyneuropathy (PN) also called 'Harvey's Disease'. Harvey was the first Leo to have been formally diagnosed with the condition in 1999. You can read more about Harvey's case by clicking here - this will take you to the University of California San Diego's web site and a summary of Harvey's case.

 

PN is a progressive degenerative neuromuscular disorder. The nerves which supply some of the major muscles in the body gradually lose their efficiency. The laryngeal nerve is often the first to be affected because it is the longest nerve in the body. In people it shares similarities with Charcot Marie Tooth Disease. John Williams, Jenga's veterinary surgeon, has provided an introduction to this condition ... click here for PN in Leonbergers.

 

The best regime for testing for PN is as follows:

  1. A complete physical and neurological examination.

  2. Blood work including CBC, chemistry screen and endocrine testing to rule out an underlying systemic disease.

  3. EMG/NCV

  4. Muscle and peripheral nerve biopsy under the same anesthesia.

 

A biopsy confirms the problem, but there are not many   laboratories in the world that resin embed the nerve biopsies. Paraffin embedded nerve biopsies are practically worthless and that is what most veterinary laboratories do.

 

If the choice is between EMG/NCV and paraffin embedded nerve sections, choose EMG/NCV. However, ideally all should be done.

Jenga found out about PN the hard way and is determined to do his best to ensure as few as possible of his community suffer. This is what he had to say of his recent experience. 

"I was two years old last November. Boss always thought I was a bit of a 'panty' dog. I always struggled a bit up them big hills on the moors where he took me walkies, but soon recovered.

I started water training last March with a bunch of great Newfies and a few Leos. It was hard work but great fun and you can't keep me out of water now! Some of the folk at the training seemed a bit concerned about me breathing. I got tired quick and stumbled often - me feet collapsed under me.

In May 2003 me and the family went for a week camping together on the Norfolk coast. Fab weather and we had a great time, but I was'nt in for any long walks.

On the way back we stopped off at Midge's to see Bear (me bruvver) and the lovely  Rika. Rika is this gorgeous Swedish dream that had recently joined the Midge pack. Ooooooooooo!! Rika!!!!! I went all gooooey and weak at the knees.  I got soooo excited!! Problem was me breathin also got out of hand - like I started hyperventilatin and it took me ages to calm down. Got Boss and Midge real worried.

Wen we got back, Boss took me down to see Eddie me vet. I had a chest X-ray, ECG, blood tests for thyroid function and loads of prodding and poking. I had to warn Eddie that I was'nt havin none of that thermometer up me bum again. In the end I had this endoscopy thing while I had a nap and Eddie said I had some unpronounceable Latin problem - larry parry or sum such thing. By this time I was pretty useless - could'nt walk nowhere to speak of - just breathless all the time and knackered.

Boss did a lot of ringing round and talking to folk. Turns out my grandad had this larry parry problem. Eventually Boss took me to see John, a specialist surgeon who done loads of these larry parry operations. John was instantly more concerned about why I got larry parry in the first place. He did'nt like my back end much, which I though a cheek cos I think it's quite pretty! But it was more the way it moved - high stepping gait he said.

John had'nt met many Leos before, but he was brill. Within hours he's got the situation sussed and spoke with this Dr Shelton - she's got all the low down on this PN thing. Lives over in California. It seems they much more aware of this PN thing over there.

I had me larry parry op which was fine. John took a bit of me leg too! He said it was for Dr Shelton - so a bit of me has been to America! The biopsy wound was a bit of a problem. Me leg swelled up like a balloon and were real painful at times but calmed down eventually. Just waitin for me mane to get back into shape - can't have Rika seein me like dis!

Hospital was a cool place. Dem vet students could'nt even recognise a Leo! Nearest one got was: 'He's a dog!'

Now I is bouncin bout the place. Still make some orrible throaty noises and soon get worn out.

This PN thing that John and Dr Shelton says I got I don't much like the sound of and nobody seems to know what'll happen next. But hey! I'm havin a great time just now so I'll leave that for another day.

Am thinkin about keeping a diary of me adventures and maybe post it here - so ta ta for now."

Woof! Woof!

        Wurf! Wharf!

 

 

   

 

Harvey at 19 months

Characteristics of Harvey's Disease

 

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Muscle atrophy - leading to mobility problems

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Exercise intolerance - easily tired out by mild exercise

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An abnormal high-stepping gait

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Tremor

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Changes in bark, wheezing, and coughing or choking while drinking or eating. These may be signs of laryngeal paralysis.

Harvey's Disease is a complex polyneuropathy (PN)/laryngeal paralysis (LP) condition. The only way of confirming the condition is by way of a muscle and/or nerve biopsy.

Dr Shelton is conducting an on-going research study to identify a DNA test that will identify LP/PN carriers and affected dogs.

It is critical that post-mortem biopsies be performed on all affected dogs. A biopsy requires the following from your veterinarian: cranial tibial muscle and peroneal nerve, preserved in 10% buffered formalin. Please send this to:

Dr Diane Shelton
Department of Pathology
University of California,

San Diego
La Jolla, CA 92093-0612

Click here for full details of what is needed.

Support and Information

There is a Yahoo List support network for breeders and owners of Leonbergers affected by Harvey's Disease. E-mail Jenga if you want to join and he will forward your request.

There is also a more established Yahoo List for breeders and owners of any dog affected by laryngeal paralysis, again click here for more details. The Southern California Veterinary Surgical Group also has an excellent explanation and description of laryngeal paralysis - click here.

Leo breeders and owners have a responsibility to be aware, open and honest about all health issues affecting this wonderful breed. In the case of Harvey's Disease this is absolutely critical. If we are, it can be defeated and driven out. So let's do it!

 

 
   
   

This site was last updated 10 October 2004