NEUROMUSCULAR CASE OF THE MONTH - AUGUST 1999

Alert! New peripheral neuropathy in Leonberger dogs
Contributed by Dr. G. Diane Shelton DVM, PhD
Diplomate ACMIM (Internal Medicine)



A peripheral neuropathy with onset between 1-2 years of age has recently been recognized in Leonberger dogs. As a manuscript is in preparation, only a brief description of this neuropathy will be given. For those who are not familiar with this breed, the Leonberger was established in 1846 in Leonberg in the region of Wurttemberg by the German breeder Einrich Essig from a crossing of the Newfoundland, the St. Bernard, and the Great Pyrenees. Qualities used to describe the Leonberger have been balance, calm and reflectiveness, good guarding, non-aggressiveness, liveliness, dynamism, and knowing how to be man's companion (From: The Leonberger by Guido Perosino, 1998). The first dog we are aware of  with this progressive neuropathy is Harvey (Fig. 1) and his story follows.

Clinical History
The owner's had Harvey since he was 11 weeks of age and he lived happily with 5 other dogs until at 1 year of age he developed a laryngeal paralysis. A surgical correction was performed (arytenoid cartilage lateralization). Over the next year Harvey developed an incoordinated gait, had exercise intolerance to the point that he would quit walking after 100-200 feet, and had problems swallowing. He was evaluated by the Neurology Service at The Ohio State University and was diagnosed with a severe polyneuropathy.  Harvey became progressively weaker and developed significant generalized muscle atrophy. The weakness progressed until by 4 years of age he was tetraplegic (Fig. 2) with the ability only to crawl, sometimes sit up, and hold up his head. Harvey died quietly at home at 5 years of age. Muscle and peripheral nerve specimens taken shortly following his death were consistent with a severe peripheral neuropathy. Since that time, other young, male Leonberger dogs have been confirmed with this breed-related disorder. Metabolic evaluations are currently in progress on a large group of Leonberger dogs and the inheritance pattern investigated. Results of electrophysiologic testing, pathological abnormalities, metabolic screening and inheritance patterns will be described fully in a scientific manuscript.


Figure 1. Harvey
at 19 months of age



Figure 2. Harvey was
tetraplegic by 4 years of age


Update:
Scientific Paper now in Press
Shelton GD, Podell M, Poncelet L, Schatzberg S, Patterson E, Powell HC, Mizism AP (2003) Inherited polyneuropathy in Leonberger dogs: a mixed or inherited form of Charcot-Marie-Tooth. Muscle Nerve (in press).

Back to Top