May 2007 – AMSC Health Committee

Canine Neuronal Ceroid-Lipofuscinosis (NCL) is a neurologic disorder that has been reported in the Miniature Schnauzer and several other breeds of dog. The age of onset and symptoms can vary between breeds, and in some breeds the scant amount of information available may not reflect all possible symptoms.

In the Miniature Schnauzer the average age of onset is between 2-4 years. Symptoms can include visual abnormalities, rapid changes in vision including blindness, bumping into objects, confusion, aimless wandering, loss of memory for learned tasks, and trembling episodes.

In some other breeds the symptoms are more dramatic, including gait changes, diminished coordination, wide-based stance, goose-stepping, aggression, compulsive pacing, circling, and changes in appetite and thirst. .
Due to limited available data it is not clear at this time if any of these symptoms may also be found in affected dogs in our breed.

In 2006 the AMSC co-sponsored research to identify the genetic mutation responsible for this defect by contributing funds from the club’s Canine Health Foundation Donor-Advised Fund. Please visit the link below for more information on this devastating disorder and for information on submitting samples from affected Miniature Schnauzers to assist the research efforts:

www.caninegeneticdiseases.net/CL_site/mainCL.htm

NCL Update 2008    

Samples Needed:

Unfortunately, the definitive diagnosis of NCL in our breed can only be done post-mortem.  In a recent update from the researcher Dr. Katz states DNA samples are needed from affected dogs.  He recommends owners of potentially affected dogs with similar clinical signs to NCL that have had other diseases ruled out should contact his team to submit a blood sample.  This sample can be stored by the team and DNA can be extracted at a later date, if the dog is diagnosed with NCL post-mortem.  He also encourages owners of potentially affected dogs to contact him prior to euthanasia or death so information on sample and tissue submission can be obtained.  This will enable the team to make an accurate diagnosis and include the DNA in their study in their attempt to identify the causative genetic mutation(s) for this disorder.