Mucopolysaccharidosis Type IIIB (Discovered in the Schipperke)

Clinical signs appear in early adulthood between 2 and 4 years of age, and are typically localized to the cerebellum (tremors, wide-based stance, poor balance, cerebellar ataxia). Progression of the disease leads to generalized ataxia, loss of muscle control and mental dullness. Ocular anomalies include corneal clouding and focal retinal degeneration. Most dogs are euthanized by 6 years of age due to the severity of their neurologic deficits.

As there is no cure for this disorder, therapy is limited to symptomatic treatments and general supportive care. The average prognosis is considered poor for affected dogs and euthanasia is often considered at or by 6 years of age.

There are many responsibilities to consider when breeding dogs. Regardless of test results it is important that your dog is in good general health and that you are in a position to care for the puppies if new responsible owners are not found. For first time or novice breeders, advice can be found at most kennel club websites.

This disease is autosomal recessive meaning that two copies of the mutation are needed for disease signs to occur. A carrier dog with one copy of the MPS IIIB mutation can be safely bred with a clear dog with no copies of the MPS IIIB mutation. About half of the puppies will have one copy (carriers) and half will have no copies of the MPS IIIB mutation. Puppies in a litter which is expected to contain carriers should be tested prior to breeding. Carrier to carrier matings are not advised as the resulting litter may contain affected puppies. Please note: It is possible that disease signs similar to the ones caused by the MPS IIIB mutation could develop due to a different genetic or clinical cause.