Detection of behavioural and cognitive dysfunction in mucopolysaccharidosis IIIA affected dogs : a thesis presented in partial fulfilment of the requirements for the degree of Master of Veterinary Science at Massey University, Palmerston North, New Zealand

Abstract

This study investigated whether behavioural and cognitive dysfunction caused by mucopolysaccharidosis (MPS) IIIA can be detected early in affected dogs’ lives, and to describe the behaviours of these dogs. No other scientific papers have been published on this topic and the population of dogs examined in this study are the only MPS IIIA affected dog colony available worldwide for study. Three main tests were performed on the population of MPS IIIA affected dogs. Physical behavioural assessment tests were performed at six and eight weeks of age and from twenty weeks of age a cognitive function task was taught and then tested to measure the dogs’ performance. A previously validated questionnaire, the canine behavioural assessment and research questionnaire (C-BARQ), was completed at three, six and twelve months of age. The researchers in these studies were blinded to the MPS IIIA status of the dogs examined. The behaviours shown by the MPS IIIA puppies at six and eight weeks of age were not significantly different from the behaviours of the unaffected puppies. This finding supported the research of other MPS IIIA studies and suggests that clinical behavioural changes do not occur at such a young age. The behaviours shown by the MPS IIIA affected puppies appeared to be normal puppy behaviours similar to those described in previous research on puppies. The C-BARQ measured the behaviours shown by the MPS IIIA affected and unaffected dogs. Most of the MPS IIIA affected dogs’ behaviours were not significantly different from the unaffected dogs’ behaviours, but MPS IIIA affected dogs did retrieve significantly more than unaffected dogs at three months of age, and were less distractible at twelve months of age. It would be worth investigating these findings further to decide whether it suggests a subtle alteration in brain functioning. The cognitive function test showed a significant decrease in the success of the MPS IIIA affected dogs in the final maze test. This is the first study on dogs affected with MPS IIIA to find a decline in cognitive function before the occurrence of cerebellar clinical signs and this new knowledge may lead to future developments measuring therapy response and disease progression. The T-shaped maze testing may be valuable in future research on cognitive function in dogs with other diseases such as epilepsy. Thus this thesis provides valuable information on canine MPS IIIA and provides a foundation for future disease investigations.