Abstract
A most disturbing aspect of Lesch-Nyhan disease is self-mutilation which can lead to loss of fingers and lip tissue. We have previously reported that the children can be trained not to self-injure using positive reinforcement and time-out procedures. An unexpected observation was that they failed to learn from aversive stimulation suggesting the possibility that this may be primary to the behavior disorder. We have now demonstrated in 3 L.N. children, ages 7, 12 and 13 years, that 20 μg of 1-Deamino-8-D-Arginine-Vasopressin (DDAVP), a vasopressin analogue, administered one hour prior to testing will partially restore the ability to learn from aversive stimulation. This approach was suggested by reports of behavioral studies on the vasopressin deficient rat.
The patients were taught to press a lever to receive a reward of a penny. The mean rate was 2.8/min. When lever-pressing was coupled with a finger shock (0.5 ma for 0.5 seconds) the mean rate was 3.4/min confirming under controlled conditions a defect in passive avoidance learning. When DDAVP was administered, the mean rate was 4.0/min without shock and 0.3/min with shock The experimental observations were repeated three or four times over a period of weeks in each child with consistent results. The effect of one dose lasted approximately 24 to 36 hours.
Log in or create a free account to read this content
Gain free access to this article, as well as selected content from this journal and more on nature.com
or
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Anderson, L., David, R., Bonnet, K. et al. 24 THE EFFECT OF DDAVP ON PASSIVE AVOIDANCE LEARNING IN LESCH-NYHAN DISEASE. Pediatr Res 12 (Suppl 4), 367 (1978). https://doi.org/10.1203/00006450-197804001-00029
Issue date:
DOI: https://doi.org/10.1203/00006450-197804001-00029
