Related Experiment Video
Updated: Jul 8, 2026
Establishing a Mouse Model of a Pure Small Fiber Neuropathy with the Ultrapotent Agonist of Transient Receptor Potential Vanilloid Type 1
Published on: February 13, 2018
Treatment of life-threatening self-injurious behavior secondary to hereditary sensory and autonomic neuropathy type
David Kuhn1, Louis Hagopian, Cindy Terlonge
1Johns Hopkins University School of Medicine, Baltimore, MD, USA. kuhn@kennedykrieger.org
Abstract:
Although self-injurious behavior is present in all subtypes of hereditary sensory and autonomic neuropathy, the literature has not sufficiently addressed the issue of treatment of self-injury in this population. Therefore, the purpose of the current case study was to describe a method for assessing and treating self-injurious behavior associated with hereditary sensory and autonomic neuropathies. This study was conducted with an 11-year-old boy diagnosed with hereditary sensory and autonomic neuropathy type II admitted to an inpatient behavioral unit over a 4-month period. A simplified version of a habit reversal treatment was used, consisting of awareness training, self-monitoring, competing responses, and social support. Treatment resulted in a 98% reduction in the rate of self-injurious behavior relative to pretreatment baseline rates. This case study illustrates that behavioral interventions may be a viable option for treating self-injury secondary to hereditary sensory and autonomic neuropathies.
