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This version was published on April 1, 2008
Journal of Child Neurology, Vol. 23, No. 4, 381-388 (2008)
DOI: 10.1177/0883073807309236

Treatment of Life-Threatening Self-Injurious Behavior Secondary to Hereditary Sensory and Autonomic Neuropathy Type II: A Controlled Case Study

David Kuhn, PhD

Johns Hopkins University School of Medicine, Baltimore, Maryland (DK, LH); Kennedy Krieger Institute, Baltimore, Maryland, kuhn{at}kennedykrieger.org

Louis Hagopian, PhD

Johns Hopkins University School of Medicine, Baltimore, Maryland (DK, LH); Kennedy Krieger Institute, Baltimore, Maryland

Cindy Terlonge, BA

Johns Hopkins University School of Medicine, Baltimore, Maryland (DK, LH); Kennedy Krieger Institute, Baltimore, Maryland, Louisiana State University, Baton Rouge, Louisiana

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.

Key Words: habit reversal • self-injurious behavior • hereditary sensory and autonomic neuropathy


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