Table 8.

Additional Treatments for Individuals with HPRT1 Disorders: LND

Physical protective devices (restraints)
  • Required by virtually all persons at some point; often required daily
  • LND is exempt from laws that prevent use of restraints for extended periods.
Behavior therapy
  • Negative reinforcement methods (punishment) worsen behavior & should not be used.
  • Extinction methods work best, & require special expertise to implement.
Pharmacologic interventions
  • No medications reliably control self injury in LND.
  • Benzodiazepines often used to ↓ anxiety
  • Neuroleptics may worsen the dystonia.
Dental interventions
  • Tooth & mouth guards rarely reliable
  • Dental extraction required in the majority
Other difficult
Behavior therapyExtinction methods sometimes required to suppress other negative behaviors incl hitting, spitting, or foul language
Social worker interventionMany families benefit from online chat services where families share experiences.

LND = Lesch-Nyhan disease

From: HPRT1 Disorders

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