Lesch-Nyhan syndrome: treatment

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Lesch-Nyhan syndrome: treatment. Behavioural therapy, S-adenosylmethionine (SAMe), Physical restraints. Fully referenced neurology checklist.

Treatments to prevent self-injury

  • Behavioural therapy
  • S-adenosylmethionine (SAMe)
  • Physical restraints
  • Intraoral appliances
  • Botulinum toxin
  • Tooth extraction
  • Deep brain stimulation (DBS)

Treatment to reduce uric acid level: Allopurinol

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Other treatments

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References

  1. Ferrão J, Rodrigues Barros C, Figueiredo L, Fernandes A. Oral self-mutilation in Lesch-Nyhan syndrome: a case report. Cureus 2022; 14:e27874. 
  2. Hull M, Parnes M, Jankovic J. Botulinum neurotoxin injections in children with self-injurious behaviors. Toxins (Basel) 2023; 15:236.
  3. Torres RJ, Puig JG. Hypoxanthine-guanine phosophoribosyltransferase (HPRT) deficiency: Lesch-Nyhan syndrome. Orphanet J Rare Dis 2007; 2:48.
  4. Satow T, Ogawa M, Komuro T. Intrathecal baclofen therapy for Lesch-Nyhan disease: illustrative case. J Neurosurg Case Lessons 2021; 1:CASE202.

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