Botulinum toxin as a novel treatment for self-mutilation in Lesch-Nyhan syndrome.

@article{Dabrowski2005BotulinumTA,
  title={Botulinum toxin as a novel treatment for self-mutilation in Lesch-Nyhan syndrome.},
  author={Edward Dabrowski and Sarah A Smathers and Curt S Ralstrom and Michael A Nigro and Jimmie P Leleszi},
  journal={Developmental medicine and child neurology},
  year={2005},
  volume={47 9},
  pages={636-9}
}
Lesch-Nyhan syndrome (LNS) is an X-linked recessive disorder resulting from a deficiency of the metabolic enzyme hypozanthine-guanine phosphoribosyltransferase (HPRT). This syndrome presents with abnormal metabolic and neurological manifestations including hyperuricemia, mental retardation*, spastic cerebral palsy (CP), dystonia, and self-mutilation. The mechanism behind the severe self-mutilating behavior exhibited by patients with LNS is unknown and remains one of the greatest obstacles in… CONTINUE READING

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Molecular syndromology • 2016
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