Carbamazepine trial for Lesch-Nyhan self-mutilation

E. S. Roach, Mauricio Delgado, Louise Anderson, Susan T. Iannaccone, Dennis K. Burns

Research output: Contribution to journalArticle

23 Citations (Scopus)

Abstract

Anticonvulsants may reduce the self-mutilation of acquired sensory neuropathy, and one-report described sensory neuropathy in an older patient with Lesch-Nyhan syndrome. We performed nerve and muscle biopsies on four patients with Lesch-Nyhan syndrome and initiated an uncontrolled pilot trial to see if carbamazepine would reduce the self-mutilation in these patients. All of the boys had clinical features typical of Lesch-Nyhan syndrome, and the diagnosis was confirmed in each by enzyme analysis. No specific abnormalities were identified in either nerve or muscle. Nevertheless, self- mutilation and the need for constant restraint diminished in all four patients, though in one the effect was only transient. Two patients had increased self-mutilation when carbamazepine was stopped, then improved a second time when treatment was restarted. Sensory neuropathy was not continued, so any effect of carbamazepine is likely to be on the central nervous system.

Original languageEnglish (US)
Pages (from-to)476-478
Number of pages3
JournalJournal of Child Neurology
Volume11
Issue number6
StatePublished - Nov 1996

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Self Mutilation
Carbamazepine
Lesch-Nyhan Syndrome
Muscles
Anticonvulsants
Central Nervous System
Biopsy
Enzymes

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health

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Carbamazepine trial for Lesch-Nyhan self-mutilation. / Roach, E. S.; Delgado, Mauricio; Anderson, Louise; Iannaccone, Susan T.; Burns, Dennis K.

In: Journal of Child Neurology, Vol. 11, No. 6, 11.1996, p. 476-478.

Research output: Contribution to journalArticle

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