Dietary L-Tyrosine supplementation in nemaline myopathy

Monique M. Ryan, Catherine Sy, Sian Rudge, Carolyn Ellaway, David Ketteridge, Laurence G. Roddick, Susan T. Iannaccone, Andrew J. Kornberg, Kathryn N. North

Research output: Contribution to journalArticlepeer-review

51 Scopus citations

Abstract

Nemaline myopathy is defined by the presence of nemaline bodies, or rods, on muscle biopsy. Facial and bulbar weakness in nemaline myopathy cause chewing and swallowing difficulties, recurrent aspiration, and poor control of oral secretions. This article discusses 5 patients (4 infants and 1 adolescent) with nemaline myopathy who received dietary supplementation with L-tyrosine (250 to 3000 mg/day). All 4 infants were reported to have an initial decrease in sialorrhoea and an increase in energy levels. The adolescent showed improved strength and exercise tolerance. No adverse effects of treatment were observed. Dietary tyrosine supplementation may improve bulbar function, activity levels, and exercise tolerance in nemaline myopathy.

Original languageEnglish (US)
Pages (from-to)609-613
Number of pages5
JournalJournal of child neurology
Volume23
Issue number6
DOIs
StatePublished - Jun 2008

Keywords

  • Myopathy
  • Nemaline
  • Sialorrhoea
  • Tyrosine

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

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