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Journal of Child Neurology
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0883073807309794v1
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Article

Dietary L-Tyrosine Supplementation in Nemaline Myopathy

Monique M. Ryan, M Med, Catherine Sy, MB BS, Sian Rudge, BSc, Carolyn Ellaway, PhD, David Ketteridge, MB BS, Laurence G. Roddick, MB BS, Susan T. Iannaccone, MD, Andrew J. Kornberg, MB BS, and Kathryn N. North, MD*

* To whom correspondence should be addressed. E-mail: Kathryn{at}chw.edu.au.


  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.

First published on December 13, 2007, doi:10.1177/0883073807309794

Journal of Child Neurology 2008;23:609.

A more recent version of this article appeared on June 1, 2008

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