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

doi:10.1177/0883073807309794

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 journal › Article › peer-review

45 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 language	English (US)
Pages (from-to)	609-613
Number of pages	5
Journal	Journal of child neurology
Volume	23
Issue number	6
DOIs	https://doi.org/10.1177/0883073807309794
State	Published - Jun 1 2008

Keywords

Myopathy
Nemaline
Sialorrhoea
Tyrosine

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health
Clinical Neurology

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10.1177/0883073807309794

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title = "Dietary L-Tyrosine supplementation in nemaline myopathy",

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.",

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AU - Ryan, Monique M.

AU - Sy, Catherine

AU - Rudge, Sian

AU - Ellaway, Carolyn

AU - Ketteridge, David

AU - Roddick, Laurence G.

AU - Iannaccone, Susan T.

AU - Kornberg, Andrew J.

AU - North, Kathryn N.

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N2 - 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.

AB - 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.

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Dietary L-Tyrosine supplementation in nemaline myopathy

Abstract

Keywords

ASJC Scopus subject areas

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