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Dietary L-Tyrosine Supplementation in Nemaline Myopathy

Neurosciences Department, The Royal Children's Hospital, Melbourne, Australia, Murdoch Children's Research Institute, Melbourne, Australia

Catherine Sy, MB BS

Western Sydney Genetics Program, The Children's Hospital at Westmead, Sydney, Australia

Sian Rudge, BSc

Institute for Neuromuscular Research, The Children's Hospital at Westmead, Sydney, Australia

Carolyn Ellaway, PhD

Western Sydney Genetics Program, The Children's Hospital at Westmead, Sydney, Australia

David Ketteridge, MB BS

Department of Paediatrics, The Women's and Children's Hospital, Adelaide, Australia

Laurence G. Roddick, MB BS

Department of Paediatrics, John Hunter Children's Hospital, Newcastle, Australia

Susan T. Iannaccone, MD

Department of Neuromuscular Disease and Neurorehabilitation, Texas Scottish Rite Hospital for Children, Dallas, Texas, USA

Andrew J. Kornberg, MB BS

Neurosciences Department, The Royal Children's Hospital, Melbourne, Australia, Murdoch Children's Research Institute, Melbourne, Australia

Kathryn N. North, MD

Institute for Neuromuscular Research, The Children's Hospital at Westmead, Sydney, Australia, kathryn{at}chw.edu.au, Discipline of Paediatrics and Child Health, The University of Sydney, Sydney, Australia

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.

Key Words: nemaline • myopathy • tyrosine • sialorrhoea

This version was published on June 1, 2008

Journal of Child Neurology, Vol. 23, No. 6, 609-613 (2008)
DOI: 10.1177/0883073807309794


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