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J Child Neurol. 2008 Jun;23(6):609-13. Epub 2007 Dec 13.

Dietary L-tyrosine supplementation in nemaline myopathy.

Author information

1
Neurosciences Department, The Royal Children's Hospital, Melbourne, Australia.

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.

PMID:
18079309
DOI:
10.1177/0883073807309794
[Indexed for MEDLINE]

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