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. Five patients (four infants and one adolescent) with nemaline myopathy received dietary supplementation with L-tyrosine (250–3000 mg/day). All four infants were reported to have an initial decrease in sialorrhoea and increase in energy levels. The adolescent showed improved strength and exercise tolerance.
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