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 | |
| State | Published - Jun 2008 |
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Keywords
- Myopathy
- Nemaline
- Sialorrhoea
- Tyrosine
ASJC Scopus subject areas
- Clinical Neurology
- Pediatrics, Perinatology, and Child Health
Cite this
Dietary L-Tyrosine supplementation in nemaline myopathy. / Ryan, Monique M.; Sy, Catherine; Rudge, Sian; Ellaway, Carolyn; Ketteridge, David; Roddick, Laurence G.; Iannaccone, Susan T.; Kornberg, Andrew J.; North, Kathryn N.
In: Journal of Child Neurology, Vol. 23, No. 6, 06.2008, p. 609-613.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Dietary L-Tyrosine supplementation in nemaline myopathy
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.
PY - 2008/6
Y1 - 2008/6
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.
KW - Myopathy
KW - Nemaline
KW - Sialorrhoea
KW - Tyrosine
UR - http://www.scopus.com/inward/record.url?scp=44149126988&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=44149126988&partnerID=8YFLogxK
U2 - 10.1177/0883073807309794
DO - 10.1177/0883073807309794
M3 - Article
C2 - 18079309
AN - SCOPUS:44149126988
VL - 23
SP - 609
EP - 613
JO - Journal of Child Neurology
JF - Journal of Child Neurology
SN - 0883-0738
IS - 6
ER -