This report presents the case of an adult male with aromatic l-amino acid decarboxylase deficiency who developed serious cardiac rhythm disturbances
during treatment with intravenous dopamine and norepinephrine for severe hypotension.
Three weeks later, he spontaneously developed atrial fibrillation while not receiving
exogenous catecholamines. He died suddenly after several months. We presume cardiac
arrhythmia was the most likely cause of his death. Patients with aromatic l-amino acid decarboxylase deficiency may be prone to cardiac arrhythmias at rest and
also may be exceptionally sensitive to exogenous catecholamines. Therefore, close
cardiac monitoring is indicated at baseline and during treatment with pressors.
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References
- Aromatic l-amino acid decarboxylase deficiency.Neurology. 1992; 42: 1980-1988
- Aromatic l-amino acid decarboxylase deficiency.J Inherit Metab Dis. 1998; 21: 240-242
- Aromatic l-amino acid decarboxylase deficiency.Ann Neurol. 2003; 54: S49-S55
- Aromatic l-amino acid decarboxylase deficiency.Neurology. 2004; 62: 1058-1065
- Aromatic l-amino acid decarboxylase deficiency.J Child Neurol. 1997; 12: 349-354
- Aromatic l-amino acid decarboxylase deficiency.Eur J Paediatr Neurol. 1997; 1: 67-71
- Clinical and therapeutic observations in aromatic l-amino acid decarboxylase deficiency.Neurology. 1999; 53: 1205-1211
- Aromatic l-amino acid decarboxylase deficiency with hyperdopaminuria.Neuropediatrics. 2002; 33: 203-208
- The influence of l-dopa on methylation capacity in aromatic l-amino acid decarboxylase deficiency.J Inherit Metab Dis. 2000; 23: 321-324
- Levodopa-responsive aromatic l-amino acid decarboxylase deficiency.Ann Neurol. 2004; 55: 435-438
Article info
Publication history
Accepted:
January 17,
2006
Received:
October 3,
2005
Identification
Copyright
© 2006 Elsevier Inc. Published by Elsevier Inc. All rights reserved.
