Fast Five Quiz: Aromatic L-Amino Acid Decarboxylase Deficiency Diagnosis and Management

Stephen L. Nelson, Jr, MD, PhD

Disclosures

September 18, 2020

Dopamine agonists, preferably non–ergot-derived, should be considered to treat patients with AADC deficiency. Dopamine agonists directly activate postsynaptic dopamine receptors. Because ergot-derived agonists with strong serotonergic (5-HT2b) agonist action are strongly associated with cardiac valvulopathy and other fibrotic complications, these types of dopamine agonists should not be used to treat patients with AADC deficiency. Non–ergot-derived dopamine agonists, on the other hand, probably have very low risk of fibrous complications.

Although ergot-derived dopamine agonists without 5-HT2b agonist action have a lower risk than those with 5-HT2b agonist action, cardiac screening should be carried out before and during treatment to monitor for cardiac fibrosis.

Learn more about AADC deficiency treatment.

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