Aromatic L-amino acid decarboxylase deficiency

ICD-10 Code E70.81

Aromatic L-amino acid decarboxylase deficiency

Definition

Aromatic L-amino acid decarboxylase deficiency is a very rare, severe, genetic neurometabolic disorder associated with clinical manifestations related to underproduction of serotonin and dopamine, mainly hypotonia, hypokinesia, ptosis oculogyric crises, and signs of autonomic dysfunction.

Also known as AADC deficiency, aromatic L-amino acid decarboxylase deficiency, aromatic L-amino-acid decarboxylase deficiency — per MONDO

Also identified as