Disorder	Organ or system affected	Signs of metabolic instability	Neurodevelopmental effects (if not treated)
Carnitine transport defect	CNS, cardiovascular	Low carnitine levels	Developmental delay, motor deficits, muscle weakness
Citrullinemia type I (carnitine palmityl transferase type I deficiency)	CNS	Hypoglycemia	Developmental delay, motor deficits
Citrullinemia type II (carnitine palmityl transferase type II deficiency)	CNS, cardiovascular	Hypoglycemia	Neonatal presentation: severe mental retardation or death
			Later onset: muscle weakness
Glutaric acidemia type II	CNS	Hypoglycemia	Severe motor deficits or mental retardation
Short-chain acyl-CoA dehydrogenase deficiency	CNS	Hypoglycemia, acidosis	Developmental delay, motor deficits, potential for mental retardation
Medium-chain acyl-CoA dehydrogenase deficiency	CNS	Hypoglycemia, acidosis	Developmental delay, motor deficits, potential for mental retardation
Long-chain acyl-CoA dehydrogenase deficiency	CNS, eye, cardiovascular	Hypoglycemia	Developmental delay, motor deficits, potential for mental retardation, poor vision
Very long-chain acyl-CoA dehydrogenase deficiency	CNS, cardiovascular	Hypoglycemia	Developmental delay, motor deficits, potential for mental retardation