Table 3.

Medications used in PDE treatment [2,17].

Medication	Route of Administration	Dosage	Indication	Monitoring	Side Effects
Pyridoxine	i.v.	100 mg single dosage	Interruption of initial status epilepticus, or of prolonged breakthrough seizures	EEG if available	May result in respiratory arrest. Administer upon availability of respiratory support
Pyridoxine	Oral/enteral	15–30 mg/kg/day Div in up to 3 single doses Up to 300 mg/day in neonates and 500 mg/day in adults	Long-term treatment	Clinical and electrophysiological signs of neuropathy	Continue with dosages above the range only if high dosage has proven essential for effective seizure control
Pyridoxine	Prenatal maternal	100 mg/day	Prevention of intrauterine seizures and irreversible brain damage. Start in early pregnancy, continue throughout pregnancy in case of positive prenatal diagnosis or if no prenatal diagnosis has been performed	Monitor for seizures and encephalopathy after delivery in NICU/SCN setting. Consider IV pyridoxine in case of neonatal seizures	Continue oral/enteral pyridoxine supplementation at 30 mg/kg/day immediately after birth and immediately initiate biochemical and molecular genetic investigations to prove or rule out ATQ deficiency
Pyridoxal phosphate	Oral/enteral	30 mg/kg/day divided in up to 3 single dosages	Interruption of initial status epilepticus: additional to IV pyridoxine in case pyridoxine initially failed to control seizures. Long-term treatment: Alternative to pyridoxine	Same as pyridoxine (EEG if available)	Same as pyridoxine
Folinic acid	Oral/enteral	3–5 mg/kg/day divided in up to 3 SD	Additional therapy if pyridoxine or PLP failed to control seizures	No particular monitoring	None

Abbreviations: i.v. = intravenous administration; div = divided; NICU = Neonatal Intensive Care Unit; SCN = Special Care Nursery; SD = single dosage.