Abstract
The characteristics common to both the pyridoxine-deficiency and the pyridoxine-dependency syndromes in infants are convulsions exclusively controlled by pyridoxine. The author reviews the clinical literature pertaining to these syndromes and gives a brief introduction to their biochemical and metabolic aspects. The differences and similarities between the two syndromes are discussed and certain clinical implications are noted. The author particularly emphasizes the importance of a diagnostic trial with a single intramuscular injection of 50 to 100 mg. pyridoxine in cases of idiopathic neonatal seizure. Clinical observation and alertness to the possible existence of this disorder are the principal means of diagnosis.