We investigated the therapeutic effects of glycine in seven patients with various forms of myoclonus. The initial phase was an open label trial. If benefit was seen in any patient, a double-blind substitution of placebo was carried out to determine if the benefit was due, in fact, to glycine. The dosage of glycine was initiated at 600 mg/day and was increased gradually until a maximum dosage of 6,000 mg/day was reached. This dosage was maintained for at least 6 weeks before lack of efficacy was declared. No improvement was seen in four patients. One patient reported improvement, but he discontinued the drug because of adverse effects encountered with the use of a concomitant medication and before he could be tested in a double-blind crossover phase. Two other patients also noticed improvement, but these improvements were not validated in the crossover phase. There were no adverse effects associated with glycine. Plasma glycine levels peaked at 30 min and returned to normal 1.5-h after 1 g of glycine.p.o. Cerebrospinal fluid (CSF) glycine levels did not change during treatment, suggesting inadequate penetration into the central nervous system of glycine at the dosage used.