Dopamine deficiency syndrome in children with a special reaction to treatment with levodopa preparations.

The authors describe the dopamine deficiency syndrome in children with the disease beginning during their first year and unusual dystonia symptoms resulting in total immobilization and speech loss. All symptoms of the disease can be eliminated by low doses of nakom but reappear on withdrawal of the drug. Tyrosine hydroxylase studies made on these patients at various stages of the disease showed a different pattern of enzyme activity from that observed in children with similar pathology failing to improve dramatically under nakom treatment.