ABSTRACT
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.
Subject(s)
Dopamine/deficiency , Dystonia/drug therapy , Levodopa/therapeutic use , Adolescent , Adult , Child , Dystonia/blood , Dystonia/physiopathology , Female , Humans , Leukocytes/enzymology , Male , Muscle Hypotonia/drug therapy , Muscle Hypotonia/physiopathology , Syndrome , Tyrosine 3-Monooxygenase/bloodABSTRACT
The authors describe the dopamine deficiency syndrome in children with the disease beginning during their first year and peculiar dystonia++ symptoms resulting in a total immobilization and speech loss. All the symptoms of the disease can be removed by low doses of Nakom and reappeared upon the drug withdrawal. Tyrosine hydroxylase studies performed in these patients at various stages of the disease showed an unusual pattern of the enzyme activity differing from that seen in children with similar pathology failing to improve dramatically under the Nakom treatment.
