Effect of supplemental ascorbic acid in a case of torsion dystonia.
Determinations of various catecholamines and their metabolites have been performed on 24-hr urine collecions obtained from a patient with torsion dystonia and compared to values obtained in a control population. This study was initiated following significant symptomatic worsening by the patient with supplemental ascorbic acid at a dosage of 2 g/day. Compared to base-line values, there resulted no significant alteration in urinary excretion of DOPA, dopamine, norepinephrine, epinephrine, or VMA for either the patient or a group of controls, receiving 1 g/day vitamin C. MHPG is the glycol metabolite of norepinephrine, and is produced both in central and systemic tissues, whereas VMA is not synthesized in brain. The MHPG excretion for the patient increased 150% with supplemental ascorbate, whereas the control individuals demonstrated a mean increase of 19.6%. It is possible that the symptomatic worsening by the patient and the increased excretion of MHPG in response to supplemental ascorbic acid are causally related. Ascorbic acid affects catecholamine biosynthesis at two metabolic loci; it is the necessary cofactor for dopamine-beta-hydroxylase and, by maintaining biopterin in reduced form, facilitates tyrosine hydroxylase holoenzyme activity. Thus, the vitamin may have effected increased central synthesis or turnover of norepinephrine, or both, with resultant clinical worsening.
D000328:Adult; D001205:Ascorbic Acid / Q000008:administration & dosage / Q000627:therapeutic use*; D004295:Dihydroxyphenylalanine / Q000652:urine; D004298:Dopamine / Q000652:urine; D004422:Dystonia Musculorum Deformans / Q000188:drug therapy* / Q000652:urine; D004837:Epinephrine / Q000652:urine; D005260:Female; D006801:Humans; D008734:Methoxyhydroxyphenylglycol / Q000652:urine; D009638:Norepinephrine / Q000652:urine; D014642:Vanilmandelic Acid / Q000652:urine
D008734:Methoxyhydroxyphenylglycol; D014642:Vanilmandelic Acid; D004295:Dihydroxyphenylalanine; D001205:Ascorbic Acid; D004298:Dopamine; D009638:Norepinephrine; D004837:Epinephrine