1. 1989 on the degree of disability at the time of diagnosis and does not seem to wear off with time. A recent study showed that five patients with dopa responsive dystonia had cerebrospinal fluid concentrations of homovanillic acid, 5-hydroxyindoleacetic acid., and biopterin that were below the normal range.6 The deficiency of biopterin in patients with diurnally fluctuating dystonia was significantly greater than the deficiency in patients with diurnally stable dystonia. The association between dopa responsive dystonia and a deficiency of biopterin can be explained by the fact that reduced biopterin is an essential cofactor for tyrosine hydroxylase, the enzyme that is rate limiting in the synthesis of catecholamines and serotonin;A.P.R.I.L.
2. Dopa-responsive dystonia;Nygaard, T.G.; Marsden, C.D.; Duvoisin, R.C.;Adv Neurol,1988
3. Hereditary progressive dystonia with marked diurnal fluctuation;Segawa, M.; Hosaka, A.; Miyagawa, F.; Nomura, Y.; Imai, H.;Adv Neurol,1976
4. Hereditary dystonia-parkinsonism syndrome of juvenile onset;Nygaard, T.G.; Duvoisin, R.C.;Neurology,1986
5. Progressive dystonia with marked diurnal fluctuation;Ouvrier, R.A.;Ann Neurol,1978