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. 1989 Oct;39(10):1393-5.
doi: 10.1212/wnl.39.10.1393.

Tetrahydrobiopterin administration in biopterin-deficient progressive dystonia with diurnal variation

J K Fink  1 , P Ravin, C E Argoff, R A Levine, R O Brady, M Hallett, N W Barton
Affiliations

Tetrahydrobiopterin administration in biopterin-deficient progressive dystonia with diurnal variation

J K Fink et al. Neurology. 1989 Oct.

Abstract

We administered tetrahydrobiopterin (BH4) to 4 patients with progressive dystonia with diurnal variation (PDDV). One patient improved clinically. Deficient CSF concentrations of HVA and 5-HIAA were unchanged despite marked elevation of CSF biopterin concentration. Variable effectiveness of BH4 in PDDV may reflect reduced number or function of biopterin-metabolizing neurons or variable entry of BH4 into these neurons.

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