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Tetrahydrobiopterin administration in biopterin-deflcient progressive dystonia with diurnal variation

Developmental and Metabolic Neurology Branch, National Institute of Neurological Disorders and Stroke, Bethesda, MD (Drs. Fink, Argoff, Brady, and Barton)
Office of the Clinical Director, National Institute of Neurological Disorders and Stroke, Bethesda, MD (Dr. Hallett)
Department of Neurology, University of Massachusetts Medical Center, Worcester, MA (Dr. Ravin)
Department of Psychiatry, Wayne State University, Detroit, MI. (Dr. Levine)

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.

Address correspondence and reprint requests to Dr. Fink, Building 10, Room 3D03, NINDS, NIH, Bethesda, MD 20892.

Received February 6, 1989. Accepted for publication in final form April 26, 1989.

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