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NEUROLOGY 1986;36:760
© 1986 American Academy of Neurology

Tetrahydrobiopterin in dystonia

Identification of abnormal metabolism and therapeutic trials

Peter A. LeWitt, MD, Leonard P. Miller, PhD, Robert A. Levine, PhD, Walter Lovenberg, PhD, Richard P. Newman, MD, Antigone Papavasiliou, MD, Ayman Rayes, MD, Roswell Eldridge, MD and R. Stanley Burns, MD

Department of Neurology (Drs. LeWitt, Papavasiliou, and Rayes), Lafayette Clinic, Detroit, MI; the Experimental Therapeutics Branch (Drs. LeWitt, Newman, and Burns) and the Intramural Research Program (Dr. Eldridge), NINCDS; and the Hypertension-Endocrine Branch (Drs. Miller, Levine, and Lovenberg), NHLBI, National Institutes of Health, Bethesda, MD.

The pteridine cofactor of tyrosine and tryptophan hydroxylases, tetrahydrobiopterin (BH4), is concentrated in the striatum and other sites of brain monoamine synthesis and is a regulatory factor in the rate-limiting step of catecholamine synthesis. CSF content was decreased in eight patients with dystonic disorders (mean, 13.0 ± 0.8 pmol/ml CSF compared with 20.6 ± 1.4 in age-matched normals). We gave several trials of synthetic BH4 intravenously to 10 dystonic patients with benefit for 2 subjects with diurnally fluctuating dystonia, 1 with hemidystonia and parkinsonism, and 1 with generalized torsion dystonia. The findings of biopterin abnormality and the observed clinical improvements may point to a role for the cofactor in the pathogenesis and, possibly, the treatment of some forms of primary dystonia.

Address correspondence and reprint requests to Dr. LeWitt, Department of Neurology, Lafayette Clinic, 951 East Lafayette, Detroit, MI 48207.

Accepted for publication October 14, 1985.




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