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Selegiline (l-deprenyl)

Disorders of BH4 synthesis

0.1-0.25mg/kg/day in 3-4 divided doses.  Dosage can be monitored by measuring CSF levels of neurotransmitter metabolites or verifying normalization of serum prolactin levels (163, 164).

5 mg capsule and tablet

PO

GI upset,  backache, headache, insomnia, rhinitis, dyskinesi.

4

163. Olsen RK, Olpin SE, Andresen BS, et al. ETFDH mutations as a major cause of riboflavin-responsive multiple acyl-CoA dehydrogenation deficiency. Brain 2007;130(Pt 8):2045-54 doi: 10.1093/brain/awm135[published Online First: Epub Date]|.

164. van Maldegem BT, Duran M, Wanders RJ, Waterham HR, Wijburg FA. Flavin adenine dinucleotide status and the effects of high-dose riboflavin treatment in short-chain acyl-CoA dehydrogenase deficiency. Pediatric research 2010;67(3):304-8 doi: 10.1203/PDR.0b013e3181cbd57b[published Online First: Epub Date]|.

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