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Based on evaluation of the literature, evidence exists for (maintenance) drug therapy, including dosage and side effects, for Phe-reduced diet, sapropterin dihydrochloride, L-Dopa with peripheral DC inhibitor (carbidopa or benserazide), 5-HTP, folinic acid, dopamine agonists (DA), selective monoamine oxidase (MAO) inhibitors (MAO-I), anticholinergic agents, catechol-O-methyl transferase (COMT) inhibitors, selective serotonin reuptake inhibitors (SSRIs), benzodiazepines, melatonin, and botulinum toxin injections