title: Levodopa-induced dyskinesias in tyrosine hydroxylase deficiency.
abstract: The objective of this study was to characterize levodopa (l-dopa)-induced dyskinesias in patients with tyrosine hydroxylase deficiency. Clinical observation was carried out on 6 patients who were diagnosed with tyrosine hydroxylase deficiency and were treated with escalating doses of l-dopa. All 6 patients showed l-dopa-induced dyskinesias of variable intensity early in the course of treatment and regardless of the age of initiation. l-Dopa-induced dyskinesias were precipitated by increases in the dose of l-dopa and also by febrile illnesses and stress. They caused dysfunction and distress in 2 patients. The dyskinesias were improved by decreasing the l-dopa dose or by slowing its titration upward. Increasing the dose frequency was helpful in 2 patients, and introducing amantadine was helpful in another 2 patients. l-Dopa-induced dyskinesias are a common phenomenon in tyrosine hydroxylase deficiency. The current observations show that l-dopa-induced dyskinesias are frequent in a dopamine-deficient state in the absence of nigrostriatal degeneration. Although l-dopa-induced dyskinesias in tyrosine hydroxylase deficiency are phenomenologically similar to those that occur in Parkinson's disease, they are different in a number of other respects, suggesting intrinsic differences in the pathophysiologic basis of l-dopa-induced dyskinesias in the 2 conditions. © 2013 Movement Disorder Society.
authors: First Department of Pediatrics, Aghia Sophia Children's Hospital, University of Athens, Athens, Greece. rosepons@otenet.gr
authors: Roser
authors: R
authors: Pons
authors: Dimitris
authors: D
authors: Syrengelas
authors: Sotiris
authors: S
authors: Youroukos
authors: Irene
authors: I
authors: Orfanou
authors: Arqirios
authors: A
authors: Dinopoulos
authors: Bru
authors: B
authors: Cormand
authors: Aida
authors: A
authors: Ormazabal
authors: Angels
authors: A
authors: Garzía-Cazorla
authors: Mercedes
authors: M
authors: Serrano
authors: Rafael
authors: R
authors: Artuch
mesh_terms: D002648:Child; D002675:Child, Preschool; D015259:Dopamine Agents / Q000009:adverse effects*; D004409:Dyskinesia, Drug-Induced / Q000209:etiology*; D020821:Dystonic Disorders / Q000151:congenital* / Q000188:drug therapy; D006801:Humans; D007223:Infant; D007980:Levodopa / Q000009:adverse effects*
chemical_list: D015259:Dopamine Agents; D007980:Levodopa
keywords: chorea; dyskinesias; levodopa; tyrosine hydroxylase deficiency