Multiple Applications of L-DOPA Therapy in Children with Neurological Disorders - 25 Year Review (P04.178)

Abstract

Objective: L -Dopa is well known for its use in childhood neurological disorders. formost is Dopa-responsive dystonia, Segawa9s Disease, and its variants. L-Dopa has increasingly been cited in many other childhood neurological diosrders in the literature. This review summarizes athe applications of L-Dopa in childhood based upon extensive computerized literature review expanding the clinical applications in and young adults. Background Previously isloated reports have cited the application of L Dopa in childhood movement disorders but no single review has comprehensively reviewed all the disorders in which L Dopa has been used. Design/Methods: Summarization of medical literature in the past 25 years searching the term L-Dopa in childhood, L -Dopa in Childhood movement disorders, L Dopa in children utilizing Pubmed database and a Cochrane Review was performed.Citations were grouped upon frequency of citation and applicability to clincial practice. Both well known applications and less commonly cited therapies were included. Results: Over 25 years at least 26 different disorders have been reported in childhood utilizing L-Dopa. Most frequent are the disorders related to Dopa responsive dystonia, (Segawa9s disease)and its variants. This includes disorders in dopamine synthesis including tyrosine hydroxylase deficiency and tetrahydrobiopterin pathway disorders. Significant citations also include: Juvenile Parkinson9s disease, Myoclonus- Dystonia, Familial paroxysmal Dystonia, PKAN, Lower Limb Dystonia, Chediak -Hegashi synsdrome, Dystonia-Parkinsonism, Benign Paroxysmal Tonic Upgaze of Childhood, Amblyopia, Aromatic l-aminoacid decarboxylase Deficiency, Restless Legs Syndrome and Attention Deficit Hyperactivity Syndrome, and potential application in Angelman Syndrome. Conclusions: In the past 25 years, over 26 childhood neurological disorders have been described that are responsive to L-Dopa therapy. While many are well known an expanded current application is summarized. This summary brings up to date the prevalent citations and provides a review of the many less commonly and less frequently recognized disorders in childhood for which L-Dopa therapy plays a major role. Disclosure: Dr. Lavenstein has nothing to disclose. Dr. Hecht has nothing to disclose.