Child Neurology: A young child with an undiagnosed case of dystonia responsive to l-dopa.

Journal Article (Journal Article)

Childhood-onset dystonias are a heterogeneously diverse group. There exists a specific set of dystonias that respond profoundly well to low doses of l-dopa (dopa-responsive dystonia [DRD]). Classical DRD is caused by deficiency of GTP cyclohydrolase 1 or tyrosine hydroxylase, but other conditions can cause dystonias that are partially responsive to dopamine. The idea of a diagnostic therapeutic trial with l-dopa for children who present with dystonia has been around for decades and is frequently advocated for; however, l-dopa trials are not without risk.

Full Text

Duke Authors

Cited Authors

  • Trau, SP; Gallentine, WB; Mikati, MA

Published Date

  • February 18, 2020

Published In

Volume / Issue

  • 94 / 7

Start / End Page

  • 326 - 328

PubMed ID

  • 32015175

Electronic International Standard Serial Number (EISSN)

  • 1526-632X

Digital Object Identifier (DOI)

  • 10.1212/WNL.0000000000008963


  • eng

Conference Location

  • United States