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Pure hypotonia in a four-year-old patient: An atypical presentation of Dopa-responsive dystonia

      Highlights

      • Dopa-responsive Dystonia (DRD) is a levodopa responsive, progressive, lower extremity dystonia with diurnal variation.
      • This condition can manifest with other symptoms including intellectual disability, seizures, autonomic dysfunction, and cerebral palsy.
      • DRD can cause pure hypotonia without dystonia or other extrapyramidal signs.
      • Consider this diagnosis and a trial of Levodopa in pediatric patients with unexplained hypotonia.
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