Dopa-responsive dystonia: Clinical and family study in Taiwanese

Rou Shayn Chen, Chin Chang Huang*, Chin Song Lu

*Corresponding author for this work

Research output: Contribution to journalJournal Article peer-review

5 Scopus citations


We report the clinical features of four female patients with dopa-responsive dystonia (DRD), and the survey of the family members. The patients were 2 sisters and 2 sporadic patients from 3 families. Their age of onset ranged between 5 and 13 years. The clinical manifestation was characterized by limb dystonia which was relieved by L-dopa treatment. Diurnal fluctuation disappeared 15 years later in one patient. There was a wide spectrum of Parkinsonian features and variability of dystonia. Response of L-dopa was still excellent 20 years later. In survey of the family members, there was neither bradykinesia, rigidity, tremor nor dystonia.

Original languageEnglish
Pages (from-to)43-46
Number of pages4
JournalClinical Neurology and Neurosurgery
Issue number1
StatePublished - 02 1996
Externally publishedYes


  • Diurnal fluctuation
  • Dopa-responsive dystonia
  • Family survey
  • L-dopa
  • Parkinsonism
  • Segawa disease


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