When should dopa-responsive dystonia be considered in the evaluation of Parkinson disease (PD)?

Updated: Jan 24, 2019
  • Author: Robert A Hauser, MD, MBA; Chief Editor: Selim R Benbadis, MD  more...
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Answer

Dopa-responsive dystonia should be considered in patients with juvenile-onset dystonia and parkinsonism, particularly with diurnal fluctuations in symptoms. In such patients, a trial of levodopa is critical. Additional tests for this condition include measurement of CSF concentrations of biopterin, neopterin, and the neurotransmitter metabolites homovanillic acid (HVA), 5-hydroxyindoleacetic acid (5-HIAA), and 3-methoxy-4-hydroxyphenylglycol (MHPG). In both forms of dopa-responsive dystonia, an altered pattern of decreases in these compounds is observed.


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