ABSTRACT
The last decade has seen an increase in the treatment options for many forms of dystonia, primarily due to the dramatic expansion in the use of botulinum toxin (BTX) and the more recent application of deep brain stimulation to both childhood and adult dystonia. During this period, despite the discovery of the DYT1 gene, there have been few new developments in the pharmacologic treatment of dystonia. Nonetheless, there still is an important role for drug therapy of both idiopathic and some symptomatic forms of dystonia:
● Patients with dopa-responsive dystonia (DRD) have dramatic, sustained improvement taking small doses of levodopa.1 Unless an alternative diagnosis is certain (e.g. a gene for an alternative form of dystonia has been found in the family), a trial of levodopa as initial therapy should be strongly considered. This applies primarily to childhood-onset dystonia, but patients with adult-onset dystonia and a history of a relative with childhood-onset dystonia might also be candidates for a trial of levodopa.
