Segawa's Syndrome (Dopa-Responsive Dystonia) is a hereditary dystonia that responds well to low doses of levodopa.
Clinical features that may pose problems during anesthesia:
(1) dysphagia
(2) hypersalivation
(3) cervical dystonia (torticollis)
In general patient's tolerate general anesthesia and nondepolarizing neuomuscular blocking agents well.
The adverse side effects of dopa (hypotension, cardiac irritability) are usually not observed but should be watched for. These adverse effects are dose-dependent, and the doses of dopa in Segawa syndrome are low. Levodopa therapy is usually maintained in the perioperative period.
One feature of Segawa's syndrome is a marked diurnal variation, with worsening during the day and improvement after sleep. This suggests surgery in the early morning may be optimum.
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