The Segawa syndrome is an autosomal dominant form of guanosine triphosphate cyclohydrolase deficiency, resulting in decreased dopamine and serotonin levels, typically presenting as a dopa-responsive dystonia.
Case presentation of a 56-year-old man with dopa-responsive dystonia, treated with electroconvulsive therapy for a psychotic depression.
Scores on the Inventory of Depressive Symptomatology dropped from 35 before treatment to 3 after the eighth treatment session. Etomidate and succinylcholine were used as anesthetics. Apart from 2 sessions with postictal agitation, the course of electroconvulsive therapy was finished uneventfully. Electroconvulsive therapy and anesthesia had no untoward effects on motor function.
Electroconvulsive therapy can be administered safely and effectively in a patient with dopa-responsive dystonia (Segawa syndrome).
*ECT Department and Department of Mood Disorders, University Psychiatric Center - Catholic University of Leuven, Campus Kortenberg, Kortenberg, Belgium; †Catholic University of Leuven; and ‡University Psychiatric Center - Catholic University of Leuven, Campus Kortenberg, Kortenberg, Belgium.
Received for publication February 9, 2009; accepted February 17, 2009.
Reprints: Pascal Sienaert, MD, University Psychiatric Center, University Psychiatric Center, Catholic University of Leuven, Campus Kortenberg, Leuvensesteenweg 517, 3070 Kortenberg, Belgium (e-mail: firstname.lastname@example.org).