Therapeutic Effects of Dual Dopaminergic Modulation With l-DOPA and Chlorpromazine in Patients With Idiopathic Cervical Dystonia.

Abstract

Imbalanced activities between dopamine D1 and D2 signals in striatal striosome-matrix system have been proposed as a cause of dystonia symptoms. The aim of this study was to assess the therapeutic effects of dual dopaminergic modulation (DDM) with l-DOPA and chlorpromazine (CPZ) in patients with idiopathic cervical dystonia (CD). We enrolled 21 patients with CD who responded poorly to botulinum toxin treatment. The severities of CD motor symptoms and CD-associated pain were determined using the Toronto Western Spasmodic Torticollis Rating Scale and the visual analog scale, respectively. In patients with CD (n = 7), oral administration of l-DOPA combined with CPZ significantly attenuated both CD motor symptoms and CD-associated pain in a dose-related manner. By contrast, there was no improvement of CD symptoms in patients (n = 7) who ingested l-DOPA alone nor in those (n = 7) who ingested CPZ alone. DDM with l-DOPA and CPZ may be an effective tool to treat dystonia symptoms in patients with botulinum toxin-resistant idiopathic CD. Our results may also indicate that CD dystonia symptoms could be attenuated through DDM inducing an increase in striosomal D1-signaling. This study provides Class III evidence that treatment of botulinum toxin-resistant idiopathic cervical dystonia with l-DOPA and chlorpromazine is superior to either one alone.