Cases of dopa-responsive dystonia (Segawa disease) in Estonia.

Authors :: Talvik I
Segawa M
Veri K
Gross-Paju K
Talvik T
Source :: Brain & development [Brain Dev] 2010 May; Vol. 32 (5), pp. 428-31. Date of Electronic Publication: 2009 May 07.
Publication Year :: 2010
Abstract: The aim of this report is to present the first four cases from three families of dopa-responsive dystonia diagnosed in Estonia. Diagnosis was performed by clinical evaluation and response to levodopa and was confirmed by gene analyses. The prevalence of dopa-responsive dystonia in Estonia was 1.4 per 100,000 (95%CI=0.39-3.65) children less than 18 years of age. In all children with dystonia it is important to think about possible dopa-responsive dystonia as this is treatable condition and improving the quality of life of children.<br /> (Copyright 2009 Elsevier B.V. All rights reserved.)