The benefits of Deep Brain Stimulation (DBS) of the Globus Pallidus internus (GPi) for isolated idiopathic or genetic (formerly termed primary) dystonia are now well recognised in both adults and children, with improvements of 52-88% being seen in the Burke-Fahn-Marsden Dystonia Rating Scale motor score (BFMDRS-m)(Coubes et al., 2004; Vidailhet et al., 2005; Zorzi et al., 2005, Alterman et al., 2007). However, isolated dystonia, in which the dystonic movements are the only feature of the neurological disease and structural neuroimaging is normal, is rare, especially in childhood (Roubertie et al., 2002).
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