Aims: To study stereotactic magnetic resonance imaging (MRI) features of the basal ganglia in DYT1 primary dystonia. Methods: Twenty-five genetically confirmed DYT1 dystonia patients (age range, 8–66 years; mean age, 22 years) underwent brain MRI under general anesthesia at the time of globus pallidus internus (GPi) deep brain stimulation (DBS) surgery. MR images were retrospectively reviewed for signal intensity alterations. Clinical improvement of patients was assessed by comparing pre- and postoperative Burke-Fahn-Marsden Dystonia Rating Scale scores. Results: Seventeen patients out of 25 (68%) exhibited T1-weighted hypointense/T2-weighted hyperintense signal abnormalities in the putamen and globus pallidus on MR images. Signals were isointense with cerebrospinal fluid in all sequences. The mean volume of focal signal abnormalities was 15 mm3 (maximum, 154.5 mm3). The total volume of focal signal abnormalities in the basal ganglia was correlated with the duration of the disease (p = 0.01). Although clinical outcome did not differ as a function of the presence of focal signal abnormalities overall, patients with signals within the GPi tended to show lesser improvement (p = 0.04). Conclusions: T1-hypointense/T2-hyperintense signal abnormalities are common findings in the putamen and globus pallidus of DYT1 patients but do not contraindicate DBS. However, their presence within the GPi may reduce the efficacy of DBS treatment.

Keywords:
Basal ganglia, Deep brain stimulation, Dyskinesia, Dystonia, Electrical stimulation, Magnetic resonance imaging, Movement disorders
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© 2008 S. Karger AG, Basel
2008
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