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A Functional Magnetic Resonance Imaging Study

Richard J. Beukers, MD; Elisabeth M. J. Foncke, MD, PhD; Johan N. van der Meer, MSc; Aart J. Nederveen, PhD; Michiel B. de Ruiter, PhD; Lo J. Bour, PhD; Dick J. Veltman, MD, PhD; Marina A. J. Tijssen, MD, PhD

Arch Neurol. 2010;67(4):469-474.

Background  Myoclonus-dystonia is an autosomal dominantly inherited movement disorder clinically characterized by myoclonic jerks and dystonic postures or movements of the upper body. Functional imaging studies in other, mainly heterogeneous groups of dystonia do agree on dysfunction of the striato-pallido-thalamo-cortical circuit.

Objective  To study cerebral activation patterns with functional magnetic resonance imaging in a genetically defined homogeneous group of patients with dystonia.

Design, Setting, and Patients  Thirteen clinically affected SGCE mutation carriers and 11 control subjects were studied in a case-control study.

Intervention  A finger-tapping motor task was performed in a block design using 3.0-T magnetic resonance imaging.

Main Outcome Measures  Blood oxygenation level–dependent signals were compared between groups.

Results  In SGCE mutation carriers, we observed significant hyperresponsiveness in contralateral inferior parietal cortical areas, ipsilateral premotor and primary somatosensory cortex, and ipsilateral cerebellum during the motor task compared with healthy control subjects.

Conclusions  The cortical activation patterns in SGCE mutation carriers during this motor task point to a disorganized sensorimotor integration in this uniform group of patients with dystonia and are consistent with functional neuroimaging studies in other types of (hereditary) dystonia.

Author Affiliations: Departments of Neurology (Drs Beukers, Foncke, and Tijssen), Clinical Neurophysiology (Mr van der Meer and Dr Bour), Radiology (Dr Nederveen), and Psychiatry (Drs de Ruiter and Veltman), Academic Medical Centre, University of Amsterdam, Amsterdam, the Netherlands.

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