Neuropediatrics 2006; 37(2): 79-82
DOI: 10.1055/s-2006-924109
Original Article

Georg Thieme Verlag KG Stuttgart · New York

Functional MRI Reveals Activation of a Subcortical Network in a 5-Year-Old Girl with Genetically Confirmed Myoclonus-Dystonia

M. F. Nitschke1 , C. Erdmann1 , 2 , P. Trillenberg1 , A. Sprenger1 , N. Kock1 , 3 , J. Sperner4 , C. Klein1 , 3
  • 1Department of Neurology, University Hospital of Schleswig-Holstein, Campus Lübeck, Lübeck, Germany
  • 2Department of Neuroradiology, University Hospital of Schleswig-Holstein, Campus Lübeck, Lübeck, Germany
  • 3Department of Human Genetics, University Hospital of Schleswig-Holstein, Campus Lübeck, Lübeck, Germany
  • 4Department of Pediatrics, University Hospital of Schleswig-Holstein, Campus Lübeck, Lübeck, Germany
Further Information

Publication History

Received: February 6, 2006

Accepted after Revision: February 13, 2006

Publication Date:
14 June 2006 (online)

Abstract

We investigated a five-year-old girl suffering from genetically confirmed, action-induced myoclonus-dystonia (M‐D) with functional magnetic resonance imaging (MRI). We compared the activation pattern by movements of her right hand as if drawing a picture, which elicited M‐D, with simple snapping movements (without overt M‐D). The drawing and snapping conditions resulted in activation of a motor network including the motor cortex, the putamen, and the cerebellar hemispheres. The direct comparison of the drawing condition with snapping as control revealed specific activations within the thalamus and the dentate nucleus. An age matched healthy control did not show significant activation within the thalamus or dentate nucleus.

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PD Dr. Matthias F. Nitschke

Department of Neurology
University Hospital of Schleswig-Holstein
Campus Lübeck

Ratzeburger Allee 160

23538 Lübeck

Germany

Email: matthias.nitschke@neuro.uni-luebeck.de

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