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AJNR - American Journal of Neuroradiology

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Investigations into the Association between Cervicomedullary Neuroschisis and Mirror Movements in Patients with Klippel-Feil Syndrome

Stuart A. Royal^a, R. Shane Tubbs^b,c, Michael G. D’Antonio^d, Michael J. Rauzzino^e and W. Jerry Oakes^b

^a Department of Pediatric Imaging, The Children’s Hospital of Alabama, University of Alabama at Birmingham, Birmingham, AL
^b Department of Pediatric Neurosurgery, The Children’s Hospital of Alabama, University of Alabama at Birmingham, Birmingham, AL
^c Department of Cell Biology, University of Alabama at Birmingham, Birmingham, AL
^d Department of Radiology, Louisiana State University School of Medicine, New Orleans, LA
^e Peninsula Neurosurgical Associates, Hampton, VA

View larger version (25K): [in a new window]   FIG 1. Illustration of mirror movements. With mirror movements, voluntary (active) movements of one extremity are mimicked by involuntary (passive) movements in the opposite extremity with a central plane of symmetry. Thus, movements of the hand and individual digits also occur in the opposite hand as if a central mirror were reflecting its image to the opposite side.

View larger version (37K): [in a new window]   FIG 2. Illustration of grading system for neuroschisis. A-F, Line drawings of the grading system of neuroschisis. G-L, Axial MR image examples of each grade chosen from patients in this study.

View larger version (28K): [in a new window]   FIG 3. Diagrammatic representation shows normal pathways of descending corticospinal tracts, including crossed lateral corticospinal tract (LCT), uncrossed anterior corticospinal tract (ACT), and anterolateral corticospinal tract (ALCT).

View larger version (41K): [in a new window]   FIG 4. Proposed mechanisms of mirror movements. A, Normal pathways. B, Proposed mechanisms of mirror movements include bilateral signals originating at the pyramidal decussation, either by cross talk or by double branching fibers. C, Deficiency at the decussation, resulting in development of accessory or double branched pathways inferiorly within the spinal cord. D, Deficient contralateral cortical inhibition, resulting in the generation of bilateral cortical signals.