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AJNR Awards, New Junior Editors, and more. Read the latest AJNR updates

Research ArticlePediatric Neuroimaging

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

Stuart A. Royal, R. Shane Tubbs, Michael G. D’Antonio, Michael J. Rauzzino and W. Jerry Oakes
American Journal of Neuroradiology April 2002, 23 (4) 724-729;
Stuart A. Royal
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R. Shane Tubbs
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Michael G. D’Antonio
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Michael J. Rauzzino
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W. Jerry Oakes
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  • Fig 1.
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    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.

  • Fig 2.
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    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.

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    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).

  • Fig 4.
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    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.

Tables

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    TABLE 1:

    Grading system for cervical cord neuroschisis

    Grade012345
    Axial imaging appearanceNormal cordDeformity without cleftPosterior cleft aloneAnterior cleft with or without posterior cleftBow tie configurationComplete neuroschisis
    • View popup
    TABLE 2:

    Correlation of mirror image movements with cervical cord clefting

    Grade012345
    Axial imaging appearanceNormal cordDeformity without cleftPosterior cleft aloneAnterior cleft with or without posterior cleftBow tie configurationComplete neuroschisisCord obscuredTotal
    MM (+)0012111*6
    MM (−)33000006
    N/E12001004
    Total451221116
    • Note.—MM indicates mirror movements; N/E, not clinically evaluated.

    • * Chiari II malformation with posterior cord obscured by tonsillar peg.

    • View popup
    TABLE 3:

    Correlation of conventional films with mirror movements

    Extent of Vertebral FusionSingle Level of Fusion>1 Level of FusionExtensive FusionTotal
    MM (+)0246
    MM (−)2226
    Total24612
    • Note.—MM indicates mirror movements.

    • View popup
    TABLE 4:

    Correlation of conventional films with neuroschisis

    Extent of Vertebral FusionSingle Level of Fusion>1 Level of FusionExtensive FusionTotal
    NS (+)0156
    NS (−)26210
    Total27716
    • Note.—NS indicates neuroschisis.

    • View popup
    TABLE 5:

    Entities associated with mirror movements

    Agenesis of the corpus callosum
    Basilar invagination of the skull
    Spina bifida occulta
    Friedrich’s ataxia
    Kallmann’s syndrome
    Usher’s syndrome
    Phenylketonuria
    Congenital hemiparesis
    Diabetes insipidus
    Mental retardation
    Schizophrenia
    Extrapyramidal system disease
    CNS insult (tumor, CVA, SAH, trauma)
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American Journal of Neuroradiology: 23 (4)
American Journal of Neuroradiology
Vol. 23, Issue 4
1 Apr 2002
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Investigations into the Association between Cervicomedullary Neuroschisis and Mirror Movements in Patients with Klippel-Feil Syndrome
Stuart A. Royal, R. Shane Tubbs, Michael G. D’Antonio, Michael J. Rauzzino, W. Jerry Oakes
American Journal of Neuroradiology Apr 2002, 23 (4) 724-729;
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Cite this article
Stuart A. Royal, R. Shane Tubbs, Michael G. D’Antonio, Michael J. Rauzzino, W. Jerry Oakes
Investigations into the Association between Cervicomedullary Neuroschisis and Mirror Movements in Patients with Klippel-Feil Syndrome
American Journal of Neuroradiology Apr 2002, 23 (4) 724-729;

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