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Dysmorphic neuromuscular junctions associated with motor ability in cerebral palsy

Authors

  • Mary C. Theroux MD,

    1. Nemours Biomedical Research, A. I. duPont Hospital for Children, 1600 Rockland Road, Wilmington, Delaware 19803, USA
    2. Department of Anesthesiology and Critical Care, A. I. duPont Hospital for Children, Wilmington, Delaware, USA
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  • Karyn G. Oberman BA,

    1. Nemours Biomedical Research, A. I. duPont Hospital for Children, 1600 Rockland Road, Wilmington, Delaware 19803, USA
    2. Department of Biological Sciences, University of Delaware, Newark, Delaware, USA
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  • Justine Lahaye MS,

    1. Nemours Biomedical Research, A. I. duPont Hospital for Children, 1600 Rockland Road, Wilmington, Delaware 19803, USA
    2. Physiology and Informatics Program, University of Poitiers, Poitiers, France
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  • Bobbie A. Boyce,

    1. Nemours Biomedical Research, A. I. duPont Hospital for Children, 1600 Rockland Road, Wilmington, Delaware 19803, USA
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  • David DuHadaway BS,

    1. Nemours Biomedical Research, A. I. duPont Hospital for Children, 1600 Rockland Road, Wilmington, Delaware 19803, USA
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  • Freeman Miller MD,

    1. Department of Orthopaedics, A. I. duPont Hospital for Children, Wilmington, Delaware, USA
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  • Robert E. Akins PhD

    Corresponding author
    1. Nemours Biomedical Research, A. I. duPont Hospital for Children, 1600 Rockland Road, Wilmington, Delaware 19803, USA
    • Nemours Biomedical Research, A. I. duPont Hospital for Children, 1600 Rockland Road, Wilmington, Delaware 19803, USA
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Abstract

Cerebral palsy (CP) is the most prevalent neurologic disease in children and a leading cause of severe physical disability. Research and clinical experience indicate that children with CP have abnormal neuromuscular junctions (NMJs), and we present evidence that nonapposition of neuromuscular junction components is associated with the severity of motor system deficit in CP. Leg muscle biopsies collected from ambulatory (n = 21) or nonambulatory (n = 38) CP patients were stained in order to detect acetylcholine receptor (AChR) and acetylcholine esterase (AChE). Image analysis was used to calculate the extra-AChE spread (EAS) of AChR staining to estimate the amount of AChR occurring outside the functional, AChE-delimited NMJ. Nonambulatory children exhibited higher average EAS (P = 0.025) and had a greater proportion of their NMJs with significantly elevated EAS (P = 0.023) than ambulatory children. These results indicate that physical disability in children with CP is associated with structurally dysmorphic NMJs, which has important implications for the management of CP patients, especially during surgery and anesthesia. Muscle Nerve, 2005

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