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Cervical cord compression from plexiform neurofibromas in neurofibromatosis-1
  1. Jeffrey R Leonard (leonardj{at}nsurg.wustl.edu)
  1. Washington University, United States
    1. Rosalie E Ferner (rosalie.ferner{at}kcl.ac.uk)
    1. Guy's Hospital, United Kingdom
      1. Nicholas Thomas (nick.thomas{at}kch.nhs.uk)
      1. King's College London, United Kingdom
        1. David H Gutmann (gutmannd{at}neuro.wustl.edu)
        1. Washington University, United States

          Abstract

          Cervical cord compression from cervical root neurofibromas represents an important clinical problem in patients with NF1, but is rarely reported. The aim of this study was to describe the clinical presentation and follow-up of children and adults with NF1 and cervical cord compression. A retrospective review of clinical records and neuroimaging studies from two large tertiary care centers between 1996 and 2006 was performed. Thirteen patients with NF1 and cervical cord compression were identified. The age at presentation ranged from nine to sixty-one years. The most common presentation was progressive quadriparesis. Eleven of thirteen patients underwent cervical decompression and subtotal resection of the associated neurofibroma. The majority of the patients had recovery of neurologic function and no further clinical progression. Progressive neurological deficit (typically quadriparesis), rather than neuroimaging appearances, should dictate the need for surgery.

          • cervical spine
          • neurofibromatosis
          • plexiform neurofibroma
          • spinal cord tumour

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