Article Text

PDF
Cervical cord compression from plexiform neurofibromas in neurofibromatosis 1
  1. J R Leonard1,
  2. R E Ferner2,
  3. N Thomas3,
  4. D H Gutmann1,4
  1. 1
    Department of Neurosurgery, St Louis Children’s Hospital and Washington University School of Medicine, St Louis, Missouri, USA
  2. 2
    Department of Neurology, Guy’s and St Thomas’ Hospitals, London, UK
  3. 3
    Department of Neurosurgery, King’s College, London, UK
  4. 4
    Department of Neurology, St Louis Children’s Hospital and Washington University School of Medicine, St Louis, Missouri, USA
  1. Dr D H Gutmann, Department of Neurology, Washington University School of Medicine, 660 South Euclid Avenue, Box 8111, St Louis MO; gutmannd{at}wustl.edu

Abstract

Cervical cord compression from cervical root neurofibromas represents an important clinical problem in patients with neurofibromatosis type 1 (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 centres between 1996 and 2006 was performed. 13 patients with NF1 and cervical cord compression were identified. Age at presentation ranged from 9 to 61 years. The most common presentation was progressive quadriparesis. 11 of 13 patients underwent cervical decompression and subtotal resection of the associated neurofibroma. The majority of patients had recovery of neurological function and no further clinical progression. Progressive neurological deficit (typically quadriparesis), rather than neuroimaging appearances, should dictate the need for surgery.

Statistics from Altmetric.com

Footnotes

  • Competing interests: None.

  • Abbreviations:
    MPNST
    malignant peripheral nerve sheath tumours
    NF1
    neurofibromatosis type 1

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.