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Extensive Mirror-Image Neurofibromas of Entire Spine Resulting in Spastic Tetraplegia.

Authors :
Bigder M
Szelemej P
Berrington N
Source :
World neurosurgery [World Neurosurg] 2017 Dec; Vol. 108, pp. 985.e7-985.e8. Date of Electronic Publication: 2017 Sep 01.
Publication Year :
2017

Abstract

Neurofibromatosis 1 (NF1) is associated with increased incidence of spinal tumors including neurofibromas. The majority of NF1-associated spine neurofibromas are asymptomatic; however, a minority of patients will experience neurologic symptoms that can range from mild paresthesia, radiculopathy, myelopathy, and focal weakness to quadriplegia in extreme cases. We present a 21-year-old male diagnosed with NF1 in infancy and followed for multiple mirror-image neurofibromas involving the entire spine. He was asymptomatic until age 14 when he developed neck pain and progressive tetraplegia with magnetic resonance imaging showing severe cord compression secondary to bilateral C2 neurofibromas. Emergent cervical decompression was performed at C1-C3 along with debulking of bilateral neurofibromas. Postoperatively he regained full strength with no signs of myelopathy several years postoperatively. This case demonstrates a dramatic neuroimaging finding and emphasizes the potential for significant neurologic deterioration in previously asymptomatic NF1 patients, highlighting the need for long-term follow-up.<br /> (Copyright © 2017 Elsevier Inc. All rights reserved.)

Details

Language :
English
ISSN :
1878-8769
Volume :
108
Database :
MEDLINE
Journal :
World neurosurgery
Publication Type :
Academic Journal
Accession number :
28867317
Full Text :
https://doi.org/10.1016/j.wneu.2017.08.139