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64th Annual Meeting of the German Society of Neurosurgery (DGNC)

German Society of Neurosurgery (DGNC)

26 - 29 May 2013, Düsseldorf

Risk of transverse section syndrome by intraspinal neurofibromas

Meeting Abstract

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  • Maria Hummel - Neurochirurgische Klinik und Poliklinik, Universitätsklinikum Würzburg
  • Ralf-Ingo Ernestus - Neurochirurgische Klinik und Poliklinik, Universitätsklinikum Würzburg
  • Cordula Matthies - Neurochirurgische Klinik und Poliklinik, Universitätsklinikum Würzburg

Deutsche Gesellschaft für Neurochirurgie. 64. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC). Düsseldorf, 26.-29.05.2013. Düsseldorf: German Medical Science GMS Publishing House; 2013. DocMO.20.01

doi: 10.3205/13dgnc173, urn:nbn:de:0183-13dgnc1730

Published: May 21, 2013

© 2013 Hummel et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.



Objective: Different to schwannomas, neurofibromas of the cervical roots cannot be resected completely without a high risk of new sensory and motor deficits. In Neurofibromatosis Type 1 (NF1), multiple neurofibromas are a rule and may cause considerable pain and increasing radicular deficits. In case of predominant intraspinal growth, progressive compression of the spinal cord bears the danger of tetraparesis or even transverse section syndrome. The clinical presentation in a cohort of NF1 patients and the surgical options were the focus of this retro-spective study.

Method: From 2005 to 2012 70 patients (36 female and 34 male) with NF1 were investigated and treated in a regular Neurofibromatosis Out-patient Clinic. A predominant spinal manifestation was identified in 23 patients (33%). Clinical signs, causes for surgery, mode of surgery, and clinical outcome were analyzed.

Results: In spinal manifestation most frequent symptoms and signs were paresthesia, hypoesthesia and pain. In 13 patients (57%) surgery was indicated, in 9 of those because of pain and gait deficits, in 4 due to progressive tetraparesis and impending transverse section syndrome. Surgery was carried out as a decompressive procedure by multi-level laminotomies and intra-capsular tumor resection with functional preservation of the nerve roots running in the intra-extra-spinal root capsule. All the patients showed subjective and functional improvement. Especially in those 4 patients with extensive intraspinal tumors and spinal cord compression a stop of their on-going deterioration and a functional recovery by at least one motor power grade continuing over several months could be documented. In two patients a re-growth of the residual tumor caused a new clinical deterioration. In one patient a second intervention was followed by clinical improvement again. The mean follow-up was three years.

Conclusions: Progressive tetraparesis with impending transverse section syndrome is a devastating situation in advanced NF1. Despite ongoing tumor progression along the extra-spinal part of the nerve roots, subtotal tumor resection within the spinal canal can provide substantial functional improvement. Obviously, the decompression of the spinal cord is the decisive element for recovery.