Accepted Manuscript Extensive mirror-image neurofibromas of the entire spine resulting in spastic tetraplegia Mark Bigder, Paul Szelemej, Neil Berrington PII:
S1878-8750(17)31440-7
DOI:
10.1016/j.wneu.2017.08.139
Reference:
WNEU 6389
To appear in:
World Neurosurgery
Received Date: 5 April 2017 Revised Date:
18 August 2017
Accepted Date: 22 August 2017
Please cite this article as: Bigder M, Szelemej P, Berrington N, Extensive mirror-image neurofibromas of the entire spine resulting in spastic tetraplegia, World Neurosurgery (2017), doi: 10.1016/ j.wneu.2017.08.139. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
Extensive mirror-image neurofibromas of the entire spine resulting in spastic tetraplegia Mark Bigder, Paul Szelemej, Neil Berrington
Neil Berrington MMed FRCS FCS(SA) Section of Neurosurgery University of Manitoba [email protected]
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Paul Szelemej MD Section of Neurosurgery University of Manitoba [email protected]
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Corresponding author: Mark Bigder MD Section of Neurosurgery Department of Surgery University of Manitoba GB129-820 Sherbrook Street Health Sciences Centre Winnipeg, MB R3A 1R9 [email protected]
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Section of Neurosurgery, Department of Surgery University of Manitoba
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Key Words: Mirror-image Neurofibromas, Neurofibroma, Neurofibromatosis, Tetraplegia Abbreviations: NF1=Neurofibromatosis 1, MRI=Magnetic Resonance Imaging, WHO=World
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ACCEPTED MANUSCRIPT Bigder 2 Abstract Neurofibromatosis 1 (NF1) is associated with increased incidence of spinal tumors including neurofibromas. The majority of NF1 associated spine neurofibromas are asymptomatic, however
RI PT
a minority of patients will experience neurological symptoms which can range from mild
paraesthesias, 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-
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image neurofibromas involving the entire spine. He was asymptomatic until age 14 when he developed neck pain and progressive tetraplegia with MRI showing severe cord compression
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secondary to bilateral C2 neurofibromas. Emergent cervical decompression was performed at C1-C3 along with debulking of bilateral neurofibromas. Post-operatively he regained full strength with no signs of myelopathy several years post-operatively. This case demonstrates a dramatic neuroimaging finding and emphasizes the potential for significant neurological
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follow-up.
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deterioration in previously asymptomatic NF1 patients, highlighting the need for long-term
ACCEPTED MANUSCRIPT Bigder 3 Extensive mirror-image neurofibromas of the entire spine resulting in spastic tetraplegia A 21 year-old male with typical cutaneous stigmata of NF1 was diagnosed with the disease in infancy and followed for multiple neurofibromas involving the entire spine. He was
RI PT
asymptomatic until age 14 at which point developed severe neck pain along with slowly
progressive weakness (4/5) of his extremities. Given his neurologic deterioration and correlated MRI findings of cord compression, we elected to proceed with cervical decompression and
SC
tumour debulking. In the 3-week interval between clinic visit and planned surgery, the patient developed spastic quadraparesis with grade 1 and 2 strength in arms and legs respectively and
M AN U
difficulty breathing. Emergent decompression at C1-C3 was performed along with debulking of bilateral tumors at C2 which was the level of maximal compression. Neuropathology findings were consistent with WHO grade 1 neurofibroma. Post-operatively the patient regained full strength with no signs of myelopathy and continues to be followed with serial MRI and clinic
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visits.
Figure 1: Sagittal T2 Fat Suppressed MRI demonstrates large neurofibromas at every level of
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neural foramina of cervical and thoracic spine.
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Figure 2: Coronal T2 Fat Suppressed images showing extensive bilateral plexiform neurofibromas involving lumbar (a) and thoracic (b) spine and paraspinal soft tissue.
Figure 3: Axial T2 at level of C2 demonstrates bilateral neurofibromas with significant thecal sac effacement and cord compression
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Abbreviations: NF1=Neurofibromatosis 1, MRI=Magnetic Resonance Imaging, WHO=World Health Organization
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S1878-8750(17)31440-7
DOI:
10.1016/j.wneu.2017.08.139
Reference:
WNEU 6389
To appear in:
World Neurosurgery
Received Date: 5 April 2017 Revised Date:
18 August 2017
Accepted Date: 22 August 2017
Please cite this article as: Bigder M, Szelemej P, Berrington N, Extensive mirror-image neurofibromas of the entire spine resulting in spastic tetraplegia, World Neurosurgery (2017), doi: 10.1016/ j.wneu.2017.08.139. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
Extensive mirror-image neurofibromas of the entire spine resulting in spastic tetraplegia Mark Bigder, Paul Szelemej, Neil Berrington
Neil Berrington MMed FRCS FCS(SA) Section of Neurosurgery University of Manitoba [email protected]
SC
TE D
Paul Szelemej MD Section of Neurosurgery University of Manitoba [email protected]
M AN U
Corresponding author: Mark Bigder MD Section of Neurosurgery Department of Surgery University of Manitoba GB129-820 Sherbrook Street Health Sciences Centre Winnipeg, MB R3A 1R9 [email protected]
RI PT
Section of Neurosurgery, Department of Surgery University of Manitoba
EP
Key Words: Mirror-image Neurofibromas, Neurofibroma, Neurofibromatosis, Tetraplegia Abbreviations: NF1=Neurofibromatosis 1, MRI=Magnetic Resonance Imaging, WHO=World
AC C
Health Organization
ACCEPTED MANUSCRIPT Bigder 2 Abstract Neurofibromatosis 1 (NF1) is associated with increased incidence of spinal tumors including neurofibromas. The majority of NF1 associated spine neurofibromas are asymptomatic, however
RI PT
a minority of patients will experience neurological symptoms which can range from mild
paraesthesias, 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-
SC
image neurofibromas involving the entire spine. He was asymptomatic until age 14 when he developed neck pain and progressive tetraplegia with MRI showing severe cord compression
M AN U
secondary to bilateral C2 neurofibromas. Emergent cervical decompression was performed at C1-C3 along with debulking of bilateral neurofibromas. Post-operatively he regained full strength with no signs of myelopathy several years post-operatively. This case demonstrates a dramatic neuroimaging finding and emphasizes the potential for significant neurological
AC C
EP
follow-up.
TE D
deterioration in previously asymptomatic NF1 patients, highlighting the need for long-term
ACCEPTED MANUSCRIPT Bigder 3 Extensive mirror-image neurofibromas of the entire spine resulting in spastic tetraplegia A 21 year-old male with typical cutaneous stigmata of NF1 was diagnosed with the disease in infancy and followed for multiple neurofibromas involving the entire spine. He was
RI PT
asymptomatic until age 14 at which point developed severe neck pain along with slowly
progressive weakness (4/5) of his extremities. Given his neurologic deterioration and correlated MRI findings of cord compression, we elected to proceed with cervical decompression and
SC
tumour debulking. In the 3-week interval between clinic visit and planned surgery, the patient developed spastic quadraparesis with grade 1 and 2 strength in arms and legs respectively and
M AN U
difficulty breathing. Emergent decompression at C1-C3 was performed along with debulking of bilateral tumors at C2 which was the level of maximal compression. Neuropathology findings were consistent with WHO grade 1 neurofibroma. Post-operatively the patient regained full strength with no signs of myelopathy and continues to be followed with serial MRI and clinic
TE D
visits.
Figure 1: Sagittal T2 Fat Suppressed MRI demonstrates large neurofibromas at every level of
EP
neural foramina of cervical and thoracic spine.
AC C
Figure 2: Coronal T2 Fat Suppressed images showing extensive bilateral plexiform neurofibromas involving lumbar (a) and thoracic (b) spine and paraspinal soft tissue.
Figure 3: Axial T2 at level of C2 demonstrates bilateral neurofibromas with significant thecal sac effacement and cord compression
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Abbreviations: NF1=Neurofibromatosis 1, MRI=Magnetic Resonance Imaging, WHO=World Health Organization
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![[The NMR tomographic findings in von Recklinghausen's disease with extensive neurofibromas].](/assets/img/hold.png)
