Determining the risk of spinal pathology progression in neurofibromatosis type 1 patients – a national tertiary neurofibromatosis type 1 centre study

Neurofibromatosis type 1 (NF1) gives rise to a variety of spinal pathologies that include dural ectasia (DE), vertebral malalignments (VMA), spinal deformities (SD), syrinx, meningoceles, spinal nerve root tumours (SNRT), and spinal plexiform tumours (SPT). The relationship between these and the pro...

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Published in:Clinical neurology and neurosurgery 2023-11, Vol.234, p.107985-107985, Article 107985
Main Authors: Robinson, Daniel, Biswas, Sayan, Torrie, Christopher, MacArthur, Joshua, Snowdon, Ella, Sial, Moska, Sarkar, Ved, George, K. Joshi
Format: Article
Language:English
Subjects:
Back pain
Chi-square test
Decision making
Disease progression
Genetic disorders
Meninges
Neurofibromatosis
Neurofibromatosis Type 1
Neurological disorders
Neurology
Pathology
Patients
Recklinghausen's disease
Regression analysis
Scoliosis
Spinal nerves
Spinal NF1
Statistical analysis
Tumors
Vertebrae
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container_title Clinical neurology and neurosurgery
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creator Robinson, Daniel
Biswas, Sayan
Torrie, Christopher
MacArthur, Joshua
Snowdon, Ella
Sial, Moska
Sarkar, Ved
George, K. Joshi
description Neurofibromatosis type 1 (NF1) gives rise to a variety of spinal pathologies that include dural ectasia (DE), vertebral malalignments (VMA), spinal deformities (SD), syrinx, meningoceles, spinal nerve root tumours (SNRT), and spinal plexiform tumours (SPT). The relationship between these and the progression of these pathologies has not been explored before in detail and this paper aims to address this. Data was retrospectively collected from adult NF1 multi-disciplinary team meetings from 2016 to 2022 involving a total of 593 patients with 20 distinct predictor variables. Data were analyzed utilizing; Chi-Square tests, binary logistic regression, and Kaplan-Meier analysis. SNRT (19.9%), SD (18.6%), and (17.7%) of VMA had the highest rates of progression. SD was significantly associated (p 
doi_str_mv 10.1016/j.clineuro.2023.107985
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SD was significantly associated (p &lt; 0.02) with the presence and progression of all spinal pathologies except for SPT. Statistically significant predictors of SD progression included the presence of DVA, VMA, syrinx, meningocele, and SNRT. Kaplan-Meier analysis revealed no statistically significant difference between the times to progression for SD (85 days), SNRT (1196 days), and VMA (2243 days). This paper explores for the first time in detail, the progression of various spinal pathologies in NF1. The presence and progression of SD is a key factor that correlated with the progression of different spinal pathologies. Early identification of SD may help support clinical decision-making and guide radiological follow-up protocols and treatment. •Association between spinal pathologies and NF1 progression is unknown.•Analysis of 593 NF1 patients with 20 spinal pathologies at a national NF1 centre.•Key predictors of disease progression: spinal deformity, nerve root tumours, and vertebral malalignment.•No significant difference in median time to radiological progression despite high 5-year progression rates.•Early identification aids clinical decisions and guides radiological follow-up for such pathologies.</description><identifier>ISSN: 0303-8467</identifier><identifier>EISSN: 1872-6968</identifier><identifier>DOI: 10.1016/j.clineuro.2023.107985</identifier><language>eng</language><publisher>Assen: Elsevier B.V</publisher><subject>Back pain ; Chi-square test ; Decision making ; Disease progression ; Genetic disorders ; Meninges ; Neurofibromatosis ; Neurofibromatosis Type 1 ; Neurological disorders ; Neurology ; Pathology ; Patients ; Recklinghausen's disease ; Regression analysis ; Scoliosis ; Spinal nerves ; Spinal NF1 ; Statistical analysis ; Tumors ; Vertebrae</subject><ispartof>Clinical neurology and neurosurgery, 2023-11, Vol.234, p.107985-107985, Article 107985</ispartof><rights>2023 Elsevier B.V.</rights><rights>2023. 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Joshi</creatorcontrib><title>Determining the risk of spinal pathology progression in neurofibromatosis type 1 patients – a national tertiary neurofibromatosis type 1 centre study</title><title>Clinical neurology and neurosurgery</title><description>Neurofibromatosis type 1 (NF1) gives rise to a variety of spinal pathologies that include dural ectasia (DE), vertebral malalignments (VMA), spinal deformities (SD), syrinx, meningoceles, spinal nerve root tumours (SNRT), and spinal plexiform tumours (SPT). The relationship between these and the progression of these pathologies has not been explored before in detail and this paper aims to address this. Data was retrospectively collected from adult NF1 multi-disciplinary team meetings from 2016 to 2022 involving a total of 593 patients with 20 distinct predictor variables. Data were analyzed utilizing; Chi-Square tests, binary logistic regression, and Kaplan-Meier analysis. SNRT (19.9%), SD (18.6%), and (17.7%) of VMA had the highest rates of progression. SD was significantly associated (p &lt; 0.02) with the presence and progression of all spinal pathologies except for SPT. Statistically significant predictors of SD progression included the presence of DVA, VMA, syrinx, meningocele, and SNRT. Kaplan-Meier analysis revealed no statistically significant difference between the times to progression for SD (85 days), SNRT (1196 days), and VMA (2243 days). This paper explores for the first time in detail, the progression of various spinal pathologies in NF1. The presence and progression of SD is a key factor that correlated with the progression of different spinal pathologies. 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Joshi</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Determining the risk of spinal pathology progression in neurofibromatosis type 1 patients – a national tertiary neurofibromatosis type 1 centre study</atitle><jtitle>Clinical neurology and neurosurgery</jtitle><date>2023-11</date><risdate>2023</risdate><volume>234</volume><spage>107985</spage><epage>107985</epage><pages>107985-107985</pages><artnum>107985</artnum><issn>0303-8467</issn><eissn>1872-6968</eissn><abstract>Neurofibromatosis type 1 (NF1) gives rise to a variety of spinal pathologies that include dural ectasia (DE), vertebral malalignments (VMA), spinal deformities (SD), syrinx, meningoceles, spinal nerve root tumours (SNRT), and spinal plexiform tumours (SPT). The relationship between these and the progression of these pathologies has not been explored before in detail and this paper aims to address this. 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source ScienceDirect Journals
subjects Back pain
Chi-square test
Decision making
Disease progression
Genetic disorders
Meninges
Neurofibromatosis
Neurofibromatosis Type 1
Neurological disorders
Neurology
Pathology
Patients
Recklinghausen's disease
Regression analysis
Scoliosis
Spinal nerves
Spinal NF1
Statistical analysis
Tumors
Vertebrae
title Determining the risk of spinal pathology progression in neurofibromatosis type 1 patients – a national tertiary neurofibromatosis type 1 centre study
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