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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Bibliographic Details
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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Summary: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 
ISSN:0303-8467
1872-6968
DOI:10.1016/j.clineuro.2023.107985