Neurofibromatosis
The neurofibromatosis team is an active and diverse team composed of consultants, clinicians and researchers employed by both the University and the Trust. We conduct translational research covering neurofibromatosis and related conditions, from basic science through observational studies to clinical trials.
- Gene discovery. Work has been carried out over a number of years and has been fundamental in discovering the genetic basis of a range of conditions including SMARCE1 meningioma predisposition (2013), and LZTR1 schwannomatosis (2015)
- Clinical diagnostics. We have a widely recognized multidisciplinary team providing top level clinical and diagnostic services for patients with genetic disorders related to neurofibromatosis including NF1, NF2, schwannomatosis and SMARCE1 meningioma predisposition.
- From discovery to translation. The translation of such discoveries into improved health provision is a major aspect of our work. Our team has developed next-generation sequencing (NGS) panels including recently discovered genes SMARCE1, LZTR1 and SMARCB1, as well as offering highly sensitive RNA testing for NF1.
- We work closely with patient groups especially the Neuro Foundation to ensure our work is relevant to patient needs.
The team also provides direct support to the joint genetics & ophthalmology clinics hosted at the Manchester Royal Eye Hospital (MREH). We work in close collaboration with other leading sites in the UK including Oxford, Cambridge and Guy’s Hospital, London, and the NIHR Clinical Research Facility (CRF).
Team Members
MCGM Clinical & Research
MCGM Laboratory
University of Manchester
Patient Groups
Partner Institutions
Funding Partners
Research Projects
- Intervention trial of Simvastatin in Neurofibromatosis Type 1-Autism (SANTA, 2012-15)
- Early Development in Neurofibromatosis 1 (EDEN) study 2015
- Sorafenib window trial in NF2 (2014-2017)
- Nilotenib window trial in NF2 (2014-2017)
- FDG PET for growing vestibular schwannomas (2015-2017)
- Exon skipping for NF2 therapy (2015-)
- Gene identification in meningioma and schwannoma (2012-)
- Use of non-invasive brain stimulation for working memory deficits in NF1 (2017-2018, Funder Newlife)
- A pilot study of proteomics in NF1 Autism (2017-2018, Funder Baily Thomas Charitable Trust)
Recent Publications
- Smith MJ, Evans DGR. Cranial Meningioma in Neurofibromatosis Type 2 Patients: Role of Mutations. Tumors of the Central Nervous System, 2014; 11, 271-276
- Lloyd SK, Glynn FJ, Rutherford SA, King AT, Mawman DJ, O’Driscoll MP, Evans DG, Ramsden RT, Freeman SR. Ipsilateral cochlear implantation after cochlear nerve preserving vestibular schwannoma surgery in patients with neurofibromatosis type 2. Otol Neurotol. 2014;35(1):43-51
- Ferner RE, Shaw A, Evans DG, McAleer D, Halliday D, Parry A, Raymond FL, Durie-Gair J, Hanemann CO, Hornigold R, Axon P, Golding JF. Longitudinal evaluation of quality of life in 288 patients with neurofibromatosis 2. J Neurol. 2014; 261(5): 963-9 Mar 12.
- Smith MJ, Wallace AJ, Bowers NL, Eaton H, Evans DG.SMARCB1 mutations in schwannomatosis and genotype correlations with rhabdoid tumors. Cancer Genet. 2014 Apr 8. pii: S2210-7762(14)00068-4
- Smith MJ, Wallace AJ, Bennett C, Hasselblatt M, Elert-Dobkowska E, Evans LT, Hickey WF, van Hoff J, Bauer D, Lee A, Hevner RF, Beetz C, du Plessis D, Kilday J-P, Newman WG, Evans DG. Germline SMARCE1 mutations predispose to both spinal and cranial clear cell meningiomas. J. Pathol, 2014;234(4):436-40
- Tysome JR, Axon PR, Donnelly NP, Durie-Gair J, Evans DG, Ferner RE, et al. Current Concepts in Management of Vestibular Schwannomas in Neurofibromatosis Type 2 Current Otorhinolaryngology Reports, 1-8
- Garg S, Plasschaert E, Descheemaeker MJ, Huson S, Borghgraef M, Vogels A, Evans DG, Legius E, Green J. Autism Spectrum Disorder Profile in Neurofibromatosis Type I. J Autism Dev Disord. 2015. 45:1649-57
- Smith MJ, Isidor B, Beetz C, Williams SG, Bhaskar SS, Richer W, O’Sullivan J, Anderson B, Daly SB, Urquhart JE, Fryer A, Rustad CF, Mills SJ, Samii A, du Plessis D, Halliday D, Barbarot S, Bourdeaut F, Newman WG, Evans DG. Mutations in LZTR1 add to the complex heterogeneity of schwannomatosis. Neurology. 2015;84(2):141-7
- Evans DG, Freeman S, Gokhale C, Wallace A, Lloyd SK, Axon P, Ward CL, Rutherford S, King A, Huson SM, Ramsden RT; on Behalf of the Manchester NF2 service. Bilateral vestibular schwannomas in older patients: NF2 or chance? J Med Genet. 2015;52:422-5
- LEHTONEN A, GARG S, ROBERTS SA, TRUMP D, EVANS DG, GREEN J, HUSON SM. Cognition in children with neurofibromatosis type 1: data from a population-based study. Dev Med Child Neurol. 2015;
- Heineman TE, Evans DG, Campagne F, Selesnick SH. In Silico Analysis of NF2 Gene Missense Mutations in Neurofibromatosis Type 2: From Genotype to Phenotype. Otol Neurotol. 2015 ;36(5):908-14.
- Stivaros SM, Stemmer-Rachamimov AO, Alston R, Plotkin SR, Nadol JB, Quesnel A, O’Malley J, Whitfield GA, McCabe MG, Freeman SR, Lloyd SK, Wright NB, Kilday JP, Kamaly-Asl ID, Mills SJ, Rutherford SA, King AT, Evans DG. Multiple synchronous sites of origin of vestibular schwannomas in neurofibromatosis Type 2. J Med Genet. 2015;52(8):557-62
- Hexter A, Jones A, Joe H, Heap L, Smith MJ, Wallace AJ, Halliday D, Parry A, Taylor A, Raymond L, Shaw A, Afridi S, Obholzer R, Axon P, King AT; English Specialist NF2 Research Group, Friedman JM, Evans DG. Clinical and molecular predictors of mortality in neurofibromatosis 2: a UK national analysis of 1192 patients. J Med Genet. 2015;52(10):699-705
- Li KL, Djoukhadar I, Zhu X, Zhao S, Lloyd S, McCabe M, McBain C, Evans DG, Jackson A. Vascular biomarkers derived from dynamic contrast-enhanced MRI predict response of vestibular schwannoma to antiangiogenic therapy in type 2 neurofibromatosis. Neuro Oncol. 2016 Feb;18(2):275-82
- Smith MJ, Urquhart JE, Harkness EF, Miles EK, Bowers NL, Byers HJ, Bulman M, Gokhale C, Wallace AJ, Newman WG, Evans DG.The Contribution of Whole Gene Deletions and Large Rearrangements to the Mutation Spectrum in Inherited Tumor Predisposing Syndromes. Hum Mutat. 2016;37(3):250-6 10.1002/humu.22938. [Epub ahead of print]
- Morris KA., Golding JF, Axon PR., Afridi S, Blesing C. Ferner RE, Halliday D, Jena R, Pretorius PM. the UK NF2 Research group, Evans DG†,. McCabe MG †, Parry A†. Bevacizumab in Neurofibromatosis type 2 (NF2) related vestibular schwannomas: a nationally coordinated approach to delivery and prospective evaluation. Neuro-Oncology Practice 2016; 0, 1–9, doi:10.1093/nop/npv065
- Lloyd SK, Evans DG, Neurofibromatosis type 2 service delivery in England Neurochirurgie 2016; Jan 27. pii: S0028-3770(15)00279-9
- Evans DG, Bowers N, Burkitt-Wright E, Miles E, Garg S, Scott-Kitching V, Penman-Splitt M, Dobbie A, Howard E, Ealing J, Vassalo G, Wallace AJ, Newman W, UK Northern NF Group, Huson SM. Comprehensive RNA Analysis of the NF1 Gene in Classically Affected NF1 Affected Individuals Meeting NIH Criteria has High Sensitivity and Mutation Negative Testing is Reassuring in Isolated Cases With Pigmentary Features Only. eBioMedicine 2016; 7:212-20
- Garg S, Heuvelman H, Huson S, Tobin H, Green J; Northern UK NF1 Research Network. Sex bias in autism spectrum disorder in neurofibromatosis type 1. J Neurodev Disord. 2016;8:26
- Smith MJ, Bowers NL, Bulman M, Gokhale C, Wallace AJ, King AT, Lloyd SK, Rutherford SA, Hammerbeck-Ward CL, Freeman SR, Evans DG. Revisiting neurofibromatosis type 2 diagnostic criteria to exclude LZTR1-related schwannomatosis. Neurology. 2017 Jan 3;88(1):87-92.