Download PDF
J Neurol Surg B Skull Base
DOI: 10.1055/a-2607-6174
Original Article

Trigeminal Neuralgia in NF2-Related Schwannomatosis

Shazia K. Afridi
1   Department of Neurology, Guy's and St Thomas NHS Trust, London, United Kingdom
,
Amoolya Mannava
2   Department of Radiology, Guy's and St Thomas NHS Trust, London, United Kingdom
,
Duaa Ali Faruqi
3   School of Medical Education King's College, London, United Kingdom
,
Lalani Carlton-Jones
2   Department of Radiology, Guy's and St Thomas NHS Trust, London, United Kingdom
› Author Affiliations
› Further Information
Also available at
   Permissions and Reprints

Abstract

Objectives

NF2-related schwannomatosis (NF2) is an autosomal dominant syndrome resulting in a predisposition for benign tumors of the nervous system, including schwannomas, meningiomas, and ependymomas. There is a lack of data on the prevalence of trigeminal neuralgia (TN) in NF2 and the relationship between trigeminal schwannomas (TS) and TN in NF2 is also unclear. We sought to investigate the factors predisposing NF2 patients to TN.

Methods

We performed a retrospective review of case notes and magnetic resonance imaging (MRI) in our cohort of adult NF2 patients over the age of 16 years meeting the international diagnostic criteria for NF2. We assessed presence and size of TS, size of vestibular schwannoma, clinical findings of neuropathy/pain, history of headache disorder, and intervention. We then looked at the subset of patients with TS and our radiologist reviewed their MRI brain imaging

Results

Fifteen of 189 NF2 patients had TN. Only six had ipsilateral TS. Ipsilateral vestibular schwannomas were significantly larger in the TN subgroup, as was history of ipsilateral radiation and migraine.

Conclusion

The majority of TN patients did not have an ipsilateral trigeminal schwannoma. Our data suggest factors associated with TN include size of vestibular schwannoma, history of previous radiotherapy treatment, and migraine.

Keywords

NF2 - schwannomatosis - trigeminal neuralgia - neurofibromatosis type 2


Publication History

Received: 19 March 2025

Accepted: 12 May 2025

Accepted Manuscript online:
14 May 2025

Article published online:
04 June 2025

© 2025. Thieme. All rights reserved.

Georg Thieme Verlag KG
Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany

 

  • References

  • 1 Forde C, Smith MJ, Burghel GJ. et al. NF2-related schwannomatosis and other schwannomatosis: an updated genetic and epidemiological study. J Med Genet 2024; 61 (09) 856-860
    PubMedSearch in Google Scholar
  • 2 Fisher LM, Doherty JK, Lev MH, Slattery III WH. Distribution of nonvestibular cranial nerve schwannomas in neurofibromatosis 2. Otol Neurotol 2007; 28 (08) 1083-1090
    PubMedSearch in Google Scholar
  • 3 Moualed D, Wong J, Thomas O. et al. Prevalence and natural history of schwannomas in neurofibromatosis type 2 (NF2): the influence of pathogenic variants. Eur J Hum Genet 2022; 30 (04) 458-464
    PubMedSearch in Google Scholar
  • 4 Headache Classification Committee of the International Headache Society. The International Classification of Headache Disorders. 3rd edition (beta version). Cephalalgia 2013; 33 (09) 629-808
    CrossrefPubMedSearch in Google Scholar
  • 5 Mueller D, Obermann M, Yoon MS. et al. Prevalence of trigeminal neuralgia and persistent idiopathic facial pain: a population-based study. Cephalalgia 2011; 31 (15) 1542-1548
    CrossrefPubMedSearch in Google Scholar
  • 6 Ferner RE, Shaw A, Evans DG. et al. Longitudinal evaluation of quality of life in 288 patients with neurofibromatosis 2. J Neurol 2014; 261 (05) 963-969
    PubMedSearch in Google Scholar
  • 7 Plotkin SR, Messiaen L, Legius E. et al; International Consensus Group on Neurofibromatosis Diagnostic Criteria (I-NF-DC). Updated diagnostic criteria and nomenclature for neurofibromatosis type 2 and schwannomatosis: an international consensus recommendation. Genet Med 2022; 24 (09) 1967-1977
    CrossrefPubMedSearch in Google Scholar
  • 8 Jensen TS, Rasmussen P, Reske-Nielsen E. Association of trigeminal neuralgia with multiple sclerosis: clinical and pathological features. Acta Neurol Scand 1982; 65 (03) 182-189
    PubMedSearch in Google Scholar
  • 9 Nomura T, Ikezaki K, Matsushima T, Fukui M. Trigeminal neuralgia: differentiation between intracranial mass lesions and ordinary vascular compression as causative lesions. Neurosurg Rev 1994; 17 (01) 51-57
    PubMedSearch in Google Scholar
  • 10 Stovner LJ, Hagen K, Linde M, Steiner TJ. The global prevalence of headache: an update, with analysis of the influences of methodological factors on prevalence estimates. J Headache Pain 2022; 23 (01) 34
    CrossrefPubMedSearch in Google Scholar
  • 11 May A, Goadsby PJ. The trigeminovascular system in humans: pathophysiologic implications for primary headache syndromes of the neural influences on the cerebral circulation. J Cereb Blood Flow Metab 1999; 19 (02) 115-127
    PubMedSearch in Google Scholar
  • 12 Ostertag C, Friedman TN, Keough MB, Kerr BJ, Sankar T. Heightened presence of inflammatory mediators in the cerebrospinal fluid of patients with trigeminal neuralgia. Pain Rep 2023; 8 (06) e1117
    PubMedSearch in Google Scholar
  • 13 Matsuda M, Huh Y, Ji RR. Roles of inflammation, neurogenic inflammation, and neuroinflammation in pain. J Anesth 2019; 33 (01) 131-139
    CrossrefPubMedSearch in Google Scholar
  • 14 de Vries M, Briaire-de Bruijn I, Malessy MJ, de Bruïne SF, van der Mey AG, Hogendoorn PC. Tumor-associated macrophages are related to volumetric growth of vestibular schwannomas. Otol Neurotol 2013; 34 (02) 347-352
    PubMedSearch in Google Scholar
  • 15 Lewis D, Donofrio CA, O'Leary C. et al. The microenvironment in sporadic and neurofibromatosis type II-related vestibular schwannoma: the same tumor or different? A comparative imaging and neuropathology study. J Neurosurg 2020; 134 (05) 1419-1429
    PubMedSearch in Google Scholar
  • 16 Maarbjerg S, Di Stefano G, Bendtsen L, Cruccu G. Trigeminal neuralgia - diagnosis and treatment. Cephalalgia 2017; 37 (07) 648-657
    PubMedSearch in Google Scholar
  • 17 Berti A, Granville M, Wu X, Huang D, Schwade JG, Jacobson RE. Delayed development of trigeminal neuralgia after radiosurgical treatment of a tentorial meningioma. Cureus 2017; 9 (08) e1628
    PubMedSearch in Google Scholar
  • 18 Brisman R. Gamma knife radiosurgery for primary management for trigeminal neuralgia. J Neurosurg 2000; 93 (Suppl. 03) 159-161
    PubMedSearch in Google Scholar
  • 19 Peciu-Florianu I, Régis J, Levivier M, Dedeciusova M, Reyns N, Tuleasca C. Trigeminal neuralgia secondary to meningiomas and vestibular schwannoma is improved after stereotactic radiosurgery: a systematic review and meta-analysis. Stereotact Funct Neurosurg 2021; 99 (01) 6-16
    PubMedSearch in Google Scholar