Have a personal or library account? Click to login
Exploring pathogenesis, prevalence, and genetic associations in Chiari malformation type 1: a contemporary perspective Cover

Exploring pathogenesis, prevalence, and genetic associations in Chiari malformation type 1: a contemporary perspective

Open Access
|Sep 2024

References

  1. Lacy M, Dedios-Stern S, Fredrickson S, Parikh S, Nader T, Frim DM. Prevalence of psychiatric diagnoses in pediatric Chiari malformation type 1. Pediatr Neurosurg. 2018; 53:371–8.
  2. Mueller DM, Oro’ JJ. Prospective analysis of presenting symptoms among 265 patients with radiographic evidence of Chiari malformation type I with or without syringomyelia. J Am Acad Nurse Pract. 2004; 16:134–8.
  3. Speer MC, Enterline DS, Mehltretter L, Hammock P, Joseph J, Dickerson M, et al. Review article: Chiari type I malformation with or without syringomyelia: prevalence and genetics. J Genet Couns. 2003; 12:297–311.
  4. Nishikawa M, Sakamoto H, Hakuba A, Nakanishi N, Inoue Y. Pathogenesis of Chiari malformation: a morphometric study of the posterior cranial fossa. J Neurosurg. 1997; 86:40–7.
  5. Abbott D, Brockmeyer D, Neklason DW, Teerlink C, Cannon-Albright LA. Population-based description of familial clustering of Chiari malformation type I. J Neurosurg. 2018; 128:460–5.
  6. Markunas CA, Enterline DS, Dunlap K, Soldano K, Cope H, Stajich J, et al. Genetic evaluation and application of posterior cranial fossa traits as endophenotypes for Chiari type I malformation. Ann Hum Genet. 2014; 78:1–12.
  7. Schijman E. History, anatomic forms, and pathogenesis of Chiari I malformations. Childs Nerv Syst. 2004; 20:323–8.
  8. Tubbs RS, Cohen-Gadol AA. Hans Chiari (1851–1916). J Neurol. 2010; 257:1218–20.
  9. Arslan A, Olguner SK, Acik V, İstemen İ, Arslan B, Ökten Aİ, Gezercan Y. Surgical outcomes of C1-2 posterior stabilization in patients with Chiari malformation type 1. Global Spine J. 2022; 12:37–44.
  10. Goel A, Milhorat TH, Kula RW, Mandell M. Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery. 1999; 45:1497–8.
  11. Arnautovic A, Splavski B, Boop FA, Arnautovic KI. Pediatric and adult Chiari malformation type I surgical series 1965–2013: a review of demographics, operative treatment, and outcomes. J Neurosurg Pediatr. 2015; 15:161–77.
  12. Bogdanov EI, Faizutdinova AT, Mendelevich EG, Sozinov AS, Heiss JD. Epidemiology of symptomatic Chiari malformation in Tatarstan: regional and ethnic differences in prevalence. Neurosurgery. 2019; 84:1090–7.
  13. Brickell KL, Anderson NE, Charleston AJ, Hope JK, Bok AP, Barber PA. Ethnic differences in syringomyelia in New Zealand. J Neurol Neurosurg Psychiatry. 2006; 77:989–91.
  14. Doberstein CA, Torabi R, Klinge PM. Current concepts in the pathogenesis, diagnosis, and management of type I Chiari malformations. R I Med J. 2017; 100:47–9.
  15. Stovner LJ, Bergan U, Nilsen G, Sjaastad O. Posterior cranial fossa dimensions in the Chiari I malformation: relation to pathogenesis and clinical presentation. Neuroradiology. 1993; 35:113–8.
  16. Markunas CA, Soldano K, Dunlap K, Cope H, Asiimwe E, Stajich J, et al. Stratified whole genome linkage analysis of Chiari type I malformation implicates known Klippel-Feil syndrome genes as putative disease candidates. PLoS One. 2013; 8:e61521. doi: 10.1371/journal.pone.0061521
  17. Marín-Padilla M. Cephalic axial skeletal-neural dysraphic disorders: embryology and pathology. Can J Neurol Sc. 1991; 18:153–69.
  18. Capra V, Iacomino M, Accogli A, Pavanello M, Zara F, Cama A, De Marco P. Chiari malformation type I: what information from the genetics? Childs Nerv Syst. 2019; 35:1665–71.
  19. Imbard A, Benoist JF, Blom HJ. Neural tube defects, folic acid and methylation. Int J Environ Res Pub Health. 2013; 10:4352–89.
  20. Burren KA, Savery D, Massa V, Kok RM, Scott JM, Blom HJ, et al. Gene-environment interactions in the causation of neural tube defects: folate deficiency increases susceptibility conferred by loss of Pax3 function. Hum Mol Genet. 2008; 17:3675–85.
  21. Kong JH, Young CB, Pusapati GV, Espinoza FH, Patel CB, Beckert F, et al. Gene-teratogen interactions influence the penetrance of birth defects by altering Hedgehog signaling strength. Development. 2021; 148:dev199867. doi: 10.1242/dev.199867
  22. Heiss JD, Argersinger DP. Epidemiology of Chiari I malformation. In: Tubbs RS, Turgut M, Oakes WJ (eds) The Chiari Malformations. Springer International Publishing, Cham; 2020, p 263–74.
  23. Fraga MF, Ballestar E, Paz MF, Ropero S, Setien F, Ballestar ML, et al. Epigenetic differences arise during the lifetime of monozygotic twins. Proc Natl Acad Sci U S A. 2005; 102:10604–9.
  24. Baisden J. Controversies in Chiari I malformations. Surg Neurol Int. 2012; 3(Suppl 3):S232–7.
  25. Telano LN, Baker S. Physiology, cerebral spinal fluid (CSF). In: StatPearls [Internet]. Treasure Island, FL: StatPearls Publishing; 2019. [cited 2019 Dec 5]. Available from http://www.ncbi.nlm.nih.gov/books/NBK519007/.
  26. Lloyd RA, Stoodley MA, Bilston LE. Statistical shape models of the posterior cranial fossa and hindbrain volumes may provide a more robust clinical metric for Chiari malformation. J Biomech. 2022; 137:111093. doi: 10.1016/j.jbiomech.2022.111093
  27. Langridge B, Phillips E, Choi D. Chiari malformation type 1: a systematic review of natural history and conservative management. World Neurosurg. 2017; 104:213–9.
  28. Merello E, Tattini L, Magi A, Accogli A, Piatelli G, Pavanello M, et al. Exome sequencing of two Italian pedigrees with non-isolated Chiari malformation type I reveals candidate genes for craniofacial development. Eur J Hum Genet. 2017; 25:952–9.
  29. Mekbib KY, Muñoz W, Allington G, McGee S, Mehta NH, Shofi JP, et al. Human genetics and molecular genomics of Chiari malformation type 1. Trends Mol Med. 2023; 29:1059–75.
  30. Musolf AM, Ho WSC, Long KA, Zhuang Z, Argersinger DP, Sun H, et al. Small posterior fossa in Chiari I malformation affected families is significantly linked to 1q43–44 and 12q23–24.11 using whole exome sequencing. Eur J Hum Genet. 2019; 27:1599–610.
  31. Sadler B, Wilborn J, Antunes L, Kuensting T, Hale AT, Gannon SR, et al. Rare and de novo coding variants in chromodomain genes in Chiari I malformation. Am J Hum Genet. 2021; 108:530–1.
  32. Haller G, Sadler B. Elucidating the genetic basis of Chiari I malformation. Neurosurg Clin N Am. 2023; 34:55–60.
  33. Glinka A, Wu W, Delius H, Monaghan AP, Blumenstock C, Niehrs C. Dickkopf-1 is a member of a new family of secreted proteins and functions in head induction. Nature. 1998; 391:357–62.
  34. Martínez-Gil N, Grinberg D, Balcells S. Search for variants of the LRP4 gene in women with high bone mass and in patients with Chiari type I malformation. Rev Osteoporos Metab Miner. 2021; 13:17–20.
  35. Speer MC, George TM, Enterline DS, Franklin A, Wolpert CM, Milhorat TH. A genetic hypothesis for Chiari I malformation with or without syringomyelia. Neurosurg Focus. 2000; 8:E12. doi: 10.3171/foc.2000.8.3.12
  36. Strahle J, Muraszko KM, Buchman SR, Kapurch J, Garton HJ, Maher CO. Chiari malformation associated with craniosynostosis. Neurosurg Focus. 2011; 31:E2. doi: 10.3171/2011.6.FOCUS11107
  37. Cinalli G, Spennato P, Sainte-Rose C, Arnaud E, Aliberti F, Brunelle F, et al. Chiari malformation in craniosynostosis. Childs Nerv Syst. 2005; 21:889–901.
  38. Ciurea AV, Toader C. Genetics of craniosynostosis: review of the literature. J Med Life. 2009; 2:5–17.
  39. Guille JT, Sherk HH. Congenital osseous anomalies of the upper and lower cervical spine in children. J Bone Joint Surg Am. 2002; 84:277–88.
  40. Jena BR, Mishra RK, Dube SK, Rath GP, Malik V, Gurjar HK. Perioperative management of a child with Klippel–Feil syndrome and severe uncorrected aortic stenosis undergoing cervical spine stabilization. J Pediatr Neurosci. 2021; 16:257–60.
  41. Clarke RA, Singh S, McKenzie H, Kearsley JH, Yip MY. Familial Klippel-Feil syndrome and paracentric inversion inv(8) (q22.2q23.3). Am J Hum Genet. 1995; 57:1364–70.
  42. Tassabehji M, Fang ZM, Hilton EN, McGaughran J, Zhao Z, de Bock CE, et al. Mutations in GDF6 are associated with vertebral segmentation defects in Klippel-Feil syndrome. Hum Mutat. 2008; 29:1017–27.
  43. Li S, Zhou H, Qin H, Guo H, Bai Y. A novel mutation in the COL2A1 gene in a Chinese family with spondyloepiphyseal dysplasia congenita. Joint Bone Spine. 2014; 81:86–9.
  44. Dikaiakou E, Vlachopapadopoulou EA, Manolakos E, Samelis P, Margariti R, Zampakides C, Michalacos S. Identification of an autosomal dominant mutation in the COL2A1 gene leading to spondyloepiphyseal dysplasia congenita in a Greek family. Mol Syndromol. 2019; 9:241–6.
  45. Fatima N, Swift DM, Montgomery EY, Weprin BE, Price AV, Whittemore BA, Braga BP. Risk factors for clinical and radiological worsening following Chiari malformation type I surgery in the pediatric population. Neurosurg Focus. 2023; 54:E7. doi: 10.3171/2022.12.FOCUS22630
  46. Ciaramitaro P, Migliaretti G, Ferraris M, Garnero A, Morana G, Carucci P, et al. Syringomyelia associated with Chiari 1 malformation in adults: positive outcome predictors after posterior fossa decompression with duraplasty. J Clin Med. 2023; 12:3019. doi: 10.3390/jcm12083019
  47. Mugge L, Caras A, Henkel N, Dassi C, Schroeder J, Prevedello DM. Headache and other symptoms in Chiari malformation type I are associated with cerebrospinal fluid flow improvement after decompression: a two-institutional study. World Neurosurg. 2022; 163:e253–62.
  48. Hwang JK, Park EK, Shim KW, Kim DS. Effectiveness of C1 laminectomy for Chiari malformation type 1: posterior fossa volume expansion and syrinx-volume decrease rate. Yonsei Med J. 2023; 64:191–6.
  49. Barpujari A, Kiley A, Ross JA, Veznedaroglu E. A systematic review of non-opioid pain management in Chiari malformation (type 1) patients: current evidence and novel therapeutic opportunities. J Clin Med. 2023; 12:3064. doi: 10.3390/jcm12093064
  50. Garcia MA, Allen PA, Li X, Houston JR, Loth F, Labuda R, Delahanty DL. An examination of pain, disability, and the psychological correlates of Chiari malformation pre-and post-surgical correction. Disabil Health J. 2019; 12:649–56.
  51. Wang J, Wang D, Zeng L, Wang S. [An infant with premature closure of cranial sutures due to variant of ERF gene and a literature review]. Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2023; 40:1009–14.
  52. Urbizu A, Garrett ME, Soldano K, Drechsel O, Loth D, Marcé-Grau A, et al. Rare functional genetic variants in COL7A1, COL6A5, COL1A2 and COL5A2 frequently occur in Chiari malformation type 1. PLoS One. 2021; 16:e0251289. doi: 10.1371/journal.pone.0251289
  53. Fujisawa H, Hasegawa M, Kida S, Yamashita J. A novel fibroblast growth factor receptor 2 mutation in Crouzon syndrome associated with Chiari type I malformation and syringomyelia. J Neurosurg. 2002; 97:396–400.
  54. Rosenblum JS, Cappadona AJ, Argersinger DP, Pang Y, Wang H, Nazari MA, . Neuraxial dysraphism in EPAS1-Associated syndrome due to improper mesenchymal transition. Neurol Genet. 2020; 6:e414. doi: 10.1212/NXG.0000000000000414
  55. Avşar T, Çaliş Ş, Yilmaz B, Demirci Otluoğlu G, Holyavkin C, Kiliç T. Genome-wide identification of Chiari malformation type I associated candidate genes and chromosomal variations. Turk J Biol. 2020; 44:449–56.
DOI: https://doi.org/10.2478/abm-2024-0021 | Journal eISSN: 1875-855X | Journal ISSN: 1905-7415
Language: English
Page range: 148 - 156
Published on: Sep 20, 2024
Published by: Chulalongkorn University
In partnership with: Paradigm Publishing Services
Publication frequency: 6 issues per year

© 2024 Siti Nornazihah Mohd Rosdi, Suzuanhafizan Omar, Mazira Mohamad Ghazali, Ab Rahman Izaini Ghani, Abdul Aziz Mohamed Yusoff, published by Chulalongkorn University
This work is licensed under the Creative Commons Attribution 4.0 License.