The site of the Media Sphera Publishers contains materials intended solely for healthcare professionals.
By closing this message, you confirm that you are a certified medical professional or a student of a medical educational institution.

Dislocations of the cerebellar tonsils in the large occipital foramen and the spectrum of Chiari malformations Type 1

Journal: . 2022;122(4): 7‑15

Read: 8029 times


To cite this article:

Dislocations of the cerebellar tonsils in the large occipital foramen and the spectrum of Chiari malformations Type 1. . 2022;122(4):7‑15. (In Russ.)
https://doi.org/10.17116/jnevro20221220417

Recommended articles:
Clinical case of auri­cular gouty tophi. Russian Bulletin of Otorhinolaryngology. 2024;(6):80-84
Obstructive sleep apnea syndrome as a predictor of atrial fibrillation. Russian Journal of Preventive Medi­cine. 2025;(1):109-114
Virus-associated progesterone-resistant chro­nic endo­metritis — the onco­risk situation?. Russian Bulletin of Obstetrician-Gynecologist. 2025;(1):25-30
Modern approaches to cystic lung diseases diagnosis. Journal of Respiratory Medi­cine. 2025;(1):30-37
Primary bile acid synthesis diso­rders. Russian Journal of Evidence-Based Gastroenterology. 2025;(1):71-90

References:

  1. Goel A. Is Chiari a «formation» or a «malformation»? J Craniovertebr Junction Spine. 2017;8:1-2.  https://doi.org/10.4103/0974-8237.199881
  2. Bolognese PA, Brodbelt A, Bloom AB, et al. Chiari I Malformation: Opinions on Diagnostic Trends and Controversies from a Panel of 63 International Experts. World Neurosurg. 2019;130:9-16.  https://doi.org/10.1016/j.wneu.2019.05.098
  3. Frič R, Eide PK. Chiari type 1-a malformation or a syndrome? A critical review. Acta Neurochir (Wien). 2020;162:1513-1525. https://doi.org/10.1007/s00701-019-04100-2
  4. Faizutdinova AT, Bogdanov EI. Clinical and radiological rationale for distinguishing subtypes of primary Chiari I malformation. S.S. Korsakov Journal of Neurology and Psychiatry = Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova. 2020;120:64-69. (In Russ.). https://doi.org/10.17116/jnevro202012008164
  5. Mendelevich EG, Mikhaylov MK, Bogdanov EI. Siringomieliya I mal’formatsiya Arnolda—Kiari (kliniko-nejrovizual’nye aspekty). Kazan: Meditsina; 2002. (In Russ.).
  6. Bejjani GK. Definition of the adult Chiari malformation: a brief historical overview. Neurosurg Focus. 2001;11:E1.  https://doi.org/10.3171/foc.2001.11.1.2
  7. Marin-Padilla M, Marin-Padilla TM. Morphogenesis of experimentally induced Arnold — Chiari malformation. J Neurol Sci. 1981;50:29-55.  https://doi.org/10.1016/0022-510x(81)90040-x
  8. Milhorat TH, Nishikawa M, Kula RW, Dlugacz YD. Mechanisms of cerebellar tonsil herniation in patients with Chiari malformations as guide to clinical management. Acta Neurochir (Wien). 2010;152:1117-1127. https://doi.org/10.1007/s00701-010-0636-3
  9. Raybaud C, Jallo GI. Chiari 1 deformity in children: etiopathogenesis and radiologic diagnosis. Handb Clin Neurol. 2018;155:25-48.  https://doi.org/10.1016/B978-0-444-64189-2.00002-0
  10. Nishikawa M, Bolognese PA, Kula R, et al. Pathogenesis and Classification of Chiari Malformation Type I Based on the Mechanism of Ptosis of the Brain Stem and Cerebellum: A Morphometric Study of the Posterior Cranial Fossa and Craniovertebral Junction. Journal of Neurological Surgery Part B: Skull Base. 2019; (Online ahead of print). https://doi.org/10.1055/s-0039-1691832
  11. Aquilina K, Merchant TE, Boop FA, Sanford RA. Chiari I malformation after cranial radiation therapy in childhood: a dynamic process associated with changes in clival growth. Childs Nerv Syst. 2009;25:1429-1436. https://doi.org/10.1007/s00381-009-0982-8
  12. Barkovich AJ, Wippold FJ, Sherman JL, Citrin CM. Significance of cerebellar tonsillar position on MR. AJNR Am J Neuroradiol. 1986;7:795-799. 
  13. Smith BW, Strahle J, Bapuraj JR, et al. Distribution of cerebellar tonsil position: implications for understanding Chiari malformation. J Neurosurg. 2013;119:812-819.  https://doi.org/10.3171/2013.5.JNS121825
  14. Milhorat TH, Chou MW, Trinidad EM, et al. Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery. 1999;44:1005-10017. https://doi.org/10.1097/00006123-199905000-00042
  15. Bogdanov EI, Faizutdinova AT, Mendelevich EG, et al. Epidemiology of Symptomatic Chiari Malformation in Tatarstan: Regional and Ethnic Differences in Prevalence. Neurosurgery. 2019;84:1090-1097. https://doi.org/10.1093/neuros/nyy175
  16. Sekula RF Jr, Jannetta PJ, Casey KF, et al. Dimensions of the posterior fossa in patients symptomatic for Chiari I malformation but without cerebellar tonsillar descent. Cerebrospinal Fluid Res. 2005;2:11.  https://doi.org/10.1186/1743-8454-2-11
  17. Bogdanov EI, Heiss JD, Mendelevich EG, et al. Clinical and neuroimaging features of «idiopathic» syringomyelia. Neurology. 2004;62:791-794.  https://doi.org/10.1212/01.wnl.0000113746.47997.ce
  18. Meadows J, Kraut M, Guarnieri M, et al. Asymptomatic Chiari Type I malformations identified on magnetic resonance imaging. J Neurosurg. 2000;92:920-926.  https://doi.org/10.3171/jns.2000.92.6.0920
  19. Strahle J, Muraszko KM, Kapurch J, et al. Chiari malformation Type I and syrinx in children undergoing magnetic resonance imaging. J Neurosurg Pediatr. 2011;8:205-213.  https://doi.org/10.3171/2011.5.PEDS1121
  20. Urbizu A, Martin BA, Moncho D, et al. Machine learning applied to neuroimaging for diagnosis of adult classic Chiari malformation: role of the basion as a key morphometric indicator. J Neurosurg. 2018;129:779-791.  https://doi.org/10.3171/2017.3.JNS162479
  21. Botelho RV, Heringer LC, Botelho PB, et al. Posterior Fossa Dimensions of Chiari Malformation Patients Compared with Normal Subjects: Systematic Review and Meta-Analysis. World Neurosurg. 2020;138:521-529.  https://doi.org/10.1016/j.wneu.2020.02.182
  22. Heffez DS, Broderick J, Connor M, et al. Is there a relationship between the extent of tonsillar ectopia and the severity of the clinical Chiari syndrome? Acta Neurochir (Wien). 2020;162:1531-1538. https://doi.org/10.1007/s00701-019-04171-1
  23. Haller G, Sadler B, Kuensting T, et al. Obex position is associated with syringomyelia and use of posterior fossa decompression among patients with Chiari I malformation. J Neurosurg Pediatr. 2020;10:1-8.  https://doi.org/10.3171/2020.2.PEDS19486
  24. Headache Classification Committee of the International Headache Society (IHS) The International Classification of Headache Disorders, 3rd edition. Cephalalgia. 2018;38:110.  https://doi.org/10.1177/0333102417738202
  25. Tubbs RS, Elton S, Grabb P, et al. Analysis of the posterior fossa in children with the Chiari 0 malformation. Neurosurgery. 2001;48:1050-1054. https://doi.org/10.1097/00006123-200105000-00016
  26. Chern JJ, Gordon AJ, Mortazavi MM, et al. Pediatric Chiari malformation Type 0: a 12-year institutional experience. J Neurosurg Pediatr. 2011;8:1-5.  https://doi.org/10.3171/2011.4.PEDS10528
  27. Azahraa HF, Qaisi I, Joudeh N, et al. The newer classifications of the chiari malformations with clarifications: An anatomical review. Clin Anat. 2018;31:314-322.  https://doi.org/10.1002/ca.23051
  28. Heffez DS, Golchini R, Ghorai J, Cohen B. Operative findings and surgical outcomes in patients undergoing Chiari 1 malformation decompression: relationship to the extent of tonsillar ectopia. Acta Neurochir (Wien). 2020;162:1539-1547. https://doi.org/10.1007/s00701-019-04172-0
  29. Thakar S, Kurudi SA, Aryan S, et al. Does the mesodermal derangement in Chiari Type I malformation extend to the cervical spine? Evidence from an analytical morphometric study on cervical paraspinal muscles. J Neurosurg Spine. 2017;27:421-427.  https://doi.org/10.3171/2016.12.SPINE16914
  30. Houston JR, Hughes ML, Bennett IJ, et al. Evidence of Neural Microstructure Abnormalities in Type I Chiari Malformation: Associations Among Fiber Tract Integrity, Pain, and Cognitive Dysfunction. Pain Med. 2020;21:2323-2335. https://doi.org/10.1093/pm/pnaa094
  31. Musolf AM, Ho WSC, Long KA, 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;10:1599-1610. https://doi.org/10.1038/s41431-019-0457-7
  32. Heiss JD, Argersinger DP. Epidemiology of Chiari I Malformation. In: Tubbs R., Turgut M., Oakes W. (eds). The Chiari Malformations. 2020;263-274. Springer, Cham. https://doi.org/10.1007/978-3-030-44862-2_21
  33. Passias PG, Pyne A, Horn SR, et al. Developments in the treatment of Chiari type 1 malformations over the past decade. J Spine Surg. 2018;4:45-54.  https://doi.org/10.21037/jss.2018.03.14
  34. Wilkinson DA, Johnson K, Garton HJ, et al. Trends in surgical treatment of Chiari malformation Type I in the United States. J Neurosurg Pediatr. 2017;19:208-216.  https://doi.org/10.3171/2016.8.PEDS16273
  35. Kantimirova EA, Shnaider NA, Petrova MM, et al. The incidence of Arnold—Chiari malformation in neurological practice. Nevrologiya, Neiropsikhiatriya, Psikhosomatika. Neurology, Neuropsychiatry, Psychosomatics. 2015;7:18-22. (In Russ.). https://doi.org/10.14412/2074-2711-2015-4-18-22
  36. Faizutdinova AT, Bogdanov EI, Shakurov AR. Outpatients service for Chiari malformation. Neurology of Siberia 2017;2:88-92. (In Russ.).
  37. Bogdanov E. Epidemiology. In: Flint G., Rusbridge C. (eds). Syringomyelia. 2014;11-24. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-13706-8_2
  38. Milhorat TH, Bolognese PA, Nishikawa M, et al. Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and chiari malformation type I in patients with hereditary disorders of connective tissue. J Neurosurg Spine. 2007;7:601-609.  https://doi.org/10.3171/SPI-07/12/601
  39. Milhorat TH, Bolognese PA, Nishikawa M, et al. Association of Chiari malformation type I and tethered cord syndrome: preliminary results of sectioning filum terminale. Surg Neurol. 2009;72:20-35.  https://doi.org/10.1016/j.surneu.2009.03.008
  40. Whitney N, Sun H, Pollock JM, Ross DA. The human foramen magnum — normal anatomy of the cisterna magna in adults. Neuroradiology. 2013;55:1333-1339. https://doi.org/10.1007/s00234-013-1269-z
  41. Kumar A, Agrawal M, Prakash S, et al. Acute Foramen Magnum Syndrome Following Single Diagnostic Lumbar Puncture: Consequence of a Small Posterior Fossa? World Neurosurg. 2016;91:677.e1-7.  https://doi.org/10.1016/j.wneu.2016.04.111
  42. Neufeld EA, Menacho ST, Shah LM. Craniocervical Junction and Posterior Fossa Dimensions can Affect Need for Decompressive Craniectomy in Posterior Cranial Fossa Hemorrhage. World Neurosurg. 2019;127:570-577.  https://doi.org/10.1016/j.wneu.2019.03.208
  43. Mendelevich EG, Bogdanov EI, Chuvashaev IR. Family cases of syringomyelia and Arnold—Chiari anomalies. Zh Nevrol Psikhiatr im. S.S. Korsakova. 2000;100(12):93-95. (In Russ.).
  44. Boyles AL, Enterline DS, Hammock PH, et al. Phenotypic definition of Chiari type I malformation coupled with high-density SNP genome screen shows significant evidence for linkage to regions on chromosomes 9 and 15. Am J Med Genet A. 2006;140:2776-2785. https://doi.org/10.1002/ajmg.a.31546
  45. Markunas CA, Ashley-Koch AE, Gregory SG. Genetics of the Chiari I and II Malformations. In: Tubbs R., Turgut M., Oakes W. (eds). The Chiari Malformations. 2020;289-297. Springer, Cham. https://doi.org/10.1007/978-3-030-44862-2_23
  46. Bogdanov EI. Syringomyelia. Nevrol. Zhurnal. 2005;5:4-11. (In Russ.).
  47. Oldfield EH. Pathogenesis of Chiari I — Pathophysiology of Syringomyelia: Implications for Therapy: A Summary of 3 Decades of Clinical Research. Neurosurgery. 2017;64 (CN suppl 1):66-77.  https://doi.org/10.1093/neuros/nyx377
  48. Klekamp J. How Should Syringomyelia be Defined and Diagnosed? World Neurosurg. 2018;111:729-745.  https://doi.org/10.1016/j.wneu.2017.12.156
  49. Petit-Lacour MC, Lasjaunias P, Iffenecker C, et al. Visibility of the central canal on MRI. Neuroradiology. 2000;42:756-761.  https://doi.org/10.1007/s002340000373
  50. Bogdanov EI, Mendelevich EG. Syrinx size and duration of symptoms predict the pace of progressive myelopathy: retrospective analysis of 103 unoperated cases with craniocervical junction malformations and syringomyelia. Clin Neurol Neurosurg. 2002;104:90-97.  https://doi.org/10.1016/s0303-8467(01)00189-5
  51. Ciaramitaro P, Garbossa D, Peretta P, et al. Interregional Chiari and Syringomyelia Consortium; on behalf of the Interregional Chiari and Syringomyelia Consortium. Syringomyelia and Chiari Syndrome Registry: advances in epidemiology, clinical phenotypes and natural history based on a North Western Italy cohort. Ann Ist Super Sanita. 2020;56:48-58.  https://doi.org/10.4415/ANN_20_01_08
  52. 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;16:134-138.  https://doi.org/10.1111/j.1745-7599.2004.tb00384.x
  53. Novegno F. Clinical diagnosis-part II: what is attributed to Chiari I. Childs Nerv Syst. 2019;35:1681-1693. https://doi.org/10.1007/s00381-019-04192-2
  54. McClugage SG, Oakes WJ. The Chiari I malformation. J Neurosurg Pediatr. 2019;24:217-226.  https://doi.org/10.3171/2019.5.PEDS18382
  55. Fischbein R, Saling JR, Marty P, et al. Patient-reported Chiari malformation type I symptoms and diagnostic experiences: a report from the national Conquer Chiari Patient Registry database. Neurol Sci. 2015;36:1617-1624. https://doi.org/10.1007/s10072-015-2219-9
  56. Zabbarova AT, Bogdanov EI. Headache by a «overcrowded» posterior cranial fossa: a clinical MRI morphometric study. Nevrol. Zhurnal. 2010;6:21-25. (In Russ.).
  57. Bezuidenhout AF, Chang YM, Heilman CB, Bhadelia RA. Headache in Chiari malformation. Neuroimaging Clin N Am. 2019;29:243-253.  https://doi.org/10.1016/j.nic.2019.01.005171
  58. Saindane AM, Bruce BB, Desai NK, et al. Transverse sinus stenosis in adult patients with Chiari malformation type I. AJR Am J Roentgenol. 2014;203:890-896.  https://doi.org/10.2214/AJR.14.12528w
  59. Ellenbogen RG, Bauer DF. Association Between Fibromyalgia, Chronic Fatigue, and the Chiari I Malformation. In: Tubbs R., Turgut M., Oakes W. (eds). The Chiari Malformations. 2020;421-426. Springer, Cham. https://doi.org/10.1007/978-3-030-44862-2_35
  60. Steinberg SN, Greenfield JP, Perrine K. Neuroanatomic Correlates for the Neuropsychological Manifestations of Chiari Malformation Type I. World Neurosurg. 2020;136:462-469.  https://doi.org/10.1016/j.wneu.2020.01.149184
  61. Urbizu A, Ferré A, Poca MA, et al. Cephalometric oropharynx and oral cavity analysis in Chiari malformation Type I: a retrospective case-control study. J Neurosurg. 2017;126:626-633.  https://doi.org/10.3171/2016.1.JNS151590
  62. Alford EN, Zimmerman K, Rocque BG. Benign Chiari I Malformation. In: Tubbs R., Turgut M., Oakes W. (eds). The Chiari Malformations. 2020;381-385. Springer, Cham. https://doi.org/10.1007/978-3-030-44862-2_30
  63. Bogdanov EI, Ibatullin MM, Mendelevich EG. Spontaneous drainage in syringomyelia: magnetic resonance imaging findings. Neuroradiology. 2000;42:676-678.  https://doi.org/10.1007/s002340000358
  64. Kyoshima K, Bogdanov EI. Spontaneous resolution of syringomyelia: report of two cases and review of the literature. Neurosurgery. 2003;53:762-768.  https://doi.org/10.1227/01.neu.0000079629.05048.a2
  65. Bogdanov EI, Heiss JD, Mendelevich EG. The post-syrinx syndrome: stable central myelopathy and collapsed or absent syrinx. J Neurol. 2006;253:707-713.  https://doi.org/10.1007/s00415-006-0091-5
  66. Bolognese PA, Brodbelt A, Bloom AB, Kula RW. Chiari I Malformation: Opinions on Diagnostic Trends and Controversies from a Panel of 63 International Experts. World Neurosurg. 2019;130:9-16.  https://doi.org/10.1016/j.wneu.2019.05.098
  67. Perrini P, Anania Y, Cagnazzo F, et al. Radiological outcome after surgical treatment of syringomyelia-Chiari I complex in adults: a systematic review and meta-analysis. Neurosurg Rev. 2020 Jan 17.11(2):45-59.  https://doi.org/10.1007/s10143-020-01239-w
  68. Zuev AA, Pedyash NV, Epifanov DS, Kostenko GV. Results of surgical treatment of syringomyelia associated with Chiari 1 malformation. An analysis of 125 cases. Zh Vopr Neirokhir im. N.N. Burdenko. 2016;80:27-34. (In Russ.). https://doi.org/10.17116/neiro201680127-34
  69. Milano JB, Barcelos ACES, Onishi FJ, et al. The effect of filum terminale sectioning for Chiari 1 malformation treatment: systematic review. Neurol Sci. 2020;41:249-256.  https://doi.org/10.1007/s10072-019-04056-2

Email Confirmation

An email was sent to test@gmail.com with a confirmation link. Follow the link from the letter to complete the registration on the site.

Email Confirmation

We use cооkies to improve the performance of the site. By staying on our site, you agree to the terms of use of cооkies. To view our Privacy and Cookie Policy, please. click here.