Keywords
Chiari malformation type 1
Coexistence
Radiological markers
Submitted : 2024-01-23
Accepted : 2024-02-07
Published : 2024-02-22
Abstract
Objective: To identify different radiological markers for the diagnosis of the coexistence of pseudotumor cerebri and Chiari malformation type 1. Method: Patients who were clinically suspected to have Chiari malformation type 1 and were referred to the Department of Radiology between 2007 and 2020, and whose diagnosis was radiologically confirmed through magnetic resonance imaging (MRI), were retrospectively evaluated. A total of 49 Chiari malformation type 1 patients with both cervical and cerebral examinations and 49 control subjects of the same age and gender without a diagnosis of Chiari malformation type 1 were included in the study. In Chiari malformation type 1 diagnosed patients, the presence of pseudotumor cerebri, the presence of syringomyelia in cervical spinal MRI images, and the distance of cerebellar tonsils and obex according to McRae line were evaluated in millimeters. Result: In Chiari malformation type 1 clinically and radiologically diagnosed cases, the cerebellar tonsils and obex were located lower in patients with a radiological diagnosis of pseudotumor cerebri compared to those without, and the rate of accompanying syringomyelia appeared to be higher. However, no statistically significant difference was observed between the two groups. Conclusion: The coexistence of pseudotumor cerebri and Chiari malformation type 1 is more common than previously estimated. Different treatment protocols in the coexistence of pseudotumor cerebri and Chiari malformation type 1 emphasize the importance of making this diagnosis. Further radiological imaging studies are needed to identify different radiological markers for the diagnosis of the coexistence of pseudotumor cerebri and Chiari malformation type 1.
References
Elster AD, Chen MY, 1992, Chiari I Malformations: Clinical and Radiologic Reappraisal. Radiology, 183(2): 347–352. https://doi.org/10.1148/radiology.183.2.1561334
Aboulezz AO, Sartor K, Geyer CA, et al., 1985, Position of Cerebellar Tonsils in the Normal Population and in Patients with Chiari Malformation: A Quantitative Approach with MR Imaging. J Comput Assist Tomogr, 9(6):1033–1036. https://doi.org/10.1097/00004728-198511000-00005
Loukas M, Noordeh N, Shoja MM, et al., 2008, Hans Chiari (1851–1916). Childs Nerv Syst, 24(3): 407–409. https://doi.org/10.1007/s00381-007-0535-y
Fernández AA, Guerrero AI, Martínez MI, et al., 2009, Malformations of the Craniocervical Junction (Chiari Type I and Syringomyelia: Classification, Diagnosis and Treatment). BMC Musculoskelet Disord, 10 Suppl 1 (Suppl 1): S1. https://doi.org/10.1186/1471-2474-10-S1-S1
Outfield EH, Muraszko K, Shawker TH, et al., 1994, Pathophysiology of Syringomyelia Associated with Chiari I Malformation of the Cerebellar Tonsils. Implications for Diagnosis and Treatment. J Neurosurg, 80(1): 3–15. https://doi.org/10.3171/jns.1994.80.1.0003
Erdogan E, Cansever T, Secer HI, et al., 2010, The Evaluation of Surgical Treatment Options in the Chiari Malformation Type I. Turk Neurosurg, 20(3): 303–313. https://doi.org/10.5137/1019-5149.JTN.2648-09.2
Milhorat TH, Chou MW, Trinidad EM, et al., 1999, Chiari I Malformation Redefined: Clinical and Radiographic Findings for 364 Symptomatic Patients. Neurosurgery, 44(5): 1105–1017. https://doi.org/10.1097/00006123-199905000-00042
Meadows J, Kraut M, Guarnieri M, et al., 2000, Asymptomatic Chiari Type I Malformations Identified on Magnetic Resonance Imaging. J Neurosurg, 92(6): 920–926. https://doi.org/10.3171/jns.2000.92.6.0920
Speer MC, George TM, Enterline DS, et al., 2000, A Genetic Hypothesis for Chiari I Malformation with or without Syringomyelia. Neurosurg Focus, 8(3): E12. https://doi.org/10.3171/foc.2000.8.3.12
Milhorat TH, Capocelli AL Jr, Anzil AP, et al., 1995, Pathological Basis of Spinal Cord Cavitation in Syringomyelia: Analysis of 105 Autopsy Cases. J Neurosurg, 82(5): 802–812.https://doi.org/10.3171/jns.1995.82.5.0802
Milhorat TH, Johnson RW, Milhorat RH, et al., 1995, Clinicopathological Correlations in Syringomyelia Using Axial Magnetic Resonance Imaging. Neurosurgery, 37(2): 206–213. https://doi.org/10.1227/00006123-199508000-00003
Koyanagi I, Houkin K, 2010, Pathogenesis of Syringomyelia Associated with Chiari Type 1 Malformation: Review of Evidences and Proposal of a New Hypothesis. Neurosurg Rev, 33(3): 271–284; discussion 284-5. https://doi.org/10.1007/s10143-010-0266-5
Tubbs RS, Beckman J, Naftel RP, et al., 2011, Institutional Experience with 500 Cases of Surgically Treated Pediatric Chiari Malformation Type 1. J Neurosurg Pediatr, 7(3): 248–256. https://doi.org/10.3171/2010.12.PEDS10379
Kelly MP, Guillaume TJ, Lenke LG, 2015, Spinal Deformity Associated with Chiari Malformation. Neurosurg Clinics North Am, 26(4): 579–585. https://doi.org/10.1016/j.nec.2015.06.005
Smith JL, 1985, Whence Pseudotumor Cerebri? J Clin Neuroophthalmol, 5(1): 55–56.
Miller NR, Newman NJ, 1998, Walsh & Hoyt’s Clinical Neuro-Ophthalmology, 1st Edition, Lippincott Williams & Wilkins, Philadelphia.
Ball AK, Clarke CE, 2006, Idiopathic Intracranial Hypertension. Lancet Neurol, 5(5): 433–442. https://doi.org/10.1016/S1474-4422(06)70442-2
Durcan FJ, Corbett JJ, Wall M, 1988, The Incidence of Pseudotumor Cerebri. Population Studies in Iowa and Louisiana. Arch Neurol, 45(8): 875–877. https://doi.org/10.1001/archneur.1988.00520320065016
Radhakrishnan K, Thacker AK, Bohlaga NH, et al., 1993, Epidemiology of Idiopathic Intracranial Hypertension: A Prospective and Case-Control Study. J Neurol Sci, 116(1): 18–28. https://doi.org/10.1016/0022-510x(93)90084-c
Suzuki H, Takanashi J, Kobayashi K, et al., 2001, MR Imaging of Idiopathic Intracranial Hypertension. AJNR Am J Neuroradiol, 22(1): 196–199.
Gibby WA, Cohen MS, Goldberg HI, et al., 1993, Pseudotumor Cerebri: CT Findings and Correlation with Vision Loss. AJR Am J Roentgenol, 160(1): 143–146. https://doi.org/10.2214/ajr.160.1.8416612
Karagöz F, Izgi N, Kapíjcíjo?lu Sencer S, 2002, Morphometric Measurements of the Cranium in Patients with Chiari Type I Malformation and Comparison with the Normal Population. Acta Neurochir (Wien), 144(2): 165–171; discussion 171. https://doi.org/10.1007/s007010200020
Aiken AH, Hoots JA, Saindane AM, et al., 2012, Incidence of Cerebellar Tonsillar Ectopia in Idiopathic Intracranial Hypertension: A Mimic of the Chiari I Malformation. AJNR Am J Neuroradiol, 33(10): 1901–1906. https://doi.org/10.3174/ajnr.A3068
Johnston I, Hawke S, Halmagyi M, et al., 1991, The Pseudotumor Syndrome. Disorders of Cerebrospinal Fluid Circulation Causing Intracranial Hypertension Without Ventriculomegaly. Arch Neurol, 48(7): 740–747. https://doi.org/10.1001/archneur.1991.00530190088020
Banik R, Lin D, Miller NR, 2006, Prevalence of Chiari I Malformation and Cerebellar Ectopia in Patients with Pseudotumor Cerebri. J Neurol Sci, 247(1): 71–75. https://doi.org/10.1016/j.jns.2006.03.016
Fagan LH, Ferguson S, Yassari R, et al., 2006, The Chiari Pseudotumor Cerebri Syndrome: Symptom Recurrence After Decompressive Surgery for Chiari Malformation Type I. Pediatr Neurosurg, 42(1): 14–19. https://doi.org/10.1159/000089504
Alkoç OA, Songur A, Eser O, et al., 2015, Stereological and Morphometric Analysis of MRI Chiari Malformation Type-1. J Korean Neurosurg Soc, 58(5): 454–461. https://doi.org/10.3340/jkns.2015.58.5.454
Huang PP, Constantini S, 1994, “Acquired” Chiari I Malformation: Case Report. J Neurosurg, 80(6): 1099–1102. https://doi.org/10.3171/jns.1994.80.6.1099
Isik N, Kalelioglu M, Isik N, et al., 1999, The Role of Neurophysiological Findings on the Surgical Treatment Options of Chiari Malformation Type I. Turk Neurosurg, 9: 35–44.
Bogdanov EI, Heiss JD, Mendelevich EG, et al., 2015, Clinical and Neuroimaging Features of “Idiopathic” Syringomyelia. Neurology, 62(5): 791–794. https://doi.org/10.1212/01.wnl.0000113746.47997.ce