{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T08:01:50Z","timestamp":1789632110803,"version":"build-2803163510"},"reference-count":164,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T00:00:00Z","timestamp":1789603200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"},{"start":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T00:00:00Z","timestamp":1789603200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Nat Rev Dis Primers"],"DOI":"10.1038\/s41572-026-00739-1","type":"journal-article","created":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T07:02:27Z","timestamp":1789628547000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Chiari I malformation"],"prefix":"10.1038","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3182-2966","authenticated-orcid":false,"given":"Andrew T.","family":"Hale","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Douglas L.","family":"Brockmeyer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William C.","family":"Davalan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A. Graham","family":"Fieggen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerald A.","family":"Grant","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric M.","family":"Jackson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6419-7955","authenticated-orcid":false,"given":"Adam J.","family":"Kundishora","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David D.","family":"Limbrick","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cormac O.","family":"Maher","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brandon G.","family":"Rocque","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer M.","family":"Strahle","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominic M.","family":"Thompson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexander M.","family":"Tucker","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5922-7547","authenticated-orcid":false,"given":"Kristopher T.","family":"Kahle","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,9,17]]},"reference":[{"key":"739_CR1","doi-asserted-by":"publisher","first-page":"1172","DOI":"10.1055\/s-0029-1206803","volume":"17","author":"H Chiari","year":"1891","unstructured":"Chiari, H. Ueber Ver\u00e4nderungen des Kleinhirns infolge von Hydrocephalie des Grosshirns. Dtsch. Med. Wochenschr. 17, 1172\u20131175 (1891). The original description of CM1 established the anatomical framework based on cerebellar tonsillar herniation.","journal-title":"Dtsch. Med. Wochenschr."},{"key":"739_CR2","doi-asserted-by":"publisher","first-page":"501","DOI":"10.1016\/j.nec.2015.06.006","volume":"26","author":"EN Kahn","year":"2015","unstructured":"Kahn, E. N., Muraszko, K. M. & Maher, C. O. Prevalence of Chiari I malformation and syringomyelia. Neurosurg. Clin. N. Am. 26, 501\u2013507 (2015).","journal-title":"Neurosurg. Clin. N. Am."},{"key":"739_CR3","doi-asserted-by":"publisher","first-page":"9","DOI":"10.1016\/j.nec.2022.08.001","volume":"34","author":"KG Holste","year":"2023","unstructured":"Holste, K. G., Muraszko, K. M. & Maher, C. O. Epidemiology of Chiari I malformation and syringomyelia. Neurosurg. Clin. N. Am. 34, 9\u201315 https:\/\/doi.org\/10.1016\/j.nec.2022.08.001 (2023).","journal-title":"Neurosurg. Clin. N. Am."},{"key":"739_CR4","doi-asserted-by":"publisher","first-page":"2191","DOI":"10.1056\/NEJMra2308055","volume":"390","author":"RM Friedlander","year":"2024","unstructured":"Friedlander, R. M. Congenital and acquired Chiari syndrome. N. Engl. J. Med. 390, 2191\u20132198 https:\/\/doi.org\/10.1056\/NEJMra2308055 (2024). Recent review highlighting the key distinctions between developmental and acquired CM1 phenotypes.","journal-title":"N. Engl. J. Med."},{"key":"739_CR5","doi-asserted-by":"publisher","first-page":"1205","DOI":"10.3171\/2024.8.Jns241295","volume":"142","author":"DL Brockmeyer","year":"2025","unstructured":"Brockmeyer, D. L., Limbrick, D. D., Maher, C. O. & Grant, G. A. Primary rhombencephalopathy: introduction of a new paradigm for the evaluation and management of craniocervical hindbrain pathology. J. Neurosurg. 142, 1205\u20131212 https:\/\/doi.org\/10.3171\/2024.8.Jns241295 (2025). Introduces the \u2018primary rhomboencephalopathy\u2019 paradigm that conceptually anchors the Primer\u2019s reframing of CM1 as coupled developmental systems disorder spanning the cerebellum, posterior fossa and CCJ.","journal-title":"J. Neurosurg."},{"key":"739_CR6","doi-asserted-by":"publisher","first-page":"449","DOI":"10.1016\/j.pediatrneurol.2009.01.003","volume":"40","author":"LA Aitken","year":"2009","unstructured":"Aitken, L. A. et al. Chiari type I malformation in a pediatric population. Pediatr. Neurol. 40, 449\u2013454 https:\/\/doi.org\/10.1016\/j.pediatrneurol.2009.01.003 (2009).","journal-title":"Pediatr. Neurol."},{"key":"739_CR7","doi-asserted-by":"publisher","first-page":"1818","DOI":"10.3174\/ajnr.A6281","volume":"40","author":"V Dangouloff-Ros","year":"2019","unstructured":"Dangouloff-Ros, V. et al. Incidental brain MRI findings in children: a systematic review and meta-analysis. Am. J. Neuroradiol. 40, 1818\u20131823 https:\/\/doi.org\/10.3174\/ajnr.A6281 (2019).","journal-title":"Am. J. Neuroradiol."},{"key":"739_CR8","doi-asserted-by":"publisher","first-page":"920","DOI":"10.3171\/jns.2000.92.6.0920","volume":"92","author":"J Meadows","year":"2000","unstructured":"Meadows, J., Kraut, M., Guarnieri, M., Haroun, R. I. & Carson, B. S. Asymptomatic Chiari type\u00a0I malformations identified on magnetic resonance imaging. J. Neurosurg. 92, 920\u2013926 https:\/\/doi.org\/10.3171\/jns.2000.92.6.0920 (2000).","journal-title":"J. Neurosurg."},{"key":"739_CR9","doi-asserted-by":"publisher","first-page":"b3016","DOI":"10.1136\/bmj.b3016","volume":"339","author":"Z Morris","year":"2009","unstructured":"Morris, Z. et al. Incidental findings on brain magnetic resonance imaging: systematic review and meta-analysis. BMJ 339, b3016 https:\/\/doi.org\/10.1136\/bmj.b3016 (2009).","journal-title":"BMJ"},{"key":"739_CR10","doi-asserted-by":"publisher","first-page":"205","DOI":"10.3171\/2011.5.Peds1121","volume":"8","author":"J Strahle","year":"2011","unstructured":"Strahle, J. et al. Chiari malformation type I and syrinx in children undergoing magnetic resonance imaging. J. Neurosurg. Pediatr. 8, 205\u2013213 https:\/\/doi.org\/10.3171\/2011.5.Peds1121 (2011). Foundational paediatric population-based imaging study establishing modern prevalence estimates and co-occurrence of syrinx, hydrocephalus and scoliosis.","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR11","doi-asserted-by":"publisher","first-page":"1033","DOI":"10.1097\/00004728-198511000-00005","volume":"9","author":"AO Aboulezz","year":"1985","unstructured":"Aboulezz, A. O., Sartor, K., Geyer, C. A. & Gado, M. H. 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, 1033\u20131036 https:\/\/doi.org\/10.1097\/00004728-198511000-00005 (1985).","journal-title":"J. Comput. Assist. Tomogr."},{"key":"739_CR12","first-page":"795","volume":"7","author":"AJ Barkovich","year":"1986","unstructured":"Barkovich, A. J., Wippold, F. J., Sherman, J. L. & Citrin, C. M. Significance of cerebellar tonsillar position on MR. Am. J. Neuroradiol. 7, 795\u2013799 (1986).","journal-title":"Am. J. Neuroradiol."},{"key":"739_CR13","doi-asserted-by":"publisher","first-page":"812","DOI":"10.3171\/2013.5.JNS121825","volume":"119","author":"BW Smith","year":"2013","unstructured":"Smith, B. W. et al. Distribution of cerebellar tonsil position: implications for understanding Chiari malformation. J. Neurosurg. 119, 812\u2013819 https:\/\/doi.org\/10.3171\/2013.5.JNS121825 (2013). Population study describing normal cerebellar tonsillar position across the age spectrum, demonstrating that \u2018normal\u2019 position changes with age, highlighting the arbitrariness of the 3 \u20135 mm radiological threshold cut-off.","journal-title":"J. Neurosurg."},{"key":"739_CR14","doi-asserted-by":"publisher","first-page":"431","DOI":"10.1016\/j.wneu.2016.10.026","volume":"97","author":"MO Krucoff","year":"2017","unstructured":"Krucoff, M. O. et al. Racial, socioeconomic, and gender disparities in the presentation, treatment, and outcomes of adult Chiari I malformations. World Neurosurg. 97, 431\u2013437 https:\/\/doi.org\/10.1016\/j.wneu.2016.10.026 (2017).","journal-title":"World Neurosurg."},{"key":"739_CR15","doi-asserted-by":"publisher","first-page":"208","DOI":"10.3171\/2016.8.PEDS16273","volume":"19","author":"DA Wilkinson","year":"2017","unstructured":"Wilkinson, D. A., Johnson, K., Garton, H. J., Muraszko, K. M. & Maher, C. O. Trends in surgical treatment of Chiari malformation type I in the United States. J. Neurosurg. Pediatr. 19, 208\u2013216 https:\/\/doi.org\/10.3171\/2016.8.Peds16273 (2017).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR16","doi-asserted-by":"publisher","first-page":"1090","DOI":"10.1093\/neuros\/nyy175","volume":"84","author":"EI Bogdanov","year":"2019","unstructured":"Bogdanov, E. I., Faizutdinova, A. T., Mendelevich, E. G., Sozinov, A. S. & Heiss, J. D. Epidemiology of symptomatic Chiari malformation in Tatarstan: regional and ethnic differences in prevalence. Neurosurgery 84, 1090\u20131097 https:\/\/doi.org\/10.1093\/neuros\/nyy175 (2019).","journal-title":"Neurosurgery"},{"key":"739_CR17","doi-asserted-by":"publisher","first-page":"989","DOI":"10.1136\/jnnp.2005.081240","volume":"77","author":"KL Brickell","year":"2006","unstructured":"Brickell, K. L. et al. Ethnic differences in syringomyelia in New Zealand. J. Neurol. Neurosurg. Psychiatry 77, 989\u2013991 https:\/\/doi.org\/10.1136\/jnnp.2005.081240 (2006).","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"739_CR18","doi-asserted-by":"publisher","first-page":"48","DOI":"10.4415\/ann_20_01_08","volume":"56","author":"P Ciaramitaro","year":"2020","unstructured":"Ciaramitaro, P. et al. Syringomyelia and Chiari syndrome registry: advances in epidemiology, clinical phenotypes and natural history based on a North Western Italy cohort. Ann. Ist. Super. Sanit\u00e0 56, 48\u201358 https:\/\/doi.org\/10.4415\/ann_20_01_08 (2020).","journal-title":"Ann. Ist. Super. Sanit\u00e0"},{"key":"739_CR19","doi-asserted-by":"publisher","first-page":"147","DOI":"10.1016\/j.jns.2011.08.045","volume":"313","author":"K Sakushima","year":"2012","unstructured":"Sakushima, K. et al. Nationwide survey on the epidemiology of syringomyelia in Japan. J. Neurol. Sci. 313, 147\u2013152 https:\/\/doi.org\/10.1016\/j.jns.2011.08.045 (2012).","journal-title":"J. Neurol. Sci."},{"key":"739_CR20","doi-asserted-by":"publisher","first-page":"288","DOI":"10.3171\/2021.9.Peds2185","volume":"29","author":"SHA Akbari","year":"2022","unstructured":"Akbari, S. H. A. et al. Socioeconomic and demographic factors in the diagnosis and treatment of Chiari malformation type I and syringomyelia. J. Neurosurg. Pediatr. 29, 288\u2013297 https:\/\/doi.org\/10.3171\/2021.9.Peds2185 (2022).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR21","doi-asserted-by":"publisher","unstructured":"Leon, T. J. et al. Patients with \u201cbenign\u201d Chiari I malformations require surgical decompression at a low rate. J. Neurosurg. Pediatr. 23, 498\u2013506 https:\/\/doi.org\/10.3171\/2018.10.peds18407 (2019).","DOI":"10.3171\/2018.10.peds18407"},{"key":"739_CR22","doi-asserted-by":"publisher","first-page":"214","DOI":"10.3171\/2011.5.Peds1122","volume":"8","author":"J Strahle","year":"2011","unstructured":"Strahle, J. et al. Natural history of Chiari malformation type I following decision for conservative treatment. J. Neurosurg. Pediatr. 8, 214\u2013221 https:\/\/doi.org\/10.3171\/2011.5.Peds1122 (2011).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR23","doi-asserted-by":"publisher","first-page":"159","DOI":"10.3171\/2014.12.Peds14301","volume":"16","author":"WJ Whitson","year":"2015","unstructured":"Whitson, W. J., Lane, J. R., Bauer, D. F. & Durham, S. R. A prospective natural history study of nonoperatively managed Chiari I malformation: does follow-up MRI surveillance alter surgical decision making? J. Neurosurg. Pediatr. 16, 159\u2013166 https:\/\/doi.org\/10.3171\/2014.12.Peds14301 (2015).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR24","doi-asserted-by":"publisher","first-page":"1653","DOI":"10.1007\/s00381-011-1545-3","volume":"27","author":"C Di Rocco","year":"2011","unstructured":"Di Rocco, C., Frassanito, P., Massimi, L. & Peraio, S. Hydrocephalus and Chiari type I malformation. Child\u2019s Nerv. Syst. 27, 1653\u20131664 https:\/\/doi.org\/10.1007\/s00381-011-1545-3 (2011).","journal-title":"Child\u2019s Nerv. Syst."},{"key":"739_CR25","doi-asserted-by":"publisher","first-page":"1211","DOI":"10.3171\/jns\/2008\/108\/6\/1211","volume":"108","author":"C Hayhurst","year":"2008","unstructured":"Hayhurst, C., Osman-Farah, J., Das, K. & Mallucci, C. Initial management of hydrocephalus associated with Chiari malformation type I-syringomyelia complex via endoscopic third ventriculostomy: an outcome analysis. J. Neurosurg. 108, 1211\u20131214 https:\/\/doi.org\/10.3171\/jns\/2008\/108\/6\/1211 (2008).","journal-title":"J. Neurosurg."},{"key":"739_CR26","doi-asserted-by":"publisher","first-page":"1701","DOI":"10.1007\/s00381-019-04245-6","volume":"35","author":"L Massimi","year":"2019","unstructured":"Massimi, L. et al. Chiari type I and hydrocephalus. Child\u2019s Nerv. Syst. 35, 1701\u20131709 https:\/\/doi.org\/10.1007\/s00381-019-04245-6 (2019).","journal-title":"Child\u2019s Nerv. Syst."},{"key":"739_CR27","doi-asserted-by":"publisher","first-page":"767","DOI":"10.1097\/brs.0000000000005289","volume":"50","author":"F Costa","year":"2025","unstructured":"Costa, F. et al. Chiari malformation: diagnosis, classifications, natural history, and conservative management. World Federation of Neurosurgical Societies Spine Committee recommendations. Spine 50, 767\u2013778 https:\/\/doi.org\/10.1097\/brs.0000000000005289 (2025).","journal-title":"Spine"},{"key":"739_CR28","doi-asserted-by":"publisher","first-page":"115535","DOI":"10.1016\/j.expneurol.2025.115535","volume":"396","author":"W Davalan","year":"2026","unstructured":"Davalan, W. et al. Concurrent Chiari malformation type I and hydrocephalus: integrating mechanistic and pathophysiological insights toward a unified management paradigm. Exp. Neurol. 396, 115535 https:\/\/doi.org\/10.1016\/j.expneurol.2025.115535 (2026).","journal-title":"Exp. Neurol."},{"key":"739_CR29","doi-asserted-by":"publisher","first-page":"32","DOI":"10.1016\/j.pediatrneurol.2019.12.005","volume":"106","author":"B Sadler","year":"2020","unstructured":"Sadler, B. et al. Prevalence and impact of underlying diagnosis and comorbidities on Chiari 1 malformation. Pediatr. Neurol. 106, 32\u201337 https:\/\/doi.org\/10.1016\/j.pediatrneurol.2019.12.005 (2020).","journal-title":"Pediatr. Neurol."},{"key":"739_CR30","doi-asserted-by":"publisher","first-page":"3","DOI":"10.3171\/jns.1994.80.1.0003","volume":"80","author":"EH Oldfield","year":"1994","unstructured":"Oldfield, E. H., Muraszko, K., Shawker, T. H. & Patronas, N. J. Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils: implications for diagnosis and treatment. J. Neurosurg. 80, 3\u201315 (1994). Landmark mechanistic study that linked CSF outflow obstruction at the foramen magnum to syringomyelia, providing the pathophysiological rationale for PFD that defines current surgical practice.","journal-title":"J. Neurosurg."},{"key":"739_CR31","doi-asserted-by":"publisher","first-page":"525","DOI":"10.3171\/2015.10.Peds15369","volume":"17","author":"JK Greenberg","year":"2016","unstructured":"Greenberg, J. K. et al. Chiari malformation type I surgery in pediatric patients. Part 2: complications and the influence of comorbid disease in California, Florida, and New York. J. Neurosurg. Pediatr. 17, 525\u2013532 https:\/\/doi.org\/10.3171\/2015.10.Peds15369 (2016).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR32","doi-asserted-by":"publisher","first-page":"1005","DOI":"10.1097\/00006123-199905000-00042","volume":"44","author":"TH Milhorat","year":"1999","unstructured":"Milhorat, T. H. et al. Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery 44, 1005\u20131017 https:\/\/doi.org\/10.1097\/00006123-199905000-00042 (1999). The defining clinico-radiographic phenotyping patient series of its era, articulating the dissociation between tonsillar descent and clinical phenotype, motivating a revised nosology.","journal-title":"Neurosurgery"},{"key":"739_CR33","doi-asserted-by":"publisher","first-page":"173","DOI":"10.3171\/2025.6.Peds22192","volume":"37","author":"EM Nturibi","year":"2026","unstructured":"Nturibi, E. M. et al. A retrospective single-center series on the surgical management and postoperative outcomes of pediatric Chiari malformation type I. Part 2: symptomatic outcomes and revision surgery. J Neurosurg. Pediatr. 37, 173\u2013179 https:\/\/doi.org\/10.3171\/2025.6.Peds22192 (2026).","journal-title":"J Neurosurg. Pediatr."},{"key":"739_CR34","doi-asserted-by":"publisher","first-page":"165","DOI":"10.1016\/j.jocn.2018.06.045","volume":"58","author":"SR Horn","year":"2018","unstructured":"Horn, S. R. et al. Chiari malformation clusters describe differing presence of concurrent anomalies based on Chiari type. J. Clin. Neurosci. 58, 165\u2013171 https:\/\/doi.org\/10.1016\/j.jocn.2018.06.045 (2018).","journal-title":"J. Clin. Neurosci."},{"key":"739_CR35","doi-asserted-by":"publisher","first-page":"21","DOI":"10.3171\/2014.12.Peds14463","volume":"16","author":"J Strahle","year":"2015","unstructured":"Strahle, J. et al. Syrinx location and size according to etiology: identification of Chiari-associated syrinx. J. Neurosurg. Pediatr. 16, 21\u201329 https:\/\/doi.org\/10.3171\/2014.12.Peds14463 (2015).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR36","doi-asserted-by":"publisher","first-page":"236","DOI":"10.1007\/s00381-025-06885-3","volume":"41","author":"R Sun","year":"2025","unstructured":"Sun, R., Punyawai, P., Furtado, N., Afshari, F. T. & Gallo, P. Paediatric idiopathic syringomyelia - a follow-up of radiological and clinical outcomes into adulthood. Child\u2019s Nerv. Syst. 41, 236 https:\/\/doi.org\/10.1007\/s00381-025-06885-3 (2025).","journal-title":"Child\u2019s Nerv. Syst."},{"key":"739_CR37","unstructured":"Al-Zahr\u0101w\u012b, A. a.-Q. Chirurgia Codex Series nova 2641 (Akademische Druck- und Verlagsanstalt, 1979)."},{"key":"739_CR38","doi-asserted-by":"publisher","first-page":"520","DOI":"10.3171\/2019.5.Peds18527","volume":"24","author":"JM Strahle","year":"2019","unstructured":"Strahle, J. M. et al. Radiological and clinical predictors of scoliosis in patients with Chiari malformation type I and spinal cord syrinx from the Park-Reeves Syringomyelia Research Consortium. J. Neurosurg. Pediatr. 24, 520\u2013527 https:\/\/doi.org\/10.3171\/2019.5.Peds18527(2019).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR39","doi-asserted-by":"publisher","first-page":"607","DOI":"10.3171\/2014.11.Peds14135","volume":"15","author":"J Strahle","year":"2015","unstructured":"Strahle, J. et al. The association between Chiari malformation type I, spinal syrinx, and scoliosis. J. Neurosurg. Pediatr. 15, 607\u2013611 https:\/\/doi.org\/10.3171\/2014.11.Peds14135 (2015).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR40","doi-asserted-by":"publisher","first-page":"53","DOI":"10.3171\/2020.1.Peds18755","volume":"26","author":"JM Strahle","year":"2020","unstructured":"Strahle, J. M. et al. Radiological and clinical associations with scoliosis outcomes after posterior fossa decompression in patients with Chiari malformation and syrinx from the Park-Reeves Syringomyelia Research Consortium. J. Neurosurg. Pediatr. 26, 53\u201359 https:\/\/doi.org\/10.3171\/2020.1.Peds18755 (2020).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR41","doi-asserted-by":"publisher","first-page":"585","DOI":"10.3171\/2021.5.Peds20915","volume":"28","author":"S Chotai","year":"2021","unstructured":"Chotai, S. et al. Longitudinal scoliosis behavior in Chiari malformation with and without syringomyelia. J. Neurosurg. Pediatr. 28, 585\u2013591 https:\/\/doi.org\/10.3171\/2021.5.Peds20915 (2021).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR42","doi-asserted-by":"publisher","first-page":"167","DOI":"10.3171\/2020.12.Peds20552","volume":"28","author":"B Sadler","year":"2021","unstructured":"Sadler, B. et al. Extradural decompression versus duraplasty in Chiari malformation type I with syrinx: outcomes on scoliosis from the Park-Reeves Syringomyelia Research Consortium. J. Neurosurg. Pediatr. 28, 167\u2013175 https:\/\/doi.org\/10.3171\/2020.12.Peds20552 (2021).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR43","doi-asserted-by":"publisher","first-page":"2527","DOI":"10.1007\/s00381-024-06403-x","volume":"40","author":"EY Montgomery","year":"2024","unstructured":"Montgomery, E. Y. et al. Predictors of syrinx presentation and outcomes in pediatric Chiari malformation type I: a single institution experience of 218 consecutive syrinx patients. Child\u2019s Nerv. Syst. 40, 2527\u20132534 https:\/\/doi.org\/10.1007\/s00381-024-06403-x (2024).","journal-title":"Child\u2019s Nerv. Syst."},{"key":"739_CR44","doi-asserted-by":"publisher","first-page":"1653","DOI":"10.1007\/s00381-019-04296-9","volume":"35","author":"DNP Thompson","year":"2019","unstructured":"Thompson, D. N. P. Chiari I\u2014a \u2018not so\u2019 congenital malformation? Child\u2019s Nerv. Syst. 35, 1653\u20131664 https:\/\/doi.org\/10.1007\/s00381-019-04296-9 (2019).","journal-title":"Child\u2019s Nerv. Syst."},{"key":"739_CR45","doi-asserted-by":"publisher","first-page":"484","DOI":"10.1038\/35081558","volume":"2","author":"VY Wang","year":"2001","unstructured":"Wang, V. Y. & Zoghbi, H. Y. Genetic regulation of cerebellar development. Nat. Rev. Neurosci. 2, 484\u2013491 https:\/\/doi.org\/10.1038\/35081558 (2001).","journal-title":"Nat. Rev. Neurosci."},{"key":"739_CR46","doi-asserted-by":"publisher","first-page":"673","DOI":"10.1038\/nrn2188","volume":"8","author":"Z Huang","year":"2007","unstructured":"Huang, Z., Di Cristo, G. & Ango, F. Development of GABA innervation in the cerebral and cerebellar cortices. Nat. Rev. Neurosci. 8, 673\u2013686 (2007).","journal-title":"Nat. Rev. Neurosci."},{"key":"739_CR47","doi-asserted-by":"publisher","first-page":"3066","DOI":"10.1523\/JNEUROSCI.5203-05.2006","volume":"26","author":"AJ Fink","year":"2006","unstructured":"Fink, A. J. et al. Development of the deep cerebellar nuclei: transcription factors and cell migration from the rhombic lip. J. Neurosci. 26, 3066\u20133076 (2006).","journal-title":"J. Neurosci."},{"key":"739_CR48","doi-asserted-by":"publisher","first-page":"889","DOI":"10.1242\/dev.011569","volume":"135","author":"MA Basson","year":"2008","unstructured":"Basson, M. A. et al. Specific regions within the embryonic midbrain and cerebellum require different levels of FGF signaling during development. Development 135, 889\u2013898 https:\/\/doi.org\/10.1242\/dev.011569 (2008).","journal-title":"Development"},{"key":"739_CR49","doi-asserted-by":"publisher","first-page":"15","DOI":"10.1016\/0168-9525(96)81383-7","volume":"12","author":"AL Joyner","year":"1996","unstructured":"Joyner, A. L. Engrailed, Wnt and Pax genes regulate midbrain-hindbrain development. Trends Genet. 12, 15\u201320 https:\/\/doi.org\/10.1016\/0168-9525(96)81383-7 (1996).","journal-title":"Trends Genet."},{"key":"739_CR50","doi-asserted-by":"publisher","first-page":"154","DOI":"10.1111\/nyas.13094","volume":"1369","author":"T Moberget","year":"2016","unstructured":"Moberget, T. & Ivry, R. B. Cerebellar contributions to motor control and language comprehension: searching for common computational principles. Ann. N. Y. Acad. Sci. 1369, 154\u2013171 https:\/\/doi.org\/10.1111\/nyas.13094 (2016).","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"739_CR51","doi-asserted-by":"publisher","first-page":"63","DOI":"10.1016\/j.neuroimage.2009.08.016","volume":"49","author":"H Tiemeier","year":"2010","unstructured":"Tiemeier, H. et al. Cerebellum development during childhood and adolescence: a longitudinal morphometric MRI study. NeuroImage 49, 63\u201370 https:\/\/doi.org\/10.1016\/j.neuroimage.2009.08.016 (2010).","journal-title":"NeuroImage"},{"key":"739_CR52","doi-asserted-by":"publisher","first-page":"112281","DOI":"10.1016\/j.celrep.2023.112281","volume":"42","author":"Y Wang","year":"2023","unstructured":"Wang, Y. et al. Longitudinal development of the cerebellum in human infants during the first 800 days. Cell Rep. 42, 112281 https:\/\/doi.org\/10.1016\/j.celrep.2023.112281 (2023).","journal-title":"Cell Rep."},{"key":"739_CR53","doi-asserted-by":"publisher","first-page":"40","DOI":"10.3171\/jns.1997.86.1.0040","volume":"86","author":"M Nishikawa","year":"1997","unstructured":"Nishikawa, M., Sakamoto, H., Hakuba, A., Nakanishi, N. & Inoue, Y. Pathogenesis of Chiari malformation: a morphometric study of the posterior cranial fossa. J. Neurosurg. 86, 40\u201347 https:\/\/doi.org\/10.3171\/jns.1997.86.1.0040 (1997).","journal-title":"J. Neurosurg."},{"key":"739_CR54","doi-asserted-by":"publisher","first-page":"1046","DOI":"10.3171\/2009.2.Jns08284","volume":"111","author":"R Noudel","year":"2009","unstructured":"Noudel, R. et al. Incidence of basioccipital hypoplasia in Chiari malformation type I: comparative morphometric study of the posterior cranial fossa. Clinical article. J. Neurosurg. 111, 1046\u20131052 https:\/\/doi.org\/10.3171\/2009.2.Jns08284 (2009).","journal-title":"J. Neurosurg."},{"key":"739_CR55","doi-asserted-by":"publisher","first-page":"165","DOI":"10.1007\/s007010200020","volume":"144","author":"F Karag\u00f6z","year":"2002","unstructured":"Karag\u00f6z, F., \u0130zgi, N. & Kap\u0131jc\u0131jo\u011flu Sencer, S. Morphometric measurements of the cranium in patients with Chiari type I malformation and comparison with the normal population. Acta Neurochir. 144, 165\u2013171 https:\/\/doi.org\/10.1007\/s007010200020 (2002).","journal-title":"Acta Neurochir."},{"key":"739_CR56","doi-asserted-by":"publisher","first-page":"137","DOI":"10.1016\/0022-510x(90)90150-l","volume":"99","author":"A Vega","year":"1990","unstructured":"Vega, A., Quintana, F. & Berciano, J. Basichondrocranium anomalies in adult Chiari type I malformation: a morphometric study. J. Neurol. Sci. 99, 137\u2013145 https:\/\/doi.org\/10.1016\/0022-510x(90)90150-l (1990).","journal-title":"J. Neurol. Sci."},{"key":"739_CR57","doi-asserted-by":"publisher","unstructured":"Pang, D. & Thompson, D. N. in Pediatric Craniovertebral Junction Diseases (eds Rocco, C. & Akalan, N.)\u00a019\u2013109 https:\/\/doi.org\/10.1007\/978-3-319-01065-6_2 (Springer, 2014).","DOI":"10.1007\/978-3-319-01065-6_2"},{"key":"739_CR58","doi-asserted-by":"publisher","first-page":"2757","DOI":"10.1007\/s00381-023-06051-7","volume":"39","author":"F Bianchi","year":"2023","unstructured":"Bianchi, F., Montedoro, B., Frassanito, P., Massimi, L. & Tamburrini, G. Chiari I malformation: management evolution and technical innovation. Child\u2019s Nerv. Syst. 39, 2757\u20132769 https:\/\/doi.org\/10.1007\/s00381-023-06051-7 (2023).","journal-title":"Child\u2019s Nerv. Syst."},{"key":"739_CR59","doi-asserted-by":"publisher","first-page":"2776","DOI":"10.1002\/ajmg.a.31546","volume":"140","author":"AL Boyles","year":"2006","unstructured":"Boyles, A. L. 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 140, 2776\u20132785 https:\/\/doi.org\/10.1002\/ajmg.a.31546 (2006).","journal-title":"Am. J. Med. Genet. A"},{"key":"739_CR60","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1111\/ahg.12041","volume":"78","author":"CA Markunas","year":"2014","unstructured":"Markunas, C. A. et al. Genetic evaluation and application of posterior cranial fossa traits as endophenotypes for Chiari type I malformation. Ann. Hum. Genet. 78, 1\u201312 https:\/\/doi.org\/10.1111\/ahg.12041 (2014).","journal-title":"Ann. Hum. Genet."},{"key":"739_CR61","doi-asserted-by":"publisher","first-page":"1599","DOI":"10.1038\/s41431-019-0457-7","volume":"27","author":"AM Musolf","year":"2019","unstructured":"Musolf, A. M. 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. 27, 1599\u20131610 https:\/\/doi.org\/10.1038\/s41431-019-0457-7 (2019).","journal-title":"Eur. J. Hum. Genet."},{"key":"739_CR62","doi-asserted-by":"publisher","first-page":"431","DOI":"10.1007\/s00018-025-05897-1","volume":"82","author":"A Al-Sayyar","year":"2025","unstructured":"Al-Sayyar, A. et al. The meningeal-cerebellar axis: a new perspective on cerebellar development. Cell. Mol. Life Sci. 82, 431 https:\/\/doi.org\/10.1007\/s00018-025-05897-1 (2025).","journal-title":"Cell. Mol. Life Sci."},{"key":"739_CR63","doi-asserted-by":"publisher","first-page":"140","DOI":"10.1227\/01.neu.0000243293.46319.35","volume":"59","author":"MJ McGirt","year":"2006","unstructured":"McGirt, M. J., Nimjee, S. M., Fuchs, H. E. & George, T. M. Relationship of cine phase-contrast MRI to outcome after decompression for Chiari I malformation. Neurosurgery 59, 140\u2013146 https:\/\/doi.org\/10.1227\/01.neu.0000243293.46319.35 (2006).","journal-title":"Neurosurgery"},{"key":"739_CR64","doi-asserted-by":"publisher","first-page":"1746","DOI":"10.1212\/wnl.45.9.1746","volume":"45","author":"J Pujol","year":"1995","unstructured":"Pujol, J. et al. Motion of the cerebellar tonsils in Chiari type I malformation studied by cine phase-contrast MRI. Neurology 45, 1746\u20131753 https:\/\/doi.org\/10.1212\/wnl.45.9.1746 (1995).","journal-title":"Neurology"},{"key":"739_CR65","doi-asserted-by":"publisher","first-page":"81","DOI":"10.1016\/j.nec.2022.08.007","volume":"34","author":"JD Heiss","year":"2023","unstructured":"Heiss, J. D. Cerebrospinal fluid hydrodynamics in Chiari I malformation and syringomyelia: modeling pathophysiology. Neurosurg. Clin. N. Am. 34, 81\u201390 https:\/\/doi.org\/10.1016\/j.nec.2022.08.007 (2023).","journal-title":"Neurosurg. Clin. N. Am."},{"key":"739_CR66","doi-asserted-by":"publisher","first-page":"E12","DOI":"10.3171\/foc.2000.8.3.12","volume":"8","author":"MC Speer","year":"2000","unstructured":"Speer, M. C. et al. A genetic hypothesis for Chiari I malformation with or without syringomyelia. Neurosurg. Focus 8, E12 https:\/\/doi.org\/10.3171\/foc.2000.8.3.12 (2000).","journal-title":"Neurosurg. Focus"},{"key":"739_CR67","doi-asserted-by":"publisher","first-page":"109","DOI":"10.3171\/2025.1.Peds24187","volume":"36","author":"KY Mekbib","year":"2025","unstructured":"Mekbib, K. Y. et al. Clinical phenotypes among patients with familial forms of Chiari malformation type 1. J. Neurosurg. Pediatr. 36, 109\u2013118 https:\/\/doi.org\/10.3171\/2025.1.Peds24187 (2025).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR68","doi-asserted-by":"publisher","first-page":"E1","DOI":"10.3171\/2011.6.Focus11104","volume":"31","author":"BD Schanker","year":"2011","unstructured":"Schanker, B. D. et al. Familial Chiari malformation: case series. Neurosurg. Focus 31, E1 https:\/\/doi.org\/10.3171\/2011.6.Focus11104 (2011).","journal-title":"Neurosurg. Focus"},{"key":"739_CR69","doi-asserted-by":"publisher","first-page":"297","DOI":"10.1023\/a:1023948921381","volume":"12","author":"MC Speer","year":"2003","unstructured":"Speer, M. C. et al. Review article: Chiari type I malformation with or without syringomyelia: prevalence and genetics. J. Genet. Couns. 12, 297\u2013311 https:\/\/doi.org\/10.1023\/a:1023948921381 (2003).","journal-title":"J. Genet. Couns."},{"key":"739_CR70","doi-asserted-by":"publisher","first-page":"100","DOI":"10.1016\/j.ajhg.2020.12.001","volume":"108","author":"B Sadler","year":"2021","unstructured":"Sadler, B. et al. Rare and de novo coding variants in chromodomain genes in Chiari I malformation. Am. J. Hum. Genet. 108, 100\u2013114\u00a0https:\/\/doi.org\/10.1016\/j.ajhg.2020.12.001\u00a0(2021). A large-scale exome sequencing study to implicate chromatin remodelling genes in sporadic CM1.","journal-title":"Am. J. Hum. Genet."},{"key":"739_CR71","doi-asserted-by":"publisher","first-page":"266","DOI":"10.3171\/2022.10.Jns22956","volume":"139","author":"DL Brockmeyer","year":"2023","unstructured":"Brockmeyer, D. L. et al. A likely HOXC4 predisposition variant for Chiari malformations. J. Neurosurg. 139, 266\u2013274 https:\/\/doi.org\/10.3171\/2022.10.Jns22956 (2023).","journal-title":"J. Neurosurg."},{"key":"739_CR72","doi-asserted-by":"publisher","first-page":"952","DOI":"10.1038\/ejhg.2017.71","volume":"25","author":"E Merello","year":"2017","unstructured":"Merello, E. et al. Exome sequencing of two Italian pedigrees with non-isolated Chiari malformation type I reveals candidate genes for cranio-facial development. Eur. J. Hum. Genet. 25, 952\u2013959 https:\/\/doi.org\/10.1038\/ejhg.2017.71 (2017).","journal-title":"Eur. J. Hum. Genet."},{"key":"739_CR73","doi-asserted-by":"publisher","first-page":"625","DOI":"10.1007\/s00439-020-02231-6","volume":"140","author":"A Provenzano","year":"2021","unstructured":"Provenzano, A. et al. Chiari 1 malformation and exome sequencing in 51 trios: the emerging role of rare missense variants in chromatin-remodeling genes. Hum. Genet. 140, 625\u2013647 https:\/\/doi.org\/10.1007\/s00439-020-02231-6 (2021).","journal-title":"Hum. Genet."},{"key":"739_CR74","doi-asserted-by":"publisher","first-page":"e0251289","DOI":"10.1371\/journal.pone.0251289","volume":"16","author":"A Urbizu","year":"2021","unstructured":"Urbizu, A. et al. Rare functional genetic variants in COL7A1, COL6A5, COL1A2 and COL5A2 frequently occur in Chiari malformation type 1. PLoS ONE 16, e0251289 https:\/\/doi.org\/10.1371\/journal.pone.0251289 (2021).","journal-title":"PLoS ONE"},{"key":"739_CR75","doi-asserted-by":"publisher","first-page":"1754","DOI":"10.1038\/s41591-020-1090-2","volume":"26","author":"SC Jin","year":"2020","unstructured":"Jin, S. C. et al. Exome sequencing implicates genetic disruption of prenatal neuro-gliogenesis in sporadic congenital hydrocephalus. Nat. Med. 26, 1754\u20131765 https:\/\/doi.org\/10.1038\/s41591-020-1090-2(2020).","journal-title":"Nat. Med."},{"key":"739_CR76","doi-asserted-by":"publisher","first-page":"260","DOI":"10.1136\/jmedgenet-2016-104215","volume":"54","author":"KA Miller","year":"2017","unstructured":"Miller, K. A. et al. Diagnostic value of exome and whole genome sequencing in craniosynostosis. J. Med. Genet. 54, 260\u2013268 https:\/\/doi.org\/10.1136\/jmedgenet-2016-104215 (2017).","journal-title":"J. Med. Genet."},{"key":"739_CR77","doi-asserted-by":"publisher","first-page":"21","DOI":"10.1007\/s00439-022-02477-2","volume":"142","author":"AT Timberlake","year":"2023","unstructured":"Timberlake, A. T. et al. De novo mutations in the BMP signaling pathway in lambdoid craniosynostosis. Hum. Genet. 142, 21\u201332 https:\/\/doi.org\/10.1007\/s00439-022-02477-2 (2023).","journal-title":"Hum. Genet."},{"key":"739_CR78","doi-asserted-by":"publisher","first-page":"1163","DOI":"10.1038\/s41593-021-00872-y","volume":"24","author":"KA Aldinger","year":"2021","unstructured":"Aldinger, K. A. et al. Spatial and cell type transcriptional landscape of human cerebellar development. Nat. Neurosci. 24, 1163\u20131175 https:\/\/doi.org\/10.1038\/s41593-021-00872-y (2021).","journal-title":"Nat. Neurosci."},{"key":"739_CR79","doi-asserted-by":"publisher","first-page":"160","DOI":"10.1038\/s41593-020-00754-9","volume":"24","author":"CI De Zeeuw","year":"2021","unstructured":"De Zeeuw, C. I., Lisberger, S. G. & Raymond, J. L. Diversity and dynamism in the cerebellum. Nat. Neurosci. 24, 160\u2013167 https:\/\/doi.org\/10.1038\/s41593-020-00754-9 (2021).","journal-title":"Nat. Neurosci."},{"key":"739_CR80","doi-asserted-by":"publisher","first-page":"298","DOI":"10.1038\/s41583-019-0152-2","volume":"20","author":"A Sathyanesan","year":"2019","unstructured":"Sathyanesan, A. et al. Emerging connections between cerebellar development, behaviour and complex brain disorders. Nat. Rev. Neurosci. 20, 298\u2013313 https:\/\/doi.org\/10.1038\/s41583-019-0152-2 (2019).","journal-title":"Nat. Rev. Neurosci."},{"key":"739_CR81","doi-asserted-by":"publisher","first-page":"788","DOI":"10.1038\/s41586-023-06884-x","volume":"625","author":"M Sepp","year":"2024","unstructured":"Sepp, M. et al. Cellular development and evolution of the mammalian cerebellum. Nature 625, 788\u2013796 https:\/\/doi.org\/10.1038\/s41586-023-06884-x (2024).","journal-title":"Nature"},{"key":"739_CR82","doi-asserted-by":"publisher","first-page":"1025","DOI":"10.1007\/s00415-003-0199-9","volume":"250","author":"HJ ten Donkelaar","year":"2003","unstructured":"ten Donkelaar, H. J., Lammens, M., Wesseling, P., Thijssen, H. O. & Renier, W. O. Development and developmental disorders of the human cerebellum. J. Neurol. 250, 1025\u20131036 https:\/\/doi.org\/10.1007\/s00415-003-0199-9 (2003).","journal-title":"J. Neurol."},{"key":"739_CR83","doi-asserted-by":"publisher","first-page":"eade1283","DOI":"10.1126\/scitranslmed.ade1283","volume":"15","author":"SA Ament","year":"2023","unstructured":"Ament, S. A. et al. A single-cell genomic atlas for maturation of the human cerebellum during early childhood. Sci. Transl. Med. 15, eade1283 https:\/\/doi.org\/10.1126\/scitranslmed.ade1283 (2023).","journal-title":"Sci. Transl. Med."},{"key":"739_CR84","doi-asserted-by":"publisher","first-page":"43","DOI":"10.1016\/j.devcel.2020.06.009","volume":"54","author":"J DeSisto","year":"2020","unstructured":"DeSisto, J. et al. Single-cell transcriptomic analyses of the developing meninges reveal meningeal fibroblast diversity and function. Dev. Cell 54, 43\u201359.e44 https:\/\/doi.org\/10.1016\/j.devcel.2020.06.009 (2020).","journal-title":"Dev. Cell"},{"key":"739_CR85","doi-asserted-by":"publisher","first-page":"39","DOI":"10.1016\/j.neuroimage.2005.06.013","volume":"28","author":"G Allen","year":"2005","unstructured":"Allen, G. et al. Magnetic resonance imaging of cerebellar-prefrontal and cerebellar-parietal functional connectivity. NeuroImage 28, 39\u201348 https:\/\/doi.org\/10.1016\/j.neuroimage.2005.06.013 (2005).","journal-title":"NeuroImage"},{"key":"739_CR86","doi-asserted-by":"publisher","first-page":"2322","DOI":"10.1152\/jn.00339.2011","volume":"106","author":"RL Buckner","year":"2011","unstructured":"Buckner, R. L., Krienen, F. M., Castellanos, A., Diaz, J. C. & Yeo, B. T. The organization of the human cerebellum estimated by intrinsic functional connectivity. J. Neurophysiol. 106, 2322\u20132345 https:\/\/doi.org\/10.1152\/jn.00339.2011 (2011).","journal-title":"J. Neurophysiol."},{"key":"739_CR87","doi-asserted-by":"publisher","DOI":"10.1038\/s41467-024-49339-1","volume":"15","author":"KM Gruver","year":"2024","unstructured":"Gruver, K. M. et al. Structured connectivity in the output of the cerebellar cortex. Nat. Commun. 15, 5563 https:\/\/doi.org\/10.1038\/s41467-024-49339-1 (2024).","journal-title":"Nat. Commun."},{"key":"739_CR88","doi-asserted-by":"publisher","first-page":"1512","DOI":"10.1038\/mp.2017.106","volume":"23","author":"T Moberget","year":"2018","unstructured":"Moberget, T. et al. Cerebellar volume and cerebellocerebral structural covariance in schizophrenia: a multisite mega-analysis of 983 patients and 1349 healthy controls. Mol. Psychiatry 23, 1512\u20131520 https:\/\/doi.org\/10.1038\/mp.2017.106 (2018).","journal-title":"Mol. Psychiatry"},{"key":"739_CR89","doi-asserted-by":"publisher","first-page":"fcab137","DOI":"10.1093\/braincomms\/fcab137","volume":"3","author":"ML Houston","year":"2021","unstructured":"Houston, M. L. et al. Functional connectivity abnormalities in type I Chiari: associations with cognition and pain. Brain Commun. 3, fcab137 (2021).","journal-title":"Brain Commun."},{"key":"739_CR90","doi-asserted-by":"publisher","first-page":"404","DOI":"10.1007\/s12311-018-0923-8","volume":"17","author":"JR Houston","year":"2018","unstructured":"Houston, J. R. et al. An electrophysiological study of cognitive and emotion processing in type I Chiari malformation. Cerebellum 17, 404\u2013418 (2018).","journal-title":"Cerebellum"},{"key":"739_CR91","doi-asserted-by":"publisher","first-page":"561","DOI":"10.1093\/brain\/121.4.561","volume":"121","author":"JD Schmahmann","year":"1998","unstructured":"Schmahmann, J. D. & Sherman, J. C. The cerebellar cognitive affective syndrome. Brain 121, 561\u2013579 (1998).","journal-title":"Brain"},{"key":"739_CR92","unstructured":"Stoodley, C. J. & Schmahmann, J. D. in Essentials of Cerebellum and Cerebellar Disorders (eds Gruol, D. L. et al.)\u00a0373\u2013381 (Springer, 2018)."},{"key":"739_CR93","doi-asserted-by":"publisher","first-page":"115473","DOI":"10.1016\/j.expneurol.2025.115473","volume":"395","author":"W Davalan","year":"2025","unstructured":"Davalan, W. et al. Animal models of Chiari malformation types 1 and 2: mechanistic insights and translational challenges. Exp. Neurol. 395, 115473 https:\/\/doi.org\/10.1016\/j.expneurol.2025.115473 (2025).","journal-title":"Exp. Neurol."},{"key":"739_CR94","doi-asserted-by":"publisher","first-page":"2914","DOI":"10.1093\/hmg\/ddu004","volume":"23","author":"F Di Rocco","year":"2014","unstructured":"Di Rocco, F. et al. FGFR3 mutation causes abnormal membranous ossification in achondroplasia. Hum. Mol. Genet. 23, 2914\u20132925 https:\/\/doi.org\/10.1093\/hmg\/ddu004 (2014).","journal-title":"Hum. Mol. Genet."},{"key":"739_CR95","doi-asserted-by":"publisher","first-page":"329","DOI":"10.1007\/s00381-003-0879-x","volume":"20","author":"P Steinbok","year":"2004","unstructured":"Steinbok, P. Clinical features of Chiari I malformations. Child\u2019s Nerv. Syst. 20, 329\u2013331 https:\/\/doi.org\/10.1007\/s00381-003-0879-x (2004).","journal-title":"Child\u2019s Nerv. Syst."},{"key":"739_CR96","doi-asserted-by":"publisher","first-page":"244","DOI":"10.3171\/2010.11.Peds1097","volume":"7","author":"CK Yarbrough","year":"2011","unstructured":"Yarbrough, C. K. et al. Patients with Chiari malformation type I presenting with acute neurological deficits: case series. J. Neurosurg. Pediatr. 7, 244\u2013247 https:\/\/doi.org\/10.3171\/2010.11.Peds1097 (2011).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR97","doi-asserted-by":"publisher","first-page":"1341","DOI":"10.1227\/neu.0000000000003249","volume":"96","author":"VP Gupta","year":"2025","unstructured":"Gupta, V. P. et al. Using artificial intelligence to identify three presenting phenotypes of Chiari type-1 malformation and syringomyelia. Neurosurgery 96, 1341\u20131352 https:\/\/doi.org\/10.1227\/neu.0000000000003249 (2025).","journal-title":"Neurosurgery"},{"key":"739_CR98","doi-asserted-by":"publisher","first-page":"430","DOI":"10.3171\/2011.8.Peds10427","volume":"8","author":"BG Rocque","year":"2011","unstructured":"Rocque, B. G., George, T. M., Kestle, J. & Iskandar, B. J. Treatment practices for Chiari malformation type I with syringomyelia: results of a survey of the American Society of Pediatric Neurosurgeons. J. Neurosurg. Pediatr. 8, 430\u2013437 https:\/\/doi.org\/10.3171\/2011.8.Peds10427 (2011).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR99","doi-asserted-by":"publisher","first-page":"118","DOI":"10.3171\/ped\/2008\/1\/2\/118","volume":"1","author":"NM Wetjen","year":"2008","unstructured":"Wetjen, N. M., Heiss, J. D. & Oldfield, E. H. Time course of syringomyelia resolution following decompression of Chiari malformation type I. J. Neurosurg. Pediatr. 1, 118\u2013123 https:\/\/doi.org\/10.3171\/ped\/2008\/1\/2\/118 (2008).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR100","doi-asserted-by":"publisher","first-page":"22","DOI":"10.3171\/2010.9.Peds10383","volume":"7","author":"DL Brockmeyer","year":"2011","unstructured":"Brockmeyer, D. L. Editorial. Chiari malformation type I and scoliosis: the complexity of curves. J. Neurosurg. Pediatr. 7, 22\u201323 https:\/\/doi.org\/10.3171\/2010.9.Peds10383 (2011).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR101","doi-asserted-by":"publisher","first-page":"25","DOI":"10.3171\/2010.10.Peds10154","volume":"7","author":"MD Krieger","year":"2011","unstructured":"Krieger, M. D., Falkinstein, Y., Bowen, I. E., Tolo, V. T. & McComb, J. G. Scoliosis and Chiari malformation type I in children. J. Neurosurg. Pediatr. 7, 25\u201329 https:\/\/doi.org\/10.3171\/2010.10.Peds10154 (2011).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR102","doi-asserted-by":"publisher","first-page":"1749","DOI":"10.1016\/j.jocn.2015.05.023","volume":"22","author":"C Glenn","year":"2015","unstructured":"Glenn, C. et al. Spinal cord detethering in children with tethered cord syndrome and Chiari type 1 malformations. J. Clin. Neurosci. 22, 1749\u20131752 https:\/\/doi.org\/10.1016\/j.jocn.2015.05.023 (2015).","journal-title":"J. Clin. Neurosci."},{"key":"739_CR103","doi-asserted-by":"publisher","first-page":"20","DOI":"10.1016\/j.surneu.2009.03.008","volume":"72","author":"TH Milhorat","year":"2009","unstructured":"Milhorat, T. H. et al. Association of Chiari malformation type I and tethered cord syndrome: preliminary results of sectioning filum terminale. Surg. Neurol. 72, 20\u201335 https:\/\/doi.org\/10.1016\/j.surneu.2009.03.008 (2009).","journal-title":"Surg. Neurol."},{"key":"739_CR104","doi-asserted-by":"publisher","first-page":"S353","DOI":"10.1007\/s10072-011-0745-7","volume":"32","author":"LG Valentini","year":"2011","unstructured":"Valentini, L. G. et al. Tethered cord: natural history, surgical outcome and risk for Chiari malformation 1 (CM1): a review of 110 detethering. Neurol. Sci. 32, S353\u2013S356 https:\/\/doi.org\/10.1007\/s10072-011-0745-7 (2011).","journal-title":"Neurol. Sci."},{"key":"739_CR105","doi-asserted-by":"publisher","first-page":"371","DOI":"10.5664\/jcsm.2592","volume":"9","author":"A Losurdo","year":"2013","unstructured":"Losurdo, A. et al. Sleep disordered breathing in children and adolescents with Chiari malformation type I. J. Clin. Sleep Med. 9, 371\u2013377 https:\/\/doi.org\/10.5664\/jcsm.2592 (2013).","journal-title":"J. Clin. Sleep Med."},{"key":"739_CR106","doi-asserted-by":"publisher","first-page":"393","DOI":"10.3171\/2024.5.Peds24105","volume":"34","author":"M Jarrell","year":"2024","unstructured":"Jarrell, M. et al. Sleep-disordered breathing in children with Chiari type I malformation. J. Neurosurg. Pediatr. 34, 393\u2013401 https:\/\/doi.org\/10.3171\/2024.5.Peds24105 (2024).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR107","doi-asserted-by":"publisher","first-page":"102618","DOI":"10.1016\/j.semerg.2025.102618","volume":"51","author":"GA Rojas-Martelo","year":"2025","unstructured":"Rojas-Martelo, G. A., McKiernan, C. M. & Jordan, P.\u00a0Chiari malformation: a differential diagnosis to keep in mind in primary care. Med. Fam. 51, 102618 https:\/\/doi.org\/10.1016\/j.semerg.2025.102618 (2025).","journal-title":"Med. Fam."},{"key":"739_CR108","doi-asserted-by":"publisher","first-page":"170","DOI":"10.3171\/2017.3.Peds16604","volume":"20","author":"TC Hankinson","year":"2017","unstructured":"Hankinson, T. C. et al. Analysis and interrater reliability of pB-C2 using MRI and CT: data from the Park-Reeves Syringomyelia Research Consortium on behalf of the Pediatric Craniocervical Society. J. Neurosurg. Pediatr. 20, 170\u2013175 https:\/\/doi.org\/10.3171\/2017.3.Peds16604 (2017).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR109","doi-asserted-by":"publisher","first-page":"176","DOI":"10.3171\/2020.12.Peds20809","volume":"28","author":"VM Ravindra","year":"2021","unstructured":"Ravindra, V. M. et al. A multicenter validation of the condylar-C2 sagittal vertical alignment in Chiari malformation type I: a study using the Park-Reeves Syringomyelia Research Consortium. J. Neurosurg. Pediatr. 28, 176\u2013182 https:\/\/doi.org\/10.3171\/2020.12.Peds20809 (2021).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR110","doi-asserted-by":"publisher","first-page":"723","DOI":"10.1227\/neu.0000000000002634","volume":"93","author":"DF Bauer","year":"2023","unstructured":"Bauer, D. F. et al. Congress of neurological surgeons systematic review and evidence-based guidelines for patients with Chiari malformation: diagnosis. Neurosurgery 93, 723\u2013726 https:\/\/doi.org\/10.1227\/neu.0000000000002633 (2023).\u00a0","journal-title":"Neurosurgery"},{"key":"739_CR111","doi-asserted-by":"publisher","first-page":"727","DOI":"10.1227\/neu.0000000000002634","volume":"93","author":"EM Jackson","year":"2023","unstructured":"Jackson, E. M. et al. Congress of neurological surgeons systematic review and evidence-based guidelines for patients with Chiari malformation: symptoms. Neurosurgery 93, 727\u2013730 https:\/\/doi.org\/10.1227\/neu.0000000000002634 (2023).\u00a0","journal-title":"Neurosurgery"},{"key":"739_CR112","doi-asserted-by":"publisher","first-page":"731","DOI":"10.1227\/neu.0000000000002635","volume":"93","author":"JV Pattisapu","year":"2023","unstructured":"Pattisapu, J. V. et al. Congress of neurological surgeons systematic review and evidence-based guidelines for patients with Chiari malformation: surgical interventions. Neurosurgery 93, 731\u2013735 https:\/\/doi.org\/10.1227\/neu.0000000000002635 (2023). Refs. 110\u2013112 are consensus guidelines on CM1 diagnosis, symptoms and treatment, highlighting the field\u2019s reliance on low-quality evidence.","journal-title":"Neurosurgery"},{"key":"739_CR113","doi-asserted-by":"publisher","first-page":"1245","DOI":"10.1007\/s00381-016-3113-3","volume":"32","author":"RS Tubbs","year":"2016","unstructured":"Tubbs, R. S. et al. Sagittal MRI often overestimates the degree of cerebellar tonsillar ectopia: a potential for misdiagnosis of the Chiari I malformation. Child\u2019s\u00a0Nerv. Syst. 32, 1245\u20131248 https:\/\/doi.org\/10.1007\/s00381-016-3113-3 (2016).","journal-title":"Child\u2019s\u00a0Nerv. Syst."},{"key":"739_CR114","doi-asserted-by":"publisher","first-page":"486","DOI":"10.3171\/2024.11.Peds24478","volume":"35","author":"NG Alexiades","year":"2025","unstructured":"Alexiades, N. G. et al. Building consensus regarding the definition of abnormal craniocervical motion in pediatric patients with Chiari malformation: a modified Delphi study. J. Neurosurg. Pediatr. 35, 486\u2013496 https:\/\/doi.org\/10.3171\/2024.11.Peds24478 (2025).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR115","doi-asserted-by":"publisher","first-page":"498","DOI":"10.3171\/2018.10.Peds18407","volume":"23","author":"TJ Leon","year":"2019","unstructured":"Leon, T. J. et al. Patients with \u201cbenign\u201d Chiari I malformations require surgical decompression at a low rate. J. Neurosurg. Pediatr. 23, 498\u2013506 https:\/\/doi.org\/10.3171\/2018.10.Peds18407 (2019).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR116","doi-asserted-by":"publisher","first-page":"101034","DOI":"10.1016\/j.eclinm.2021.101034","volume":"38","author":"G Frija","year":"2021","unstructured":"Frija, G. et al. How to improve access to medical imaging in low- and middle-income countries? eClinicalMedicine 38, 101034 https:\/\/doi.org\/10.1016\/j.eclinm.2021.101034 (2021).","journal-title":"eClinicalMedicine"},{"key":"739_CR117","doi-asserted-by":"publisher","first-page":"151","DOI":"10.1016\/j.nec.2022.08.011","volume":"34","author":"S Das","year":"2023","unstructured":"Das, S., Stone, L., Godzik, J. & Kelly, M. Spine deformity associated with Chiari I malformation and syringomyelia. Neurosurg. Clin. N. Am. 34, 151\u2013157 https:\/\/doi.org\/10.1016\/j.nec.2022.08.011 (2023).","journal-title":"Neurosurg. Clin. N. Am."},{"key":"739_CR118","doi-asserted-by":"publisher","first-page":"61","DOI":"10.1016\/j.nec.2022.09.001","volume":"34","author":"M Ellington","year":"2023","unstructured":"Ellington, M. & Francomano, C. A. Chiari I malformations and the heritable disorders of connective tissue. Neurosurg. Clin. N. Am. 34, 61\u201365 https:\/\/doi.org\/10.1016\/j.nec.2022.09.001 (2023).","journal-title":"Neurosurg. Clin. N. Am."},{"key":"739_CR119","doi-asserted-by":"publisher","first-page":"195","DOI":"10.1002\/ajmg.c.31549","volume":"175","author":"FC Henderson Sr","year":"2017","unstructured":"Henderson, F. C. Sr et al. Neurological and spinal manifestations of the Ehlers\u2013Danlos syndromes. Am. J. Med. Genet. C 175, 195\u2013211 https:\/\/doi.org\/10.1002\/ajmg.c.31549 (2017).","journal-title":"Am. J. Med. Genet. C"},{"key":"739_CR120","doi-asserted-by":"publisher","first-page":"1484504","DOI":"10.3389\/fneur.2024.1484504","volume":"15","author":"IS Ruhoy","year":"2024","unstructured":"Ruhoy, I. S. et al. Comorbidities and neurosurgical interventions in a cohort with connective tissue disorders. Front. Neurol. 15, 1484504 https:\/\/doi.org\/10.3389\/fneur.2024.1484504\u00a0(2024).","journal-title":"Front. Neurol."},{"key":"739_CR121","doi-asserted-by":"publisher","first-page":"3287","DOI":"10.3390\/jcm12093287","volume":"12","author":"WMN Bin Wan Hassan","year":"2023","unstructured":"Bin Wan Hassan, W. M. N. et al. Overview of spontaneous intracranial hypotension and differential diagnosis with Chiari I malformation. J. Clin. Med. 12, 3287 https:\/\/doi.org\/10.3390\/jcm12093287 (2023).","journal-title":"J. Clin. Med."},{"key":"739_CR122","doi-asserted-by":"publisher","first-page":"2307","DOI":"10.1007\/s00234-022-02993-y","volume":"64","author":"SA Ebrahimzadeh","year":"2022","unstructured":"Ebrahimzadeh, S. A. et al. MRI findings differentiating tonsillar herniation caused by idiopathic intracranial hypertension from Chiari I malformation. Neuroradiology 64, 2307\u20132314 https:\/\/doi.org\/10.1007\/s00234-022-02993-y (2022).","journal-title":"Neuroradiology"},{"key":"739_CR123","doi-asserted-by":"publisher","first-page":"116","DOI":"10.3171\/2014.10.Spine14176","volume":"22","author":"A Goel","year":"2015","unstructured":"Goel, A. Is atlantoaxial instability the cause of Chiari malformation? Outcome analysis of 65 patients treated by atlantoaxial fixation. J. Neurosurg. Spine 22, 116\u2013127 https:\/\/doi.org\/10.3171\/2014.10.Spine14176 (2015).","journal-title":"J. Neurosurg. Spine"},{"key":"739_CR124","doi-asserted-by":"publisher","first-page":"820","DOI":"10.3171\/2015.3.Spine15233","volume":"23","author":"DL Brockmeyer","year":"2015","unstructured":"Brockmeyer, D. L. et al. Chiari malformation type 1 and atlanto axial instability: a letter from the Pediatric Craniocervical Society. J. Neurosurg. Spine 23, 820\u2013821 https:\/\/doi.org\/10.3171\/2015.3.Spine15233 (2015).","journal-title":"J. Neurosurg. Spine"},{"key":"739_CR125","doi-asserted-by":"publisher","first-page":"1356","DOI":"10.3171\/2020.9.Jns202641","volume":"135","author":"AT Yahanda","year":"2021","unstructured":"Yahanda, A. T., Simon, L. E. & Limbrick, D. D. Outcomes for various dural graft materials after posterior fossa decompression with duraplasty for Chiari malformation type I: a systematic review and meta-analysis. J. Neurosurg. 135, 1356\u20131369 https:\/\/doi.org\/10.3171\/2020.9.Jns202641 (2021).","journal-title":"J. Neurosurg."},{"key":"739_CR126","doi-asserted-by":"publisher","first-page":"184","DOI":"10.3171\/ped.2004.101.2.0184","volume":"101","author":"FM Limonadi","year":"2004","unstructured":"Limonadi, F. M. & Selden, N. R. Dura-splitting decompression of the craniocervical junction: reduced operative time, hospital stay, and cost with equivalent early outcome. J. Neurosurg. 101, 184\u2013188 https:\/\/doi.org\/10.3171\/ped.2004.101.2.0184 (2004).","journal-title":"J. Neurosurg."},{"key":"739_CR127","doi-asserted-by":"publisher","first-page":"2015","DOI":"10.1056\/NEJMoa2402821","volume":"394","author":"DD Limbrick Jr","year":"2026","unstructured":"Limbrick, D. D. Jr et al. Decompression with or without duraplasty for Chiari I and syringomyelia. N. Engl. J. Med. 394, 2015\u20132025 https:\/\/doi.org\/10.1056\/NEJMoa2402821 (2026). A multicentre cluster-randomized trial comparing PFD with PFD-D in paediatric CM1 with syringomyelia, the highest level of evidence to directly answer a long-standing clinical controversy in the field.","journal-title":"N. Engl. J. Med."},{"key":"739_CR128","doi-asserted-by":"publisher","first-page":"e345","DOI":"10.1227\/ons.0000000000000875","volume":"25","author":"I Dolas","year":"2023","unstructured":"Dolas, I. et al. Full-endoscopic technique for posterior fossa decompression in Chiari malformation. Oper. Neurosurg. 25, e345\u2013e351 https:\/\/doi.org\/10.1227\/ons.0000000000000875 (2023).","journal-title":"Oper. Neurosurg."},{"key":"739_CR129","doi-asserted-by":"publisher","first-page":"3049","DOI":"10.1007\/s00586-024-08299-2","volume":"33","author":"G G\u00fcnerhan","year":"2024","unstructured":"G\u00fcnerhan, G., \u00c7a\u011f\u0131l, E., Da\u011flar, Z., Dalg\u0131\u00e7, A. & Belen, A. D. A comparative analysis of neuroendoscopic foramen magnum decompression versus traditional open surgery for Chiari malformation type I. Eur. Spine J. 33, 3049\u20133059 https:\/\/doi.org\/10.1007\/s00586-024-08299-2 (2024).","journal-title":"Eur. Spine J."},{"key":"739_CR130","doi-asserted-by":"publisher","first-page":"260","DOI":"10.1159\/000120969","volume":"23","author":"AH Menezes","year":"1995","unstructured":"Menezes, A. H. Primary craniovertebral anomalies and the hindbrain herniation syndrome (Chiari I): data base analysis. Pediatr. Neurosurg. 23, 260\u2013269 https:\/\/doi.org\/10.1159\/000120969 (1995).","journal-title":"Pediatr. Neurosurg."},{"key":"739_CR131","doi-asserted-by":"publisher","first-page":"520","DOI":"10.1097\/00006123-199903000-00050","volume":"44","author":"PA Grabb","year":"1999","unstructured":"Grabb, P. A., Mapstone, T. B. & Oakes, W. J. Ventral brain stem compression in pediatric and young adult patients with Chiari I malformations. Neurosurgery 44, 520\u2013527 https:\/\/doi.org\/10.1097\/00006123-199903000-00050 (1999).","journal-title":"Neurosurgery"},{"key":"739_CR132","doi-asserted-by":"publisher","first-page":"332","DOI":"10.1093\/neuros\/nyaa460","volume":"88","author":"TS CreveCoeur","year":"2021","unstructured":"CreveCoeur, T. S. et al. Occipital-cervical fusion and ventral decompression in the surgical management of Chiari-1 malformation and syringomyelia: analysis of data from the Park-Reeves Syringomyelia Research Consortium. Neurosurgery 88, 332\u2013341 https:\/\/doi.org\/10.1093\/neuros\/nyaa460 (2021).","journal-title":"Neurosurgery"},{"key":"739_CR133","doi-asserted-by":"publisher","first-page":"439","DOI":"10.3171\/2020.4.Peds20113","volume":"26","author":"VM Ravindra","year":"2020","unstructured":"Ravindra, V. M. et al. Defining the role of the condylar\u2013C2 sagittal vertical alignment in Chiari malformation type I. J. Neurosurg. Pediatr. 26, 439\u2013444 https:\/\/doi.org\/10.3171\/2020.4.Peds20113 (2020).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR134","doi-asserted-by":"publisher","first-page":"189","DOI":"10.3171\/ped.2004.101.2.0189","volume":"101","author":"LJ Kim","year":"2004","unstructured":"Kim, L. J., Rekate, H. L., Klopfenstein, J. D. & Sonntag, V. K. Treatment of basilar invagination associated with Chiari I malformations in the pediatric population: cervical reduction and posterior occipitocervical fusion. J. Neurosurg. 101, 189\u2013195 https:\/\/doi.org\/10.3171\/ped.2004.101.2.0189 (2004).","journal-title":"J. Neurosurg."},{"key":"739_CR135","doi-asserted-by":"publisher","first-page":"119","DOI":"10.1016\/j.nec.2022.08.002","volume":"34","author":"BJ Dlouhy","year":"2023","unstructured":"Dlouhy, B. J. & Menezes, A. H. Management of ventral brainstem compression in Chiari malformation type I. Neurosurg. Clin. N. Am. 34, 119\u2013129 https:\/\/doi.org\/10.1016\/j.nec.2022.08.002 (2023).","journal-title":"Neurosurg. Clin. N. Am."},{"key":"739_CR136","doi-asserted-by":"publisher","first-page":"134","DOI":"10.3171\/2012.3.Peds11340","volume":"10","author":"RJ Bollo","year":"2012","unstructured":"Bollo, R. J., Riva-Cambrin, J., Brockmeyer, M. M. & Brockmeyer, D. L. Complex Chiari malformations in children: an analysis of preoperative risk factors for occipitocervical fusion. J. Neurosurg. Pediatr. 10, 134\u2013141 https:\/\/doi.org\/10.3171\/2012.3.Peds11340 (2012).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR137","doi-asserted-by":"publisher","first-page":"221","DOI":"10.3171\/2020.12.Peds20729","volume":"28","author":"RR Iyer","year":"2021","unstructured":"Iyer, R. R., Grimmer, J. F. & Brockmeyer, D. L. Endoscopic transnasal\/transoral odontoid resection in children: results of a combined neurosurgical and otolaryngological protocolized, institutional approach. J. Neurosurg. Pediatr. 28, 221\u2013228 https:\/\/doi.org\/10.3171\/2020.12.Peds20729 (2021).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR138","doi-asserted-by":"publisher","first-page":"694","DOI":"10.1179\/1743132812y.0000000066","volume":"34","author":"KE Hekman","year":"2012","unstructured":"Hekman, K. E. et al. Positive and negative predictors for good outcome after decompressive surgery for Chiari malformation type 1 as scored on the Chicago Chiari Outcome Scale. Neurol. Res. 34, 694\u2013700 https:\/\/doi.org\/10.1179\/1743132812y.0000000066 (2012).","journal-title":"Neurol. Res."},{"key":"739_CR139","doi-asserted-by":"publisher","first-page":"E3","DOI":"10.3171\/2022.12.FOCUS22625","volume":"54","author":"L Antkowiak","year":"2023","unstructured":"Antkowiak, L. et al. External validation of the Chicago Chiari Outcome Scale in adults with Chiari malformation type I. Neurosurg. Focus 54, E3 https:\/\/doi.org\/10.3171\/2022.12.FOCUS22625 (2023).","journal-title":"Neurosurg. Focus"},{"key":"739_CR140","doi-asserted-by":"publisher","first-page":"679","DOI":"10.3171\/2014.3.Peds13503","volume":"13","author":"CK Yarbrough","year":"2014","unstructured":"Yarbrough, C. K. et al. External validation of the Chicago Chiari Outcome Scale. J. Neurosurg. Pediatr. 13, 679\u2013684 https:\/\/doi.org\/10.3171\/2014.3.Peds13503 (2014).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR141","doi-asserted-by":"publisher","first-page":"76","DOI":"10.3171\/2015.5.PEDS1513","volume":"17","author":"TR Ladner","year":"2016","unstructured":"Ladner, T. R., Westrick, A. C., Wellons, J. C. 3rd & Shannon, C. N. Health-related quality of life in pediatric Chiari type I malformation: the Chiari Health Index for Pediatrics. J. Neurosurg. Pediatr. 17, 76\u201385 https:\/\/doi.org\/10.3171\/2015.5.PEDS1513 (2016).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR142","doi-asserted-by":"publisher","first-page":"139","DOI":"10.3171\/2020.7.PEDS20250","volume":"27","author":"GE Sellyn","year":"2021","unstructured":"Sellyn, G. E. et al. Effectiveness of the Chiari Health Index for Pediatrics instrument in measuring postoperative health-related quality of life in pediatric patients with Chiari malformation type I. J. Neurosurg. Pediatr. 27, 139\u2013144 https:\/\/doi.org\/10.3171\/2020.7.PEDS20250 (2021).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR143","doi-asserted-by":"publisher","first-page":"248","DOI":"10.3171\/2010.12.peds10379","volume":"7","author":"RS Tubbs","year":"2011","unstructured":"Tubbs, R. S. et al. Institutional experience with 500 cases of surgically treated pediatric Chiari malformation type I. J. Neurosurg. Pediatr. 7, 248\u2013256 https:\/\/doi.org\/10.3171\/2010.12.peds10379 (2011).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR144","doi-asserted-by":"publisher","first-page":"879","DOI":"10.1177\/03331024221079296","volume":"42","author":"DC Thunstedt","year":"2022","unstructured":"Thunstedt, D. C. et al. Headache characteristics and postoperative course in Chiari I malformation. Cephalalgia 42, 879\u2013887 https:\/\/doi.org\/10.1177\/03331024221079296 (2022).","journal-title":"Cephalalgia"},{"key":"739_CR145","doi-asserted-by":"publisher","first-page":"430","DOI":"10.3171\/2011.8.peds10427","volume":"8","author":"BG Rocque","year":"2011","unstructured":"Rocque, B. G., George, T. M., Kestle, J. & Iskandar, B. J. Treatment practices for Chiari malformation type I with syringomyelia: results of a survey of the American Society of Pediatric Neurosurgeons. J. Neurosurg. Pediatr. 8, 430\u2013437 https:\/\/doi.org\/10.3171\/2011.8.peds10427 (2011).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR146","doi-asserted-by":"publisher","first-page":"629","DOI":"10.3171\/2020.1.PEDS19493","volume":"25","author":"AT Hale","year":"2020","unstructured":"Hale, A. T. et al. Factors associated with syrinx size in pediatric patients treated for Chiari malformation type I and syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium. J. Neurosurg. Pediatr. 25, 629\u2013639 https:\/\/doi.org\/10.3171\/2020.1.PEDS19493 (2020).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR147","doi-asserted-by":"publisher","first-page":"176","DOI":"10.1007\/s11065-018-9368-6","volume":"28","author":"JM Rogers","year":"2018","unstructured":"Rogers, J. M., Savage, G. & Stoodley, M. A. A systematic review of cognition in Chiari I malformation. Neuropsychol. Rev. 28, 176\u2013187 https:\/\/doi.org\/10.1007\/s11065-018-9368-6 (2018).","journal-title":"Neuropsychol. Rev."},{"key":"739_CR148","doi-asserted-by":"publisher","first-page":"952","DOI":"10.1007\/s11065-023-09622-2","volume":"34","author":"M Garcia","year":"2024","unstructured":"Garcia, M. et al. Cognition in Chiari malformation type I: an update of a systematic review. Neuropsychol. Rev. 34, 952\u2013973 https:\/\/doi.org\/10.1007\/s11065-023-09622-2 (2024).","journal-title":"Neuropsychol. Rev."},{"key":"739_CR149","doi-asserted-by":"publisher","first-page":"1450","DOI":"10.1227\/neu.0000000000003481","volume":"97","author":"B Shao","year":"2025","unstructured":"Shao, B. et al. Cognitive-affective improvement on Cerebellar Neuropsychiatric Rating Scale scores in adults and children after decompression of Chiari malformation type I. Neurosurgery 97, 1450\u20131458 https:\/\/doi.org\/10.1227\/neu.0000000000003481 (2025).","journal-title":"Neurosurgery"},{"key":"739_CR150","doi-asserted-by":"publisher","first-page":"371","DOI":"10.1159\/000488460","volume":"53","author":"M Lacy","year":"2018","unstructured":"Lacy, M. et al. Prevalence of psychiatric diagnoses in pediatric Chiari malformation type 1. Pediatr. Neurosurg. 53, 371\u2013378 https:\/\/doi.org\/10.1159\/000488460 (2018).","journal-title":"Pediatr. Neurosurg."},{"key":"739_CR151","doi-asserted-by":"publisher","first-page":"262","DOI":"10.3171\/2014.9.JNS14406","volume":"122","author":"JK Greenberg","year":"2015","unstructured":"Greenberg, J. K. et al. Outcome methods used in clinical studies of Chiari malformation type I: a systematic review. J. Neurosurg. 122, 262\u2013272 https:\/\/doi.org\/10.3171\/2014.9.JNS14406 (2015).","journal-title":"J. Neurosurg."},{"key":"739_CR152","doi-asserted-by":"publisher","first-page":"686","DOI":"10.3171\/2023.7.PEDS22114","volume":"32","author":"KA Kelly","year":"2023","unstructured":"Kelly, K. A. et al. The role of traditional social determinants of health in referral patterns, timing of surgery, and Chiari Health Index for Pediatrics-determined quality of life in children with symptomatic Chiari type I malformation. J. Neurosurg. Pediatr. 32, 686\u2013691 https:\/\/doi.org\/10.3171\/2023.7.PEDS22114 (2023).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR153","doi-asserted-by":"publisher","first-page":"204","DOI":"10.1016\/j.jpeds.2016.08.074","volume":"179","author":"SK Lam","year":"2016","unstructured":"Lam, S. K., Mayer, R. R., Luerssen, T. G. & Pan, I. W. Hospitalization cost model of pediatric surgical treatment of Chiari type 1 malformation. J. Pediatr. 179, 204\u2013210 e203 https:\/\/doi.org\/10.1016\/j.jpeds.2016.08.074 (2016).","journal-title":"J. Pediatr."},{"key":"739_CR154","doi-asserted-by":"publisher","first-page":"382","DOI":"10.3171\/2023.11.PEDS23448","volume":"33","author":"GM Koller","year":"2024","unstructured":"Koller, G. M. et al. Patient and caregiver perceptions of Chiari malformation: a qualitative analysis of online discussion boards. J. Neurosurg. Pediatr. 33, 382\u2013389 https:\/\/doi.org\/10.3171\/2023.11.PEDS23448 (2024).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR155","doi-asserted-by":"publisher","first-page":"1758","DOI":"10.3174\/ajnr.A3435","volume":"34","author":"AM Bagci","year":"2013","unstructured":"Bagci, A. M., Lee, S. H., Nagornaya, N., Green, B. A. & Alperin, N. Automated posterior cranial fossa volumetry by MRI: applications to Chiari malformation type I. Am. J. Neuroradiol. 34, 1758\u20131763 https:\/\/doi.org\/10.3174\/ajnr.A3435 (2013).","journal-title":"Am. J. Neuroradiol."},{"key":"739_CR156","doi-asserted-by":"publisher","first-page":"65","DOI":"10.3171\/2017.8.Peds17345","volume":"21","author":"SSS Khalsa","year":"2018","unstructured":"Khalsa, S. S. S. et al. Morphometric and volumetric comparison of 102 children with symptomatic and asymptomatic Chiari malformation type I. J. Neurosurg. Pediatr. 21, 65\u201371 https:\/\/doi.org\/10.3171\/2017.8.Peds17345 (2018).","journal-title":"J. Neurosurg. Pediatr."},{"key":"739_CR157","doi-asserted-by":"publisher","first-page":"647","DOI":"10.3171\/2010.11.Jns102148","volume":"115","author":"R Noudel","year":"2011","unstructured":"Noudel, R. et al. Posterior fossa volume increase after surgery for Chiari malformation type I: a quantitative assessment using magnetic resonance imaging and correlations with the treatment response. J. Neurosurg. 115, 647\u2013658 https:\/\/doi.org\/10.3171\/2010.11.Jns102148 (2011).","journal-title":"J. Neurosurg."},{"key":"739_CR158","doi-asserted-by":"publisher","first-page":"189","DOI":"10.1016\/j.wneu.2011.02.022","volume":"76","author":"M Kumar","year":"2011","unstructured":"Kumar, M. et al. Correlation of diffusion tensor imaging metrics with neurocognitive function in Chiari I malformation. World Neurosurg. 76, 189\u2013194 https:\/\/doi.org\/10.1016\/j.wneu.2011.02.022 (2011).","journal-title":"World Neurosurg."},{"key":"739_CR159","doi-asserted-by":"publisher","first-page":"2323","DOI":"10.1093\/pm\/pnaa094","volume":"21","author":"JR Houston","year":"2020","unstructured":"Houston, J. R. et al. Evidence of neural microstructure abnormalities in type I Chiari malformation: associations among fiber tract integrity, pain, and cognitive dysfunction. Pain Med. 21, 2323\u20132335 https:\/\/doi.org\/10.1093\/pm\/pnaa094 (2020).","journal-title":"Pain Med."},{"key":"739_CR160","doi-asserted-by":"publisher","first-page":"1112","DOI":"10.3171\/2015.9.Jns151196","volume":"125","author":"V Krishna","year":"2016","unstructured":"Krishna, V. et al. Diffusion tensor imaging assessment of microstructural brainstem integrity in Chiari malformation type I. J. Neurosurg. 125, 1112\u20131119 https:\/\/doi.org\/10.3171\/2015.9.Jns151196 (2016).","journal-title":"J. Neurosurg."},{"key":"739_CR161","doi-asserted-by":"publisher","first-page":"E381","DOI":"10.1097\/brs.0000000000000781","volume":"40","author":"H Yan","year":"2015","unstructured":"Yan, H. et al. Diffusion tensor imaging in cervical syringomyelia secondary to Chiari I malformation: preliminary results. Spine 40, E381\u2013E387 https:\/\/doi.org\/10.1097\/brs.0000000000000781 (2015).","journal-title":"Spine"},{"key":"739_CR162","doi-asserted-by":"publisher","first-page":"462","DOI":"10.1016\/j.wneu.2020.01.149","volume":"136","author":"SN Steinberg","year":"2020","unstructured":"Steinberg, S. N., Greenfield, J. P. & Perrine, K. Neuroanatomic correlates for the neuropsychological manifestations of Chiari malformation type I. World Neurosurg. 136, 462\u2013469 https:\/\/doi.org\/10.1016\/j.wneu.2020.01.149 (2020).","journal-title":"World Neurosurg."},{"key":"739_CR163","doi-asserted-by":"publisher","first-page":"124","DOI":"10.1016\/j.wneu.2021.06.052","volume":"152","author":"L Antkowiak","year":"2021","unstructured":"Antkowiak, L., Rogalska, M., Stogowski, P., Anuszkiewicz, K. & Mandera, M. Clinical application of diffusion tensor imaging in Chiari malformation type I- advances and perspectives. A systematic review. World Neurosurg. 152, 124\u2013136 https:\/\/doi.org\/10.1016\/j.wneu.2021.06.052 (2021).","journal-title":"World Neurosurg."},{"key":"739_CR164","doi-asserted-by":"publisher","first-page":"1617","DOI":"10.1007\/s10072-015-2219-9","volume":"36","author":"R Fischbein","year":"2015","unstructured":"Fischbein, R. et al. Patient-reported Chiari malformation type I symptoms and diagnostic experiences: a report from the national Conquer Chiari Patient Registry database. Neurol. Sci. 36, 1617\u20131624 https:\/\/doi.org\/10.1007\/s10072-015-2219-9 (2015).","journal-title":"Neurol. Sci."}],"container-title":["Nature Reviews Disease Primers"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.nature.com\/articles\/s41572-026-00739-1.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.nature.com\/articles\/s41572-026-00739-1","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.nature.com\/articles\/s41572-026-00739-1.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T07:02:33Z","timestamp":1789628553000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.nature.com\/articles\/s41572-026-00739-1"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9,17]]},"references-count":164,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2026,12]]}},"alternative-id":["739"],"URL":"https:\/\/doi.org\/10.1038\/s41572-026-00739-1","relation":{},"ISSN":["2056-676X"],"issn-type":[{"value":"2056-676X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,9,17]]},"assertion":[{"value":"26 August 2026","order":1,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"17 September 2026","order":2,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"The authors declare no competing interests.","order":1,"name":"Ethics","label":"Competing interests","group":{"name":"EthicsHeading","label":"Ethics"}}],"article-number":"62"}}