{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T01:55:09Z","timestamp":1790387709512,"version":"4.1.0"},"reference-count":32,"publisher":"MDPI AG","issue":"20","license":[{"start":{"date-parts":[[2023,10,23]],"date-time":"2023-10-23T00:00:00Z","timestamp":1698019200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Arnold Chiari syndrome is a rare congenital disease of unknown prevalence and whose origin is still under study. It is encompassed within the posterior cranial malformations, showing a wide spectrum of symptomatology that can range from severe headache, dizziness, and paresthesia to complete asymptomatology. It is for this reason that early diagnosis of the disease is difficult, and it is usually diagnosed in adolescence. Treatment is based on remodeling and decompression of the malformed posterior cranial fossa, although the risk of residual symptoms after surgery is high. The aim of this review is to update all the existing information on this pathology by means of an exhaustive analysis covering all the scientific literature produced in the last 5 years. In addition, it has been carried out following the PRISMA model and registered in PROSPERO with code CRD42023394490. One of the main conclusions based on the results obtained in this review is that the origin of the syndrome could have a genetic basis and that the treatment of choice is the decompression of the posterior cerebral fossa.<\/jats:p>","DOI":"10.3390\/jcm12206694","type":"journal-article","created":{"date-parts":[[2023,10,23]],"date-time":"2023-10-23T05:54:35Z","timestamp":1698040475000},"page":"6694","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["Chiari Syndrome: Advances in Epidemiology and Pathogenesis: A Systematic Review"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2086-1222","authenticated-orcid":false,"given":"Raquel","family":"Rodr\u00edguez-Blanque","sequence":"first","affiliation":[{"name":"Research Group CTS1068, Andalusia Research Plan, Junta de Andaluc\u00eda, 18071 Granada, Spain"},{"name":"San Cecilio University Hospital, 18071 Granada, Spain"},{"name":"Nursing Department, Faculty of Health Sciences, University of Granada, 18071 Granada, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Almaz\u00e1n-Soto","sequence":"additional","affiliation":[{"name":"Hospital Vall D\u2019Hebron, 08035 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Beatriz","family":"Piqueras-Sola","sequence":"additional","affiliation":[{"name":"Research Group CTS1068, Andalusia Research Plan, Junta de Andaluc\u00eda, 18071 Granada, Spain"},{"name":"Hospital University Virgen de las Nieves, 18014 Granada, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4650-2159","authenticated-orcid":false,"given":"Juan Carlos","family":"S\u00e1nchez-Garc\u00eda","sequence":"additional","affiliation":[{"name":"Research Group CTS1068, Andalusia Research Plan, Junta de Andaluc\u00eda, 18071 Granada, Spain"},{"name":"Nursing Department, Faculty of Health Sciences, University of Granada, 18071 Granada, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1185-2941","authenticated-orcid":false,"given":"Andr\u00e9s","family":"Reinoso-Cobo","sequence":"additional","affiliation":[{"name":"Department of Nursing and Podiatry, Faculty of Health Sciences, University of Malaga, Arquitecto Francisco Pe\u00f1alosa 3, Ampliaci\u00f3n de Campus de Teatinos, 29071 Malaga, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0425-9135","authenticated-orcid":false,"given":"Mar\u00eda Jos\u00e9","family":"Menor-Rodr\u00edguez","sequence":"additional","affiliation":[{"name":"\u00c1rea Sanitaria Santiago de Compostela-Barbanza, Subdirecci\u00f3n de Humanizaci\u00f3n y Atenci\u00f3n a la Ciudadan\u00eda, 15706 Santiago de Compostela, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4019-8555","authenticated-orcid":false,"given":"Jonathan","family":"Cort\u00e9s-Mart\u00edn","sequence":"additional","affiliation":[{"name":"Research Group CTS1068, Andalusia Research Plan, Junta de Andaluc\u00eda, 18071 Granada, Spain"},{"name":"Nursing Department, Faculty of Health Sciences, University of Granada, 18071 Granada, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,10,23]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1136\/jnnp.68.1.13","article-title":"Arnold Chiari, or \u201cCruveilhier Cleland Chiari\u201d Malformation","volume":"68","author":"Pearce","year":"2000","journal-title":"J. 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