b93b4dd904c149a0467968a7d1c6eb8e jcm-12-04626.pdf ecb071a6c89fce1b25f22ce15a0ddb926b3aa300 jcm-12-04626.pdf a7532a588564b868dc5085dfbd481144dc8c1b72038c01c2b7e93006f9b8468f jcm-12-04626.pdf Title: A Critical Update of the Classification of Chiariand Chiari-like Malformations Subject: Chiari malformations are a group of craniovertebral junction anomalies characterized by the herniation of cerebellar tonsils below the foramen magnum, often accompanied by brainstem descent. The existing classification systems for Chiari malformations have expanded from the original four categories to nine, leading to debates about the need for a more descriptive and etiopathogenic terminology. This review aims to examine the various classification approaches employed and proposes a simplified scheme to differentiate between different types of tonsillar herniations. Furthermore, it explores the most appropriate terminology for acquired herniation of cerebellar tonsils and other secondary Chiari-like malformations. Recent advances in magnetic resonance imaging (MRI) have revealed a higher prevalence and incidence of Chiari malformation Type 1 (CM1) and identified similar cerebellar herniations in individuals unrelated to the classic phenotypes described by Chiari. As we reassess the existing classifications, it becomes crucial to establish a terminology that accurately reflects the diverse presentations and underlying causes of these conditions. This paper contributes to the ongoing discussion by offering insights into the evolving understanding of Chiari malformations and proposing a simplified classification and terminology system to enhance diagnosis and management. Keywords: Arnold-Chiari Malformation; rare diseases; syringomyelia; Chiari malformation;classification Author: Juan Sahuquillo, Dulce Moncho, Alex Ferré, Diego López-Bermeo, Aasma Sahuquillo-Muxi and Maria A. 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application/pdf PDFMetadata: Objects: 1223 FreeObjects: 1 IncrementalUpdates: 0 DocumentCatalog: PageLayout: SinglePage PageMode: UseNone Outlines: Item: Title: Introduction Destination: section.1 Item: Title: A Brief Historical Review of the Chiari Malformation and the ‘Arnold-Chiari’ Eponym Destination: section.2 Item: Title: Neuroradiology. Early Radiological Classification of Chiari Malformations Destination: section.3 Item: Title: MRI: The Breakthrough for the Diagnosis of Chiari Malformations Destination: section.4 Item: Title: Classification Schemes: Cerebellar Tonsillar Herniation versus Chiari Malformations Destination: section.5 Item: Title: Primary Chiari Malformations Destination: section.6 Item: Title: Secondary Chiari Malformations Destination: section.7 Item: Title: Underdevelopment of the Posterior Fossa: The Original Sin in Primary CMs Destination: section.8 Item: Title: Chiari-like Malformations in the Cavalier King Charles Spaniel Dog Destination: section.9 Item: Title: Morphometric Studies of the Posterior Fossa in Humans Destination: section.10 Item: Title: Do I Have a Chiari Malformation 1? The Origin of the 3 5 mm Magic Rule Destination: section.11 Item: Title: The Canonical Chiari Types and New Phenotypes Destination: section.12 Children: Item: Title: CM1 (‘Classic CM1') Destination: subsection.12.1 Item: Title: CM2 and Spina Bifida Destination: subsection.12.2 Item: Title: The Chiari 0 Malformation (CM0) Destination: section.13 Item: Title: The Chiari 1.5 Malformation (CM1.5) Destination: section.14 Item: Title: Complex Malformations: A Puzzle of Mesodermic Disorders Destination: section.15 Item: Title: Syringomyelia in Chiari Malformations: An Elusive Pathophysiology Destination: section.16 Item: Title: References Destination: section.17 Info: Title: A Critical Update of the Classification of Chiariand Chiari-like Malformations Author: Juan Sahuquillo, Dulce Moncho, Alex Ferré, Diego López-Bermeo, Aasma Sahuquillo-Muxi and Maria A. Poca Subject: Chiari malformations are a group of craniovertebral junction anomalies characterized by the herniation of cerebellar tonsils below the foramen magnum, often accompanied by brainstem descent. The existing classification systems for Chiari malformations have expanded from the original four categories to nine, leading to debates about the need for a more descriptive and etiopathogenic terminology. This review aims to examine the various classification approaches employed and proposes a simplified scheme to differentiate between different types of tonsillar herniations. Furthermore, it explores the most appropriate terminology for acquired herniation of cerebellar tonsils and other secondary Chiari-like malformations. Recent advances in magnetic resonance imaging (MRI) have revealed a higher prevalence and incidence of Chiari malformation Type 1 (CM1) and identified similar cerebellar herniations in individuals unrelated to the classic phenotypes described by Chiari. As we reassess the existing classifications, it becomes crucial to establish a terminology that accurately reflects the diverse presentations and underlying causes of these conditions. This paper contributes to the ongoing discussion by offering insights into the evolving understanding of Chiari malformations and proposing a simplified classification and terminology system to enhance diagnosis and management. 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