title: Review Article: Chiari Type I Malformation with or Without Syringomyelia: Prevalence and Genetics.
abstract: Chiari type I malformation has traditionally been defined as a downward herniation of the cerebellar tonsils of ≥5 mm through the foramen magnum and it is likely associated with a volumetrically reduced posterior fossa. Syringomyelia is commonly associated with Chiari type I malformation. We estimate the prevalence of these two conditions and determine that they are more common than previously expected. We identify the genetic syndromes associated with some cases of Chiari type I malformation, and we provide evidence in favor of a genetic hypothesis for at least a subset of the nonsyndromic cases.
authors.affiliation: Duke University Medical Center, Durham, North Carolina, marcy@chg.duhs.duke.edu.
authors.forename: Marcy C
authors.initials: MC
authors.lastname: Speer
authors.forename: David S
authors.initials: DS
authors.lastname: Enterline
authors.forename: Lorraine
authors.initials: L
authors.lastname: Mehltretter
authors.forename: Preston
authors.initials: P
authors.lastname: Hammock
authors.forename: Judith
authors.initials: J
authors.lastname: Joseph
authors.forename: Margaret
authors.initials: M
authors.lastname: Dickerson
authors.forename: Richard G
authors.initials: RG
authors.lastname: Ellenbogen
authors.forename: Thomas H
authors.initials: TH
authors.lastname: Milhorat
authors.forename: Michael A
authors.initials: MA
authors.lastname: Hauser
authors.forename: Timothy M
authors.initials: TM
authors.lastname: George