title: An unusual cause of neck pain: acquired Chiari malformation leading to brainstem herniation and death.
abstract: BACKGROUND
  Chiari malformations are structural defects in which portions of the cerebellum are located below the foramen magnum. Of the four types of Chiari malformation, emergency physicians are most likely to encounter Type I (Chiari I). Chiari I malformations may be congenital or acquired. Congenital Chiari I malformations are most frequently encountered in the emergency department (ED) setting due to an exacerbation of subacute or chronic Chiari-related symptoms. However, acute Chiari-associated symptoms from an occult congenital or a secondary (acquired) Chiari malformation may occur.
  
  
  OBJECTIVE
  To present a literature-guided approach to the identification and initial management of patients with Chiari I malformations in the ED setting.
  
  
  METHODS
  We present the case of a 30-year-old man who presented to the ED with isolated cervical region pain, and who subsequently died as a result of acute brainstem herniation from an acquired Chiari I malformation.
  
  
  CONCLUSIONS
  Although rare, acute Chiari I malformation may present to the ED. The new finding of a Chiari I malformation should be presumed acquired until proved otherwise, and should trigger an evaluation for central nervous system lesions or hydrocephalus. Brain imaging to exclude increased intracranial pressure and, in certain cases, specialty consultation, are important considerations.
authors.affiliation: Department of Emergency Medicine, Washington Hospital Center & Georgetown University Hospital, Washington, DC 20007, USA.
authors.forename: Autumn
authors.initials: A
authors.lastname: Graham
authors.forename: Jonathan E
authors.initials: JE
authors.lastname: Davis
authors.forename: Alice J
authors.initials: AJ
authors.lastname: Gouvernayre
authors.forename: J Alex
authors.initials: JA
authors.lastname: Thomas
mesh_terms: D000328:Adult; D001139:Arnold-Chiari Malformation / Q000150:complications*; D001933:Brain Stem; D004677:Encephalocele / Q000209:etiology*; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D019547:Neck Pain / Q000209:etiology*