title: Noninvasive diagnosis and management of spontaneous intracranial hypotension in patients with marfan syndrome: Case Report and Review of the Literature.
abstract: BACKGROUND
  Spontaneous intracranial hypotension is an uncommon clinical entity. Heritable connective tissue disorders (HCTD), such as Marfan syndrome, are frequently implicated as an underlying cause, due to dural structural weaknesses that predispose patients to spontaneous cerebrospinal fluid (CSF) leak. Due to the high prevalence of multi-system disease in HCTD, diagnosis and treatment are often complicated.
  
  
  METHODS
  We present a 58-year-old female with Marfan syndrome on anticoagulation for a mechanical aortic valve replacement who came to medical attention with severe, acute-onset headache following a straining episode. Noninvasive magnetic resonance (MR) myelography confirmed thoracic CSF extravasations and multiple lumbar diverticula. The patient was treated conservatively and her symptoms resolved.
  
  
  CONCLUSIONS
  We discuss the common presentation, diagnostic tools, and treatment options for spontaneous CSF leaks in patients with Marfan syndrome or related HCTD with an emphasis on noninvasive modalities and a review of the major radiographic criteria used to diagnose dural abnormalities, such as dural ectasia.
authors.affiliation: Department of Neurosurgery, University of Utah School of Medicine, USA.
authors.forename: Luigi
authors.initials: L
authors.lastname: Bassani
authors.affiliation: Department of Neurosurgery, New York University School of Medicine, New York, USA.
authors.forename: Christopher S
authors.initials: CS
authors.lastname: Graffeo
authors.affiliation: Department of Emergency Medicine, New York-Presbyterian Hospital, New York, USA.
authors.forename: Navid
authors.initials: N
authors.lastname: Behrooz
authors.affiliation: Department of Neurosurgery, New York University School of Medicine, New York, USA.
authors.forename: Vineet
authors.initials: V
authors.lastname: Tyagi
authors.affiliation: Department of Neurosurgery, New York University School of Medicine, New York, USA.
authors.forename: Taylor
authors.initials: T
authors.lastname: Wilson
authors.affiliation: Department of Neurosurgery, New York University School of Medicine, New York, USA.
authors.forename: Saul
authors.initials: S
authors.lastname: Penaranda
authors.affiliation: Department of Pathology, New York University School of Medicine, New York, USA.
authors.forename: David
authors.initials: D
authors.lastname: Zagzag
authors.affiliation: Department of Cell Biology, New York University School of Medicine, New York, USA.
authors.forename: Daniel B
authors.initials: DB
authors.lastname: Rifkin
authors.affiliation: Department of Cell Biology, New York University School of Medicine, New York, USA ; Department of Radiation Oncology, New York University School of Medicine, New York, USA.
authors.forename: Mary Helen
authors.initials: MH
authors.lastname: Barcellos-Hoff
authors.affiliation: Department of Radiology, New York University School of Medicine, New York, USA.
authors.forename: Girish
authors.initials: G
authors.lastname: Fatterpekar
authors.affiliation: Department of Neurosurgery, New York University School of Medicine, New York, USA.
authors.forename: Dimitris
authors.initials: D
authors.lastname: Placantonakis
keywords: Magnetic resonance myelography; Marfan syndrome; spontaneous intracranial hypotension