Spontaneous dural tear leading to intracranial hypotension and tonsillar herniation in Marfan syndrome: a case report

BMC Neurol. 2010 Jun 28:10:54. doi: 10.1186/1471-2377-10-54.

Abstract

Background: We describe the case of a 38 year old male with Marfan syndrome who presented with orthostatic headaches and seizures.

Case presentation: The patient was diagnosed with Spontaneous Intracranial Hypotension secondary to CSF leaks, objectively demonstrated by MR myelogram with intrathecal contrast. Epidural autologous blood patch was administered at the leakage site leading to significant improvement.

Conclusion: Our literature search shows that this is the second reported case of a Marfan patient presenting with symptomatic spontaneous CSF leaks along with tonsillar herniation.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Blood Patch, Epidural
  • Brain / pathology
  • Cerebrospinal Fluid
  • Contrast Media
  • Dura Mater / pathology*
  • Encephalocele / etiology*
  • Encephalocele / pathology
  • Encephalocele / surgery
  • Follow-Up Studies
  • Humans
  • Intracranial Hypotension / etiology*
  • Intracranial Hypotension / pathology
  • Intracranial Hypotension / surgery
  • Magnetic Resonance Imaging / methods
  • Male
  • Marfan Syndrome / complications*
  • Spinal Cord / pathology
  • Transplantation, Autologous
  • Treatment Outcome

Substances

  • Contrast Media