Tolosa-Hunt syndrome: an arcane pathology of cavernous venous sinus

BMJ Case Rep. 2015 Aug 20:2015:bcr2015210646. doi: 10.1136/bcr-2015-210646.

Abstract

Tolosa-Hunt syndrome, an idiopathic granulomatous inflammation of the cavernous sinus, is primarily a diagnosis of exclusion. The majority of patients present with unilateral orbital pain and features suggestive of paralysis of one or more of the cranial nerves passing through the cavernous sinus and/or superior orbital fissure. MRI of the head may show unilateral enhancement of the cavernous sinus and orbital apex. Treatment is with high-dose intravenous steroids followed by tapering oral steroids. Rapid amelioration of pain within 24-48 h supports this rare diagnosis. Resolution of neuropathies may take longer. We describe a case of a young man who presented with left periorbital pain, complete ophthalmoplaegia and ptosis of the left eye. MRI showed enhancement of the left cavernous sinus and orbital apex. High dose steroids led to complete resolution of pain, while ptosis and ophthalmoplaegia improved gradually.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Blepharoptosis / diagnosis*
  • Blepharoptosis / drug therapy
  • Blepharoptosis / etiology
  • Cavernous Sinus / pathology*
  • Cranial Nerves / pathology*
  • Diagnosis, Differential
  • Facial Pain / diagnosis*
  • Facial Pain / drug therapy
  • Facial Pain / etiology
  • Humans
  • Inflammation / diagnosis
  • Inflammation / drug therapy
  • Inflammation / etiology
  • Magnetic Resonance Imaging
  • Male
  • Ophthalmoplegia / diagnosis*
  • Ophthalmoplegia / drug therapy
  • Ophthalmoplegia / etiology
  • Orbit / pathology
  • Steroids / therapeutic use*
  • Tolosa-Hunt Syndrome / complications
  • Tolosa-Hunt Syndrome / diagnosis*
  • Tolosa-Hunt Syndrome / pathology

Substances

  • Steroids