Validation of ICHD-3 beta diagnostic criteria for 13.7 Tolosa-Hunt syndrome: Analysis of 77 cases of painful ophthalmoplegia

Cephalalgia. 2014 Jul;34(8):624-32. doi: 10.1177/0333102413520082. Epub 2014 Jan 29.


Background: Three editions of International Classification of Headache Disorders (ICHD) diagnostic criteria for Tolosa-Hunt syndrome (THS) have been published in 1988, 2004 and 2013, in ICHD-3 beta, there have been considerable changes [corrected]. The validity of these new diagnostic criteria remains to be established.

Methods: We retrospectively identified 77 patients with non-traumatic painful ophthalmoplegia (PO) admitted between 2003 and 2013. We reviewed patients' age at onset and gender, time courses between onset of pain and development of cranial nerve palsy, the cranial nerves involved, imaging findings, therapeutic efficacy of steroid treatment and recurrence of attacks.

Results: THS was the most frequent type of PO (46/77). In THS patients, the third cranial nerve was most commonly involved (76.3%). The median time interval between pain and cranial nerve palsy was two days, although in five patients (10.9%) the interval ranged from 16 to 30 days. Definitely abnormal MRI findings were found in 24 patients (52.2%).

Conclusions: It is essential to rule out other causes of PO in diagnosing THS, with MRI playing a crucial role in differential diagnosis. It may be helpful to understand and master the entity of THS for researchers and clinicians to adjust the gradation and ranking of the diagnostic criteria.

Keywords: ICHD-3 beta; Painful ophthalmoplegia; Tolosa-Hunt syndrome; diagnostic criteria.

Publication types

  • Validation Study

MeSH terms

  • Adult
  • Aged
  • Dexamethasone / therapeutic use
  • Diagnosis, Differential
  • Female
  • Humans
  • Image Enhancement
  • International Classification of Diseases*
  • Magnetic Resonance Imaging
  • Male
  • Methylprednisolone / therapeutic use
  • Middle Aged
  • Ophthalmoplegia / classification*
  • Ophthalmoplegia / diagnosis*
  • Ophthalmoplegia / drug therapy
  • Ophthalmoplegia / etiology
  • Prednisone / therapeutic use
  • Retrospective Studies
  • Tolosa-Hunt Syndrome / classification*
  • Tolosa-Hunt Syndrome / diagnosis*
  • Tolosa-Hunt Syndrome / drug therapy
  • Tolosa-Hunt Syndrome / etiology
  • Treatment Outcome


  • Dexamethasone
  • Prednisone
  • Methylprednisolone