Tolosa-Hunt Syndrome: Clinical Manifestations in Children

Pediatr Neurol. 2019 Oct:99:60-63. doi: 10.1016/j.pediatrneurol.2019.02.013. Epub 2019 Feb 20.


Background: Tolosa-Hunt syndrome (THS) is a rare condition in children characterized by painful ophthalmoplegia caused by inflammation of unknown etiology in the cavernous sinus, superior orbital fissure, or orbital apex. Our main purpose was to report two pediatric cases of THS, a typical one and another extremely rare one preceded by facial palsy.

Methods: Both cases were diagnosed with THS based on the 2013 International Classification of Headache Disorders (ICHD-3 beta) criteria. A literature review was also performed concerning epidemiology, clinical and imaging features, diagnostic criteria, treatment, and outcome of THS with a focus on children.

Results: The first patient was a 14-year-old boy who presented with third nerve palsy, four weeks after the diagnosis and treatment of peripheral seventh nerve palsy and the second patient was a 10-year-old girl who presented with a five-day history left-sided ptosis, periorbital pain, and diplopia.

Conclusions: THS is a rare condition in pediatric population that requires an extensive evaluation before the final diagnosis is made. Seventh nerve palsy preceding the diagnosis of THS is particularly rare in children. This patient represents only the second reported example of seventh nerve involvement in a child with THS.

Keywords: Painful ophthalmoplegia; Pediatric; Tolosa-Hunt syndrome; Tolosa-Hunt syndrome seventh nerve paresis.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adolescent
  • Anti-Inflammatory Agents / therapeutic use
  • Blepharoptosis / etiology
  • Cavernous Sinus / diagnostic imaging
  • Cavernous Sinus / pathology
  • Child
  • Diagnosis, Differential
  • Diplopia / etiology
  • Facial Paralysis / etiology
  • Female
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Methylprednisolone / therapeutic use
  • Neuroimaging
  • Oculomotor Nerve Diseases / etiology
  • Tolosa-Hunt Syndrome / complications
  • Tolosa-Hunt Syndrome / diagnosis*
  • Tolosa-Hunt Syndrome / drug therapy
  • Tomography, X-Ray Computed
  • Vomiting / etiology


  • Anti-Inflammatory Agents
  • Methylprednisolone

Supplementary concepts

  • Ophthalmoplegia, painful