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Tolosa-Hunt syndrome
Other Resources UpToDate PubMed

Tolosa-Hunt syndrome

Contributors: Andrea Wasilewski MD, Richard L. Barbano MD, PhD
Other Resources UpToDate PubMed


Tolosa-Hunt syndrome is a rare, idiopathic granulomatous inflammation of the cavernous sinus, superior orbital fissure, or orbit that leads to unilateral headache and orbital pain with diplopia and ipsilateral paresis of cranial nerves III, IV, VI, and/or the superior branches of V. Occasionally, vision can be affected. Patients may present at any age. This condition is typically unilateral but can be bilateral in 5% of cases.

Symptoms are very responsive to glucocorticoids but tend to relapse and remit if left untreated. The syndrome can resolve spontaneously or result in permanent neurologic damage.


H49.40 – Progressive external opthalmoplegia, unspecified eye

95794005 – Tolosa-Hunt syndrome

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Cavernous sinus syndrome
  • Cavernous artery aneurysm or Cavernous sinus thrombosis
  • Carotid-cavernous fistula
  • Cerebral venous thrombosis (see Cerebral stroke)
  • Giant cell arteritis
  • Infection (eg, Aspergillosis, Mucormycosis, Tuberculosis, Herpes simplex virus)
  • Orbital trauma
  • Migraine headache
  • Idiopathic intracranial hypertension
  • Orbital tumor
  • Central nervous system Lymphoma
  • Meningioma
  • Brain metastases
  • Optic neuritis
  • Ischemic mononeuropathy
  • Diabetic ophthalmoplegia
  • Pituitary tumor
  • Craniopharyngioma
  • Sarcoidosis
  • Granulomatosis with polyangiitis
  • Thyroid disease
  • Myasthenia gravis

Best Tests

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Management Pearls

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Last Reviewed:07/25/2018
Last Updated:07/25/2018
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Tolosa-Hunt syndrome
A medical illustration showing key findings of Tolosa-Hunt syndrome : Fatigue, Headache, Diplopia, Eyelid ptosis, Proptosis, Unilateral retroorbital pain
Copyright © 2024 VisualDx®. All rights reserved.