This document discusses Tolosa-Hunt syndrome, which causes painful ophthalmoparesis. It presents the typical symptoms, which include severe retro-orbital or periorbital pain of acute onset followed by diplopia from ophthalmoparesis. MRI with contrast and labs are used to diagnose, while steroids are the primary treatment, with pain relief usually within 72 hours. The document outlines criteria to distinguish Tolosa-Hunt syndrome from other causes of painful ophthalmoparesis and conditions that should raise concern if not responding to steroids.

1. A Case of Painful
Ophthalmoplegia

2. Monocular ophthalmoparesis

3. Cavernous Sinus Localization
 Oculomotor paresis?
 Horner’s?
 Loss of corneal reflex?
ALL ENHANCE LOCALIZATION TO
THE CAVERNOUS SINUS

4. History in Patients with Tolosa Hunt
 Patients present with usually severe retro-orbital or periorbital pain of acute
onset. This pain may be described as constant and "boring" in nature.
 Diplopia related to ophthalmoparesis follows the onset of pain (in rare cases, the
ophthalmoparesis precedes the pain but this is unusual).
 Patients may report visual loss. This is noted if the inflammation extends into
the orbit to affect the optic nerve, and it is not a factor in disease limited to the
cavernous sinus.
 Paresthesias along the forehead may be described if the first division of the
trigeminal nerve is involved.
 Tolosa-Hunt syndrome is most often unilateral, although bilateral cases have
been described.

5. MRI with Contrast

6. Tolosa Hunt: Labs
 CBC
 Erythrocyte sedimentation rate (ESR)
 Thyroid function tests
 Fluorescent treponemal antibody (FTA),
 Antinuclear antibody (ANA)
 DS DNA
 Antineutrophil cytoplasmic antibody (ANCA),
 Serum protein electrophoresis and immunofixation
 Lyme titer
 Angiotensin-converting enzyme (ACE) level, and
 HIV titre

8. Treatment of Tolosa Hunt
 Steroids
 Substantial pain improvement within 72 hours
 Immunosupression for refractory cases
Azathioprine (Imuran), methotrexate, or radiation
therapy
 Ophthalmoparesis may take week to resolve and there are
some cases of permanent deficit

9. MRI Improvement After Steroid
Treatment

10. When To Worry
 ALWAYS!
 This is a diagnosis of exclusion!
 However……
 The ophthalmoplegia PRECEEDS the eye pain
 In THS ophthalmoplegia begins coincident with or after onset of pain
 Fever
 Significant proptosis
 Congestion of the sclera or chemosis
 No response to steroids after 72 hours
 Bilateral onset
 Hemicranial rather than orbital pain

11. The “A”s of Life Threatening Painful Ophthalmoplegia
 Acute headache in the elderly with visual loss
 Arteritis (Temporal arteritis)
 Acute orbital apex syndrome in poorly controlled DM
 Abscess (especially mucormycosis)
 Acute painful anisocoria suggesting 3rd nerve palsy
 Aneurysm (posterior communicating)
 Painful small pupil (Horner’s)
 Arterial dissection
 Acute bitemporal visual loss
 Apoplexy (Pituitary )

12. Causes of Painful Opthalmoplegia…(but
wait!...there’s more!)

13. Causes of Painful Ophthalmoplegia