1. Intern 趙若雁 2005.11.29. Supervisor: Dr. 俞芹英
Tolosa-Hunt Syndrome
Intern 趙若雁
Supervisor: Dr. 俞芹英

2. Outline Introduction Clinical presentation
Laboratory and Image studies
Diagnostic criteria
Treatment

3. Tolosa-Hunt syndrome
Painful ophthalmoplegia caused by nonspecific inflammation of the cavernous sinus or superior orbital fissure.

4. Pathophysiology
Nonspecific inflammation (noncaseating granulomatous or nongranulomatous) within the cavernous sinus or superior orbital fissure
Pain
Ophthalmoplegia (Cranial nerve III, IV, VI)
Pupillary dysfunction
Trigeminal nerve involvement (V1)

5. Epidemiology Incidence: uncommon No gender predilection
Age: rare during the first 2 decades of life

6. Clinical Presentation
History~
Severe retro-orbital or periorbital pain of acute onset, boring and constant in nature
Diplopia follows the onset of pain
Patient may have visual loss
Paresthesia along the forehead
May be self-limited, relapsing-remitting
Most often unilateral

7. Clinical Presentation
Physical exam~
Painful ophthalmoparesis or ophthalmoplegia
Evidence of ocular motor nerve palsies (III, IV, VI)
Loss of corneal reflex (V1 involvement).
Ptosis
Horner syndrome

8. International Headache Society criteria for THS
Episode(s) of unilateral orbital pain for an average of 8 weeks if untreated
Associated paresis of the 3rd, 4th or 6th cranial nerves, which may coincide the onset of pain or follow it by a period of up to 2 weeks
Pain is relieved within 48 hours of steroid therapy initiation. Exclusion of other conditions by neuroimaging and (not compulsory) angiography.

9. Differential Diagnosis

11. Laboratory Studies
a diagnosis of exclusion requiring careful patient evaluation
(J. Neurol. Neurosurg. Psychiatry
2001; 71; )

12. Image studies Contrast-enhanced MR imaging HRCT Cerebral angiography
- narrowing of the intracavernous portion of internal carotid artery
Phlebography

14. MR Imaging
(Headache 1999; 39: )

15. MR Imaging
Convex enlargement of the symptomatic cavernous sinus by an abnormal tissue isointense with gray matter on short TR/TE images and iso-hypointense on long TR/TE images
This abnormal tissues markedly increase in signal intensity after contrast injection and extend into contiguous regions, mainly the orbital apex and subtemporal fossa ipsilaterally

16. Conventional and Dynamic MR
5 patients with THS and 12 control subjects
Dynamic MRI in coronal planes
Dynamic images: Spine-echo (SE) sequences in 3 patients and fast spine-echo (FSE) in 2 patients
Conventional MR images: T1W pre- and post-contrast SE, T2W FSE sequences
European Journal of Radiology 51(2004)

17. Dynamic and Conventional MR

18. Conventional MRI
A 38-year-old patient with left Tolosa–Hunt syndrome (THS), T1-weighted coronal (a) pre-contrast (400/20) and (b) post-contrast (500/30) images show enlarged left cavernous sinus with outer bulging of the lateral wall.
The signal intensity and contrast enhancement of the left affected cavernous sinus are similar to that of the right unaffected cavernous sinus.

19. Dynamic MR Imaging
Comparing with the right unaffected cavernous sinus, (c) the dynamic coronal (500/14.2) images show an area along the inferior-lateral wall (arrowheads) of the left cavernous sinus, which enhance very slowly and gradually from the early (obtained at 15 s) to the late (obtained at 75 s) images.

20. Follow-up MR images after steroid therapy, the left cavernous sinus appears normal on T1W coronal (d) pre-contrast (400/14) and (e) post-contrast (400/20) images.
(f) The dynamic coronal images (500/14.2) show no residual gradually enhancing area along the lateral wall of the left previously affected cavernous sinus.

22. Conventional and Dynamic MR
Conventional MRI:
Enlarged cavernous sinus (CS) with abnormal soft tissue
Post-contrast images:
 marked enhancement of the enlarged CS
 But no exact demarcation depicted between normal and abnormal tissues
Dynamic MRI:
Better demarcation of normal structures within the venous spaces of the CS
Small slowly and gradually enhancing areas from the early to the subsequent dynamic images adjacent to the dark filling defects of the cranial nerves
 Consistent with the pathologic processes ”granulomatous inflammation”

23. Medical Treatment Corticosteroid (treatment of choice)
 mg/day for 7-10 days, then taper…
 Significant pain relief: within hr of therapy initiation
 Ophthalmoparesis: weeks to months for resolution
For refractory cases:
- Azathioprine, methotrexate, radiation therapy

24. Follow-up
Positive MRI or CT remain suspect until a malignant tumor or inflammation other than THS is excluded by biopsy or follow-up examination(s).
Clinical and radiological follow-up examination performed for at least 2 years.
J Neurol (1999) 246;

25. Thanks for Your Attention

26. References www.emedicine.com/neuro/topic373.htm
Nosological entities? The Tolosa-Hunt syndrome. J. Neurol. Neurosurg. Psychiatry. 2001; 71;
The criteria of the International Headache Society for Tolosa-Hunt syndrome need to be revised. J. Neurol. (1999) 246;
Neuroimaging diagnosis of Tolosa-Hunt syndrome: MRI contribution. Headache 1999; 39;
Dynamic MR imaging in Tolosa-Hunt syndrome. Eur. J. Radiol. 51(2004)