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

Outline Introduction Clinical presentation Laboratory and Image studies Diagnostic criteria Treatment

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

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)

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

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

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

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.

Differential Diagnosis

Laboratory Studies a diagnosis of exclusion requiring careful patient evaluation (J. Neurol. Neurosurg. Psychiatry 2001; 71; 577-582)

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

MR Imaging (Headache 1999; 39: 321-325)

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

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) 209-217

Dynamic and Conventional MR

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.

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.

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.

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”

Medical Treatment Corticosteroid (treatment of choice)  60-120 mg/day for 7-10 days, then taper…  Significant pain relief: within 24-72 hr of therapy initiation  Ophthalmoparesis: weeks to months for resolution For refractory cases: - Azathioprine, methotrexate, radiation therapy

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; 371-377

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References www.emedicine.com/neuro/topic373.htm Nosological entities? The Tolosa-Hunt syndrome. J. Neurol. Neurosurg. Psychiatry. 2001; 71; 577-582 The criteria of the International Headache Society for Tolosa-Hunt syndrome need to be revised. J. Neurol. (1999) 246; 371-377 Neuroimaging diagnosis of Tolosa-Hunt syndrome: MRI contribution. Headache 1999; 39; 321-325 Dynamic MR imaging in Tolosa-Hunt syndrome. Eur. J. Radiol. 51(2004) 209-217