Orbitaspitzensyndrom

Tolosa-Hunt

syndrome • Tolosa Hunt syndrome - Ganzer Fall bei Radiopaedia

Balachandran, G. Tolosa Hunt syndrome. Case study, Radiopaedia.org. (accessed on 20 Oct 2022) https://doi.org/10.53347/rID-10627 • CC by-nc-sa 3.0 (modified)

Tolosa-Hunt

syndrome • Tolosa-Hunt syndrome - Ganzer Fall bei Radiopaedia

Ibrahim, D. Tolosa-Hunt syndrome. Case study, Radiopaedia.org. (accessed on 20 Oct 2022) https://doi.org/10.53347/rID-29642 • CC by-nc-sa 3.0 (modified)

Tolosa-Hunt

syndrome • Tolosa-Hunt syndrome - Ganzer Fall bei Radiopaedia

Ma, W. Tolosa-Hunt syndrome. Case study, Radiopaedia.org. (accessed on 20 Oct 2022) https://doi.org/10.53347/rID-66643 • CC by-nc-sa 3.0 (modified)

Tolosa-Hunt

syndrome • Tolosa-Hunt syndrome - Ganzer Fall bei Radiopaedia

Harbawi, B. Tolosa-Hunt syndrome. Case study, Radiopaedia.org. (accessed on 20 Oct 2022) https://doi.org/10.53347/rID-75426 • CC by-nc-sa 3.0 (modified)

Tolosa-Hunt

syndrome • Tolosa-Hunt syndrome - Ganzer Fall bei Radiopaedia

Botz, B. Tolosa-Hunt syndrome. Case study, Radiopaedia.org. (accessed on 20 Oct 2022) https://doi.org/10.53347/rID-92579 • CC by-nc-sa 3.0 (modified)

Tolosa-Hunt

syndrome • Tolosa-Hunt syndrome - Ganzer Fall bei Radiopaedia

Ayodele, A. Tolosa-Hunt syndrome. Case study, Radiopaedia.org. (accessed on 20 Oct 2022) https://doi.org/10.53347/rID-154211 • CC by-nc-sa 3.0 (modified)

Cavernous

sinus mass • Tolosa-Hunt syndrome - Ganzer Fall bei Radiopaedia

Ibrahim, D. Tolosa-Hunt syndrome. Case study, Radiopaedia.org. (accessed on 22 Oct 2022) https://doi.org/10.53347/rID-29642 • CC by-nc-sa 3.0 (modified)

Tolosa-Hunt

syndrome • Tolosa-Hunt syndrome - Ganzer Fall bei Radiopaedia

Gaillard F, Tolosa-Hunt syndrome. Case study, Radiopaedia.org (Accessed on 27 Nov 2022) https://doi.org/10.53347/rID-150349 • CC by-nc-sa 3.0 (modified)

Imaging of

cranial nerves: a pictorial overview. Tolosa-Hunt syndrome. MRI T1-weighted post-gadolinium axial image (a, and enlargement in b) shows enhancing inflammatory infiltration in the left orbital apex extending into the cavernous sinus (empty arrows)

insightsimaging.springeropen.com • CC-by-4.0

Radiopaedia • CC-by-nc-sa 3.0 • de

Tolosa-Hunt syndrome (THS) is an idiopathic inflammatory condition that involves the cavernous sinus and orbital apex and is essentially a clinical diagnosis of exclusion.

Epidemiology

The estimated incidence of Tolosa-Hunt syndrome is 1 per 1,000,000 person years with an average age of onset at 41 years .

Associations

inflammatory myofibroblastic tumor (IMT)
idiopathic hypertrophic pachymeningitis (IHP)

Clinical presentation

Clinically it refers to the presence of a painful ophthalmoplegia secondary to surrounding cavernous sinus inflammation. Tolosa-Hunt syndrome is essentially a clinical diagnosis of exclusion.

Pathology

The constant pain that characterizes the disorder is due to infiltration of lymphocytes and plasma cells, along with thickening of dura mater within the cavernous sinus.

Radiographic features

CT

May show asymmetrical enlargement in the region of the cavernous sinus on the affected side +/- contrast enhancement .

The secondary criteria are internal carotid artery narrowing, extension towards the superior orbital fissure and orbital apex.

MRI

May show evidence of inflammatory changes in the region of the anterior cavernous sinus, superior orbital fissure +/- orbital apex. Signal characteristics are non-specific (clinical scenario essential to diagnosis) but may include:

T1: involved region is isointense to hyperintense compared with muscle
T2: involved area is hyperintense
T1 C+ (Gd): may show contrast enhancement during active phase with resolution of enhancement following treatment

Treatment and prognosis

The condition is often successfully amenable to steroid treatment.

History and etymology

It was initially described by Eduardo Tolosa Colomer (1900-1981) , a Spanish neurosurgeon, in 1954 (note his son is a pioneering neurologist with a similar name: Eduardo Tolosa Sarró (fl. 2019)) and then by the American neurosurgeon William Edward Hunt (1921-1999) et al. in 1961 . The disease is also known as painful opthalmoplegia.

Differential diagnosis

Consider other pathological processes presenting with similar clinical features such as meningioma, sarcoidosis, pituitary tumors, tuberculous meningitis, lymphoma, cavernous sinus thrombosis .

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