Citation, DOI and article data
The estimated incidence of Tolosa-Hunt syndrome is 1 per 1,000,000 person years with an average age of onset at 41 years 14.
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.
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.
May show asymmetrical enlargement in the region of the cavernous sinus on the affected side +/- contrast enhancement 1.
May show evidence of inflammatory changes in the region of the anterior cavernous sinus, superior orbital fissure +/- orbital apex. Signal characteristics are non-specific 10 (clinical scenario essential to diagnosis) but may include:
- T1: involved region is isointense 2 to hyperintense 8 compared with muscle
- T2: involved area is hyperintense
- T1 C+ (Gd): may show contrast enhancement during active phase with resolution of enhancement following treatment 5,9
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) 12, a Spanish neurosurgeon, in 1954 3 (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 4,12. The disease is also known as painful opthalmoplegia.
- 1. Kwan ES, Wolpert SM, Hedges TR et-al. Tolosa-Hunt syndrome revisited: not necessarily a diagnosis of exclusion. AJR Am J Roentgenol. 1988;150 (2): 413-8. AJR Am J Roentgenol (abstract) - Pubmed citation
- 2. Yousem DM, Atlas SW, Grossman RI et-al. MR imaging of Tolosa-Hunt syndrome. AJR Am J Roentgenol. 1990;154 (1): 167-70. AJR Am J Roentgenol (abstract) - Pubmed citation
- 3. Tolosa E. Periarteritic lesions of the carotid siphon with the clinical features of a carotid infraclinoidal aneurysm. J. Neurol. Neurosurg. Psychiatr. 1954;17 (4): 300-2. doi:10.1136/jnnp.17.4.300 - Free text at pubmed - Pubmed citation
- 4. Hunt WE, Meagher JN, Lefever HE et-al. Painful opthalmoplegia. Its relation to indolent inflammation of the carvernous sinus. Neurology. 1961;11 : 56-62. - Pubmed citation
- 5. Lee JH, Lee HK, Park JK et-al. Cavernous sinus syndrome: clinical features and differential diagnosis with MR imaging. AJR Am J Roentgenol. 2003;181 (2): 583-90. AJR Am J Roentgenol (full text) - Pubmed citation
- 6. Mckinney AM, Short J, Lucato L et-al. Inflammatory myofibroblastic tumor of the orbit with associated enhancement of the meninges and multiple cranial nerves. AJNR Am J Neuroradiol. 27 (10): 2217-20. AJNR Am J Neuroradiol (full text) - Pubmed citation
- 7. Zournas C, Trakadas S, Kapaki E et-al. Gadopentetate dimeglumine-enhanced MR in the diagnosis of the Tolosa-Hunt syndrome. AJNR Am J Neuroradiol. 1995;16 (4): 942-4. AJNR Am J Neuroradiol (abstract) - Pubmed citation
- 8. Goto Y, Hosokawa S, Goto I et-al. Abnormality in the cavernous sinus in three patients with Tolosa-Hunt syndrome: MRI and CT findings. J. Neurol. Neurosurg. Psychiatr. 1990;53 (3): 231-4. doi:10.1136/jnnp.53.3.231 - Free text at pubmed - Pubmed citation
- 9. Thomas DJ, Charlesworth MC, Afshar F et-al. Computerised axial tomography and magnetic resonance scanning in the Tolosa-Hunt syndrome. Br J Ophthalmol. 1988;72 (4): 299-302. Br J Ophthalmol (link) - Free text at pubmed - Pubmed citation
- 10. Saremi F, Helmy M, Farzin S et-al. MRI of cranial nerve enhancement. AJR Am J Roentgenol. 2005;185 (6): 1487-97. doi:10.2214/AJR.04.1518 - Pubmed citation
- 11. Yousem DM, Grossman RI. Neuroradiology, The Requisites. Mosby. (2010) ISBN:0323045219. Read it at Google Books - Find it at Amazon
- 12.Giménez-Roldán S. Spanish eponyms in clinical neurology. Neurosciences and History 2017;5 (1): 1-19. Direct link
- 13. Michael C. Plewa, Prasanna Tadi, Mohit Gupta. Cavernous Sinus Thrombosis. (2019) Pubmed
- 14. Amrutkar C, Burton EV. Tolosa-Hunt Syndrome. [Updated 2020 Aug 10]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK459225/