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Ocular System

Tolosa Hunt syndrome (THS)

Severe and unilateral periorbital headache associated with painful and restricted eye movements.

Severe and unilateral periorbital headache associated with painful and restricted eye movements.

History:

In his initial report, Dr. Eduardo Tolosa described the pathophysiology as “non-specific, chronic inflammation of the septa and wall of the cavernous sinus with the proliferation of fibroblasts an infiltration with lymphocytes and plasma cells.” Hunt et al. not only agreed with above observation but also added that “such inflammatory changes, in a tight connective tissue, may exert pressure upon the penetrating nerves.” These nerves can be cranial nerves III, IV, VI, as well as the superior division of the fifth cranial nerve. Further studies showed the presence of granulomatous material deposits along with epitheloid cells and giant cells. Occasionally necrosis has been seen, but it is rare. There is also associated thickening of dura mater within the cavernous sinus.


Aetiology

Idiopathic (M/C)

Thought to be from non-specific inflammation in the region of the cavernous sinus and/or superior orbital fissure

Potential triggers:

  • Traumatic injury
  • Tumors
  • Aneurysm

Clinical features

Pain tends to be the presenting symptom and can precede ophthalmoplegia by up to 30 days. Tolosa Hunt syndrome tends to have a relapsing and remitting course, with attacks recurring every few months or years.

Unilateral pain/headache (Hallmark feature):

Sharp, shooting, stabbing, boring, severe, and intense periorbital pain (can be retro-orbital, with extension into the frontal and temporal areas)

Painful ophthalmoplegia:

Acute ophthalmoplegia with/without involvement of optic nerve and ophthalmic division of trigeminal nerve
  • Oculomotor nerve (CN III) (M/C involved, 80%)
  • Abducens nerve (70%)
  • Ophthalmic branch of trigeminal nerve (approximately 30%)
  • Trochlear nerve (29%)

Pupillary abnormalities:

  • Sympathetic (third order neuron Horner syndrome) (20% cases)
  • Parasympathetic (oculomotor) involvement

Diagnosis

Tolosa Hunt syndrome is diagnosed through the clinical presentation, neuroimaging studies, and response to steroids.

MRI:

Better visualization of the region of the cavernous sinus and/or superior orbital fissure
  • Thickening of the cavernous sinus (because of the presence of abnormal soft tissue which is isointense on T1, iso or hypointense on T2, and enhances with contrast)
  • Convexity of the lateral wall of the cavernous sinus
  • Extension into the orbital apex
(a) Postcontrast T1 axial scan shows thickening and enhancement of the lateral dural wall of the left cavernous sinus with extension up to the left orbital apex. (b) Follow-up post contrast T1 scan shows resolution of the lesion | Arthur, A., Sivadasan, A., Mannam, P., Prabakhar, A. T., Aaron, S., Mathew, V., Karthik, M., Benjamin, R. N., Iqbalahmed, S. A., Rynjah, G. L., & Alexander, M. (2020). Tolosa-Hunt Syndrome: Long-Term Outcome and Role of Steroid-Sparing Agents. Annals of Indian Academy of Neurology, 23(2), 201–205. https://doi.org/10.4103/aian.AIAN_368_18

Glucocorticoid response:

Treating with high doses of systemic steroids leads to a dramatic improvement in pain within 2-3 days
  • Cranial nerve dysfunction improves
  • Reduction in abnormal tissue volume as well as signal intensity on MRI

Management

Glucocorticoids:

Mainstay of treatment

Immunosuppressive treatment:

A very small percentage of patients will require immunosuppression with other agents, either to avoid side effects of long-term steroid therapy or for long-term suppression of the disease process itself
  • Azathioprine, methotrexate, mycophenolate mofetil, cyclosporine, and infliximab

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