2023 American Academy of Neurology Abstract Website

Objective:

Tolosa Hunt Syndrome (THS) is an idiopathic inflammatory condition presenting with multiple unilateral cranial nerve deficits and pain typically localized to the septa and cavernous sinus wall. The differential can be broad, oftentimes requiring extensive workup. This case presentation offers a streamlined approach to diagnosis and treatment while providing meaningful background knowledge.

Background:

THS is rare, its pathophysiology involves granulomatous deposition along epithelioid and giant cells leading to inflammation of affected tissues. Its dramatic presentation and clinical features can often be narrowed down to vascular, neoplastic, or inflammatory conditions, which could be differentiated by biopsy.

Design/Methods:

N/A

Results:

A 27-year-old male presented with Left eye pain, ptosis, ophthalmoplegia, facial paresthesias, and unrelenting severe headaches. He was previously seen and treated for meningoencephalitis after neuroimaging revealed right frontal dural enhancement, but was discharged after a negative infectious workup. Symptoms progressed with examination now revealing complete cranial nerve III, IV, V1, V2, and VI palsies.

MRI/MRA/MRV revealed pachymeningeal enhancement and thickening of the left middle fossa, cavernous sinus, tentorium, and orbital apex, but no vascular abnormalities. CT chest/abdomen/pelvis revealed an indeterminate renal lobulation, Ophthalmologic exam was normal, and CSF studies were benign. A left middle cranial fossa biopsy later revealed aggregates of inflammatory histiocytes, T-cells, plasma and B-cells clustered around nerves and inside endoneurium. ACE levels later returned normal.

Conclusions:

He underwent a 5-day course of high-dose steroids, followed by a prolonged oral taper. On day two, extraocular movements, ptosis, and pain were improved. By two months, he had ameliorated cranial nerve function and reduction in dural enhancement and thickening.

While most cases of THS are non-relapsing and responsive to steroids, some cases require second-line systemic immunosuppressants or focal radiation, both of which require a biopsy-proven diagnosis. Should location be amenable, we recommend pursuing early biopsy, with subsequent high-dose glucocorticoid treatment for timely diagnosis and treatment.