Objectives We present the first case of Tolosa-Hunt syndrome (THS) with internal carotid artery luminal narrowing resulting in cerebral infarcts. TSH is a nonspecific inflammatory disorder affecting the cavernous sinus (CS) and superior orbital fissure, which commonly presents with painful ophthalmoplegia, and resolves dramatically with steroids. Internal carotid artery (ICA) stenosis, although reported in THS, has not been associated with infarcts.
Case A 54-year-old male of South African origin presented with a 3 months history of worsening left sided frontal and retro-orbital headache, associated with lacrimation, conjunctival irritation and nasal symptoms. He was on treatment for possible cluster headache, however symptoms worsened and was followed by diplopia. There was no ptosis, anisocoria, facial sensory or motor involvement. MRI revealed a contrast enhancing left CS mass encasing the left anterior clinoid process and the cavernous, para-clinoid and supra-clinoid portions of the ICA with high grade luminal narrowing of the ICA. CSF examination showed mildly elevated protein with no cells and negative TB, fungal and viral studies and normal cell cytology. A biopsy was not performed due to procedural risks. Interval MRI revealed punctate acute infarcts in the left postcentral gyrus and left frontal centrum semiovale. High dose oral predinsiolone was commenced and associated with near immediate symptomatic improvement. Further MRI revealed reduction in degree of ICA stenosis.
Conclusions TSH is a rare cause of ICA stenosis but has not been reported to result in ischaemic strokes. The presumed mechanism of stenosis is due to non-specific periarteritic granuloma and is generally responsive to steroid therapy. The mechanism for stroke in this patient is presumably haemodynamic and this case illustrates that THS should be recognised as a potentially reversible cause for stroke in the appropriate clinical context.
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