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Tolosa-Hunt Syndrome: A Diagnostic Challenge and the Complexities of Long-Term Corticosteroid Management
Guilherme Henriques1, Margarida Pistola1, Cláudia Resende1
1Family Medicine, Unidade Local de Saúde da Lezíria, Santarém, PRT.
Abstract:
Tolosa-Hunt syndrome (THS) is an uncommon cause of painful ophthalmoplegia resulting from idiopathic granulomatous inflammation involving the cavernous sinus, superior orbital fissure, or orbit. Its diagnosis remains challenging because early manifestations may be nonspecific and its clinical and radiological features overlap with several vascular, infectious, inflammatory, and neoplastic conditions. We report the case of a 59-year-old woman with type 2 diabetes mellitus, arterial hypertension, dyslipidemia, and post-thyroidectomy hypothyroidism who initially presented to her family physician with retro-orbital headache and blurred vision, with no focal neurological abnormalities on examination. Her symptoms subsequently progressed to diplopia, complete right-sided ptosis and ophthalmoplegia, and hypoesthesia involving the ophthalmic and maxillary divisions of the right trigeminal nerve. MRI demonstrated focal thickening and gadolinium enhancement of the right cavernous sinus together with right extraocular muscle involvement. Following an extensive investigation that did not identify a more compelling infectious, vascular, autoimmune, neoplastic, or neuromuscular etiology, THS was considered the most likely diagnosis. High-dose IV methylprednisolone resulted in rapid resolution of retro-orbital pain and progressive recovery of ptosis and ocular motility, with subsequent radiological improvement. However, prolonged corticosteroid therapy, continued for approximately one year, was associated with significant deterioration of glycemic control requiring insulin treatment and intensification of antihypertensive therapy. This case illustrates both the diagnostic complexity of THS and the importance of longitudinal care beyond neurological recovery. Close collaboration between primary and secondary care was essential for monitoring clinical evolution, managing corticosteroid-related complications, optimizing chronic comorbidities, and coordinating long-term multidisciplinary follow-up.
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