Tolosa-Hunt syndrome (THS) is an uncommon diagnosis with an incidence of nearly 1 to 2 cases per million hallmarked by the presence of painful ophthalmoplegia (PO) due to a granulomatous inflammation (GI). Diagnostically, the major THS challenges encountered are owing to the exclusion of other GI presenting conditions necessitating multi-specialization consultations. This article presents uniquely advances in diagnosis and challenges encountered attempting to exclude THS mimics, details on physical examination and laboratory investigations have been incorporated. Tolosa Hunt MRI protocol (contrast-enhanced MRI), restricted diffusion and CISS MRI have lately proved to be precise investigations for THS diagnosis and follow up, on the contrary, number of false-negative/positive MRI diagnoses appears to be rising, hence proposed that MRI or biopsy shouldn’t be mandatory criteria for diagnosis as opposed to IHS 2018 guidelines. Despite corticosteroids being the cornerstone therapy for THS, there are controversies concerning the better administration route, optimal dosage, and therapy longevity, through case reports, high dose IV mPSL of 500 mg BID for 3 days, followed with maintenance dose of prednisone 60 mg resulted in the earliest recovery, hereafter standardized guidelines are required. Alternatively, infliximab (300 mg infusion), azathioprine, methotrexate, and acupuncture can be used, farther studies are required to appraise the effectiveness and their safety. On the other side, microsurgery can be utilized for GI evacuation however associated risks of permanent CN VI palsy have been stated, nonetheless, GKRS can be employed when contraindication or corticosteroid intolerability exists though the hazard of late malignancy is a drawback.
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