Tolosa-Hunt syndrome (THS) is an uncommon diagnosis with an incidence\nof nearly 1 to 2 cases per million hallmarked by the presence of painful ophthalmoplegia\n(PO) due to a granulomatous inflammation (GI). Diagnostically,\nthe major THS challenges encountered are owing to the exclusion of other\nGI presenting conditions necessitating multi-specialization consultations.\nThis article presents uniquely advances in diagnosis and challenges encountered\nattempting to exclude THS mimics, details on physical examination and\nlaboratory investigations have been incorporated. Tolosa Hunt MRI protocol\n(contrast-enhanced MRI), restricted diffusion and CISS MRI have lately\nproved to be precise investigations for THS diagnosis and follow up, on the\ncontrary, number of false-negative/positive MRI diagnoses appears to be rising,\nhence proposed that MRI or biopsy shouldnâ??t be mandatory criteria for\ndiagnosis as opposed to IHS 2018 guidelines. Despite corticosteroids being\nthe cornerstone therapy for THS, there are controversies concerning the better\nadministration route, optimal dosage, and therapy longevity, through case\nreports, high dose IV mPSL of 500 mg BID for 3 days, followed with maintenance\ndose of prednisone 60 mg resulted in the earliest recovery, hereafter\nstandardized guidelines are required. Alternatively, infliximab (300 mg infusion),\nazathioprine, methotrexate, and acupuncture can be used, farther studies\nare required to appraise the effectiveness and their safety. On the other\nside, microsurgery can be utilized for GI evacuation however associated risks\nof permanent CN VI palsy have been stated, nonetheless, GKRS can be employed\nwhen contraindication or corticosteroid intolerability exists though\nthe hazard of late malignancy is a drawback.
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