Tolosa Hunt Syndrome: Current Diagnostic Challenges and Treatment

Abstract

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.

Disappearing diplopia - An unusual presentation of Tolosa-Hunt syndrome: A case report

“…Rapid resolution of symptoms within 1-3 days of steroid administration can also be used in the diagnosis [1,2]. We were unable perform biopsy in our patient, but this has been the case in many reported cases [9]. In case of a fungal infection steroids could be detrimental and a biopsy may be beneficial.…”

“…Steroids may have a place in altering the natural course of the disease, although conclusive evidence of the exact effect is unavailable [5]. Some cases have used intravenous methyl prednisolone in patients whereas others have preferred oral high dose steroids [9]. We commenced our patient on oral prednisolone and the patient achieved complete clinical resolution.…”

Azathioptrine in Refractory Tolosa-Hunt Syndrome: Two Case Report

“…In refractory cases, azathioprine, methotrexate, or radiation therapy may be considered for partial response to steroid treatment. 4,10,11 The use of immunotherapy to prevent recurrences is still controversial. Oral azathioprine 50 mg twice daily (2 mg/kg/day) was given with close monitoring of the complete blood count, liver, and renal function tests.…”

“…Other immunosuppressants such as azathioprine, methotrexate, or radiation therapy may be employed in refractory cases or in patients not tolerating steroid therapy. 2,4 As a diagnosis of elimination, THS should be carefully distinguished from more malignant causes of cavernous sinus involvement and painful ophthalmoplegia. At this moment, we present two case reports of successful refractory THS with azathioprine as the second-line treatment option.…”