Tolosa–Hunt Syndrome: Case Report

Introduction: Tolosa–Hunt syndrome is an idiopathic granulomatous inflammation of the cavernous sinus, superior orbital fissure, or orbit, and is primarily a diagnosis of exclusion. The majority of patients present with unilateral orbital or periorbital pain associated with paresis of one or more of the III, IV, and/or VI cranial nerves. Tolosa–Hunt syndrome is a very rare diagnosis. Was first classified by the International Headache Society in 2004 and now is a part of Classification ICHD-3. Physicians should rule out the more common causes of cavernous sinus syndrome, including cavernous sinus thrombosis, intraorbital or intraparenchymal tumors, vasculitis, basal meningitis, systemic granulomatous diseases such as sarcoidosis and tuberculosis, diabetes mellitus or more recent diagnosis like IgG4-related disease. MRI of the head may show unilateral enhancement of the cavernous sinus and orbital apex. For accuracy diagnosis, endoscopic transnasal image-guided approach biopsy to orbital apex, optic canal or pterygopalatine fossa lesions can be realized by an ENT surgeon. High-dose glucocorticoids are the first line treatment considering its inflammatory pathology, initially with high-dose intravenous steroids followed by tapering oral steroids. There is not sufficient evidence for the appropriate dose, route of administration and duration of therapy. For steroid-resistant patients, antimetabolic agents such as methotrexate, infliximab, and mycophenolate mofetil have also been shown to cause dramatic improvement.

Materials and Methods: A 30-year-old previously healthy man presented with a 2-month history of moderate to severe pain in cheek and retroocular left side, sharp and continuous. This was associated with subsequent development of left horizontal diplopia, without vomiting, dizziness, loss of consciousness or limb weakness 24-hour previously. He was afebrile, alert, conscious, and oriented to time, place and person. Examination of the left eye revealed partial ptosis, left horizontal diplopia, normal visual acuity and visual field. The rest of the neurological and systemic examination was unremarkable. CT was normal and MRI showed enhancement of the left cavernous sinus, orbital apex, inferior part of Meckel's cave, V2 and left pterigopalatine fossa. Transnasal endoscopic biopsy was performed to rule out malignancy from pterygopalatine fossa. Absence of adipose tissue with intense fibrosis was observed. Pathological reports show inflammatory and sclerosing lesions, with fusiform cells without atypia and granulomatous tissue without signs of malignancy that did not fulfill the criteria of IgG4-related disease. Hematological malignancy was excluded. Postoperative day 9, severe epistaxis was observed and angiography embolization on left maxillary artery with coils was performed. High-dose intravenous steroids followed by tapering oral steroids were performed with resolution of facial pain and diplopia. One year after diagnostic, the patient still continues asymptomatic.

Discussion/Conclusions: Tolosa–Hunt syndrome is a diagnosis of exclusion. Neurologists should rule out the more common causes of cavernous sinus syndrome. ENT surgeon can play a role making differential diagnosis using endoscopic transnasal image-guided approach biopsy from affected tissues. Steroids have both diagnostic as well as therapeutic significance in Tolosa–Hunt syndrome. Patients should be followed-up regularly to ensure resolution of symptoms and signs, and also for early detection of relapse, which happens in nearly 50% of the patients.