Tolosa-Hunt Syndrome

A 19-year-old Dominican woman presented to the emergency department with nausea, vomiting, and a right-sided headache of 1 month’s duration. The nausea and vomiting were associated with anorexia and a 20-lb weight loss since the symptoms began. Within the past 2 weeks, blurry vision had developed and the patient noted that it was difficult to keep her right eye open. Her past medical history and family history were noncontributory.

The patient was afebrile but appeared frail. Blood pressure was 111/62 mm Hg. Ptosis and complete ophthalmoplegia were noted in the right eye. Extraocular motion was intact in her left eye, and both pupils were equally reactive to light. There were no other pertinent physical findings or abnormal laboratory results.

Cerebrospinal fluid (CSF) cytology analysis was negative, and CSF-VDRL was nonreactive. A rapid plasma reagin test and an HIV enzyme immunoassay were negative. In addition, both the C-reactive protein level and the erythrocyte sedimentation rate were normal.

A CT angiogram of the head showed no evidence of aneurysm or vessel occlusion. However, an MRI scan of the brain revealed leptomeningeal enhancement along the floor of the right middle cranial fossa, with extension into the right orbital apex and into the right foramen ovale along a portion of the trigeminal nerve. This was thought to be consistent with sarcoidosis or lymphoma. However, subsequent CT scans of the chest, abdomen, and pelvis were negative for malignancy.

Four days later, the patient began empiric prednisone therapy (60 mg/d of intravenous methylprednisolone) for suspected Tolosa-Hunt syndrome (THS). One day after treatment was started, a right cranial nerve palsy developed. Within 2 days, the headache, nausea, and vomiting had resolved. Three days after beginning prednisone therapy, the patient regained some degree of extraocular movement in her right eye and was able to partially open the eye. In addition, her right-sided facial droop seemed to have improved. A repeat brain MRI scan 4 days later showed regression of the leptomeningeal enhancement—and the patient continued to improve clinically.

THS can affect persons of all ages from the first to the eighth decades of life without sex predilection; its etiology remains largely unknown.¹ The patient classically presents with a painful ophthalmoplegia, characterized by recurrent unilateral orbital pain and ipsilateral oculomotor paralysis.² All three ocular motor cranial nerves can be affected to various degrees, and other cranial nerves, such as the facial nerve, can also be affected.¹

THS causes a nonspecific chronic granulomatous inflammatory lesion that is often visible on high-resolution MRI studies within the cavernous sinus and/or the orbital apex.³ Because histologic proof is usually difficult and hazardous to obtain owing to the inaccessibility of the lesions and their close proximity to vital intracranial structures, some authorities suggest resolution of imaging abnormalities after systemic corticosteroid treatment to be “diagnostic” of THS.¹ However, it is important to note that THS remains a diagnosis of exclusion.

This patient was discharged from the hospital 2 weeks after her initial presentation, showing evidence of both clinical and radiologic improvement. She was discharged on a 40 mg/d regimen of oral prednisone, with a plan to taper the dosage over a course of several months. 

REFERENCES:

1.Kline LB, Hoyt WF. The Tolosa-Hunt syndrome. J Neurol Neurosurg Psychiatry. 2001;71:577-582.

2.La Mantia L, Curone M, Rapoport A, Bussone G. Tolosa-Hunt syndrome: critical literature review based on HIS 2004 criteria. Cephalalgia. 2006;26:772-781.

3.Jain R, Sawhney S, Koul R, Chand P. Tolosa-Hunt syndrome: MRI appearances. J Med Imaging Radiat Oncol. 2008; 52:447-451