Tolosa-Hunt syndrome is a rare neurological disorder with an incidence of one case per million. It is a granulomatous inflammatory condition that affects the cavernous sinus and is characterized by painful ophthalmoplegia and headaches. We present a 57-year-old male veteran with past medical history of alcohol use disorder, hepatitis c, hypertension and tobacco use who presented with complaints of double vision and headaches for 2 days. The patient also had a history of well controlled cluster headaches treated with sumatriptan. But, he reported that from several days prior to admission, the headaches were getting more frequent, sharp, localized to the left side, and preventing him from sleeping. On physical exam, vitals were stable, the neck was supple, the pupils were equal in size and reactive to light but limited left eye abduction and external rotation were noted. The remainder of physical exam was unremarkable including the remaining cranial nerves. Computerized tomography (CT) head was done which showed no evidence of acute stroke. Magnetic resonance imagining (MRI) brain was performed which showed an asymmetric bulge of the left cavernous sinus which raised suspicion for cavernous sinus inflammation. Systemic high dose steroid trial was given. Patient’s symptoms improved within 72 hours and diagnosis of Tolosa-Hunt Syndrome was confirmed. According to the National Organisation for Rare Disorders (NORD) the average age of onset for this condition is 41 years. The pathogenesis is thought to be inflammation of unknown etiology. The criteria for diagnosis fo Tolosa-Hunt syndrome is given by International Headache Society, which includes: Unilateral headache; MRI or biopsy demonstrating granulomatous inflammation of cavernous sinus, superior orbital fissure or orbit; ipsilateral nerve palsy involving one or more of 3rd, 4th and/or 6th cranial nerves; no alternate diagnosis based on the symptoms; specific history of ipsilateral headache localized to the ipsilateral brow and eye and it should occur 2 week before the oculomotor palsy or along with it. Our patient met all the criteria mentioned above. Ruling out other causes of headache and ophthalmoplegia is important in making the diagnosis. A differential diagnosis includes cavernous sinus thrombosis, stroke, vasculitis, myasthenia gravis, Miller Fisher variant of GBS, multiple sclerosis and idiopathic intracranial hypertension. Currently, inadequate data is available to determine the best route and duration of treatment with steroids. Our patient received oral steroid 100 mg for 3 days followed by slow steroid taper and had improvement in symptoms. Although a rare disorder, it is important to consider Tolosa-Hunt syndrome in the differential diagnosis of patients who presents with headaches and visual changes, especially after ruling out other common causes.
| Identifer | oai:union.ndltd.org:ETSU/oai:dc.etsu.edu:asrf-1090 |
| Date | 05 April 2018 |
| Creators | Maguire, Joseph, El Iskandarani, Mahmoud, Elamparithi, Sudharsan Aswin, Bansal, Apurva, Snyder, Thad |
| Publisher | Digital Commons @ East Tennessee State University |
| Source Sets | East Tennessee State University |
| Detected Language | English |
| Type | text |
| Format | application/pdf |
| Source | Appalachian Student Research Forum |
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