A 58 year-old female was evaluated for chest pain, bradycardia, severe headache, and left periorbital pain with ipsilateral ophthalmoplegia. A warm sensation on the left side of her face with decreased sensation to light touch was noted. Her past medical history included hypertension, arrhythmia, and trauma. She previously visited the emergency department at Sutter Memorial Hospital (Sacramento, CA, USA) on two separate occasions for severe hemicranial pain, which remained constant for 1 week. Computed tomography (CT) scans of the brain without contrast were performed during both visits, with neither showing any acute intracranial abnormalities. CT angiogram (CTA) showed no signs of a dissecting ICA or mass in the superior stellate ganglion of the neck, but it revealed 39% stenosis of the left ICA at the intracavernous portion (Figure 1). No enlargement of the superior orbital vein was noted. The patient was observed to be bradycardic with a pulse ranging from 29 to 50 beats per minute, including a 3.2 second pause. However, electrocardiogram, cardiac serology (creatinine kinase, creatinine kinase-muscle/brain enzyme type, troponin I, prothrombin time, partial thromboplastin time), chest radiography, and coronary arteriography were all normal. Initial magnetic resonance imaging (MRI) and magnetic resonance angiogram (MRA) showed no abnormalities of the orbital apex or surrounding structures. Thrombus was not observed in the superior orbital vein or cavernous sinus. Neurologic consultation considered it to be an isolated third nerve palsy.
Ophthalmologic evaluation disclosed complete ptosis and ophthalmoplegia in the left eye, with the eye in central position (Figure 2). Proptosis was not noted. Visual acuity was 20/25 in the right eye and 20/50 in the left eye. Both pupils were equal, round, and reactive to light. Intraocular pressures were normal. Ophthalmoscopy revealed a slightly edematous left optic nerve, but no engorgement or tortuosity of the retinal vasculature. Ophthalmologic findings were consistent with a partial third, and complete fourth and sixth nerve paresis with hypoesthesia in the ophthalmic and maxillary distributions of the left trigeminal nerve. A subsequent MRI performed 3 days after the initial MRI disclosed a lesion in the left superior orbital fissure with mild extension along the anterior aspect of the left cavernous sinus (Figure 3A), and mild edema of the lateral rectus (Figure 3B). Complete blood count, thyroid stimulating hormone, glucose, sedimentation rate, rheumatoid factor, antinuclear antigen, proteinase 3 antibodies, myeloperoxidase antibodies, Quantiferon -TB Gold (Cellestis Limited, Carnegie, VIC, Australia), human immunodeficiency virus, herpes simplex virus, and fluorescent treponemal antibody absorption test results were normal. Two complete sets of cerebrospinal fluid studies (CSF), including opening pressure, India ink smear, glucose, total protein, angiotensin converting enzyme, and a venereal disease research laboratory test showed no evidence of malignancy or infection. The patient was administered 1 g of intravenous (IV) methylprednisolone (Solu-Medrol ; Pfizer, New York, NY, USA) over 45 minutes, followed by 250 mg every 8 hours daily for 3 days while in hospital. She was discharged with 40 mg of oral prednisone on a tapered course (40 mg for 5 days, 20 mg for 5 days, 10 mg for 5 days, 5 mg for 5 days, 2.5 mg for 5 days, then discontinued).
The patient's periorbital and hemicranial pain was reduced within 48 hours. She was referred for neurosurgical consultation, but she declined. An additional MRI/MRA was obtained at a 4-month follow-up and revealed no lesions or abnormalities. The ophthalmoplegia and ptosis had also resolved. She continues to be monitored and remains symptom free (March 2014).
Written informed consent was obtained from the patient to allow the publication of photographs for educational purposes. This study adhered to the tenets of the Declaration of Helsinki and was Health Insurance Portability and Accountability Act (HIPAA) compliant. Sutter Health Central Area Institutional Review Committee ruled that approval was not required for this study.