Multiple Cranial Nerve Palsies in Giant Cell Arteritis

Original Contribution Multiple Cranial Nerve Palsies in Giant Cell Arteritis Michael Ross, MD, Lulu Bursztyn, MD, Rosanne Superstein, MD, Mark Gans, MD Abstract: Giant cell arteritis (GCA) is a systemic vasculitis of medium and large arteries often with ophthalmic involvement, including ischemic optic neuropathy, retinal artery occlusion, and ocular motor cranial nerve palsies. This last complication occurs in 2%-15% of patients, but typically involves only 1 cranial nerve. We present 2 patients with biopsy-proven GCA associated with multiple cranial nerve palsies. Journal of Neuro-Ophthalmology 2017;37:398-400 doi: 10.1097/WNO.0000000000000529 © 2017 by North American Neuro-Ophthalmology Society A lthough vision loss is the most common neuroophthalmic manifestation of giant cell arteritis (GCA), diplopia is also a recognized complication. This usually occurs because of isolated cranial nerve involvement (1,2). We evaluated 2 patients with GCA with multiple cranial nerves affected, a rare occurrence with this inflammatory vasculitis. CASE REPORTS Patient 1 An 80-year-old man was evaluated in the emergency department for generalized weakness. In addition, he had a 3-week history of presumed bacterial conjunctivitis in his right eye, and a 1-week history of a bilateral frontal headaches and pain on chewing. He was found to be unable to see with his right eye, but the precise onset was unclear, as the patient was minimally communicative. His Department of Ophthalmology (MR), McGill University, McGill Academic Eye Centre, Montreal, Quebec, Canada; Department of Ophthalmology (LB), Ivey Eye Institute, Schulich School of Medicine & Dentistry, Western University, London, Ontario, Canada; and Department of Ophthalmology (RS, MG), McGill University Montreal, Quebec, Canada. The authors report no conflicts of interest. Address correspondence to Michael Ross, MD, Department of Ophthalmology, McGill University, McGill Academic Eye Centre, 5252 de Maisonneuve West, Suite 400, Montreal, Quebec H4A 3S5, Canada; E-mail: [e-mail] 398 medical history was significant for memory loss over the past 5 years. In the 5 months preceding his presentation, he suffered from lethargy, weight loss, and anorexia. On examination, he was lethargic but responsive. Vision was no light perception (NLP) in the right eye, and 20/50 in the left eye. The pupils were isocoric, but there was a right relative afferent pupillary defect and complete right ptosis and ophthalmoplegia. Eyelid position and eye movements were normal in the left eye. Ophthalmoscopy in each eye was unremarkable. Laboratory studies revealed a sedimentation rate (ESR) of 57 mm/h and C reactive protein (CRP) of 49 mg/L (normal range: 0-10.0 mg/L). Noncontrast brain computed tomography (CT) demonstrated suspicion for fullness of the right superior ophthalmic vein, but was otherwise normal. CT angiography and venography were normal. The patient was treated with intravenous methylprednisolone (1 g daily for three days) followed by 60 mg of prednisone daily. A temporal artery biopsy demonstrated active arteritis, with inflammation of the intima and destruction of the internal elastic lamina. The dose of prednisone was slowly tapered, and the patient was taking 20 mg/d 6 months after initial presentation. His lethargy and generalized weakness resolved, but vision in the right eye remained NLP, and the right optic disc became pale. His right eye movements improved with only a residual mild deficit in upgaze. Patient 2 A 73-year-old man was admitted to hospital with general malaise, nausea, and constipation. He complained of chronic right-sided headache, jaw pain, anorexia, and a 50 lb weight loss over the previous 6 months. On the day following admission, he developed complete loss of vision in his right eye. Vision transiently decreased in his left eye, but improved over several hours. On examination, acuity was NLP in the right eye and 20/50 in the left eye. Pupils measured 4 mm in the right eye, 3 mm in the left eye, with a right relative afferent pupillary defect. There was complete ptosis and near total ophthalmoplegia of the right eye, with preservation of incyclotorsion on attempted downglaze. Ross et al: J Neuro-Ophthalmol 2017; 37: 398-400 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Original Contribution Sensation was decreased along the right forehead, whereas facial movement was intact. The right cornea was edematous, preventing a view of the fundus. Intraocular pressures were 7 mm Hg in the right eye and 8 mm Hg in the left eye. The left anterior segment, retina, and optic disc were normal. The patient's ESR was 63 mm/hr, and a CRP was 15.1 mg/L (normal: ,0.5 mg/L). Brain magnetic resonance imaging revealed abnormal signal involving the right optic nerve (Fig. 1). There were no other cranial nerve abnormalities detected. A temporal artery biopsy demonstrated marked intimal thickening with disruption of the internal elastic lamina and rare multinucleated giant cells. Treatment with intravenous dexamethasone (4 mg 4 times daily for one week) was followed by 60 mg of prednisone. The patient's headache, appetite, and energy level improved, but there was no recovery of vision, and the right cornea remained edematous. At 2 months followup, right ptosis and limited eye movements of the right eye had resolved, and intraocular pressures were 8 mm Hg in the right eye and 13 mm Hg in the left eye. The right cornea continued to be edematous and ophthalmoscopy revealed optic disc pallor and attenuation of the retinal arteries. DISCUSSION We describe 2 patients with GCA who developed vision loss because of posterior ischemic optic neuropathy (PION) and multiple other cranial neuropathies (Patient 1: 3, 4, 6; Patient 2: 3, 6, trigeminal). Although vision loss in GCA is most always permanent, other cranial nerve palsies resolve or improve (3,4), as was the case in our patients. Diplopia due to ophthalmoplegia may be due to ocular motor cranial nerve involvement or extraocular muscle ischemia. Although the third nerve is most frequently affected (1,5), fourth and sixth nerve palsies also have been reported (3,6). Multiple cranial neuropathies due to GCA are rare. Li et al (7) reported a 74-year-old man with biopsyproven GCA who experienced right ischemic optic neuropathy and ipsilateral third and sixth nerve palsies. Fytili et al (8) described a 68-year-old man with right sixth and eighth nerve involvement, who subsequently experienced hoarseness because of laryngeal nerve palsy. His temporal artery biopsy was also positive. The pathophysiology of GCA is a result of luminal occlusion of inflamed arterial walls, and may affect a number of vessels supplying the eye. Anterior ischemic optic neuropathy is due to involvement of the short posterior ciliary arteries (1,2,9), whereas PION, as in our patients, is due either to intraorbital involvement of the pial vessels perfusing the optic nerves, or intracranial involvement of branches of the internal carotid, anterior cerebral, and anterior communicating arteries (1). Extraocular muscles may be affected by ischemia, providing another pathophysiologic mechanism for diplopia (10). This may fall into the spectrum of orbital infarction syndrome, which has been reported in GCA (11). In this syndrome, all orbital contents are affected, causing blindness, orbital pain, anterior and posterior segment ischemia, and total ophthalmoplegia. Our patient 2 demonstrated optic neuropathy, partial ophthalmoplegia, and ischemia of anterior and posterior segments. Although this patient could be considered within the spectrum of orbital infarction syndrome, we believed that sparing of the fourth nerve and lack of eye pain were more in keeping with multiple cranial nerve palsies than true orbital infarction. Similar to our patients, reported cases of GCA causing orbital infarction or multiple cranial neuropathies demonstrated permanent visual impairment, but with resolution of ophthalmoplegia and anterior segment ischemia (7,8,11,12). Our report adds to the myriad of findings that can occur in patients with GCA. Although multiple cranial nerve FIG. 1. A. Diffusion-weighted imaging shows hyperintense signal along the course of the right optic nerve (A) that is also apparent on the fluid-attenuated inversion recovery study (B). Ross et al: J Neuro-Ophthalmol 2017; 37: 398-400 399 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Original Contribution involvement is rare in patients with GCA, this vasculitis must be kept in the differential diagnosis. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: M. Gans, R. Superstein, L. Bursztyn, and M. Ross; b. Acquisition of data: R. Superstein, L. Bursztyn, and M. Ross; c. Analysis and interpretation of data: M. Gans, R. Superstein, L. Bursztyn, and M. Ross. Category 2: a. Drafting the manuscript: M. Gans, R. Superstein, L. Bursztyn, and M. Ross; b. Revising it for intellectual content: M. Gans, R. Superstein, L Bursztyn, and M. Ross. Category 3: a. Final approval of the completed manuscript: M. Gans, R. Superstein, L. Bursztyn, and M. Ross. REFERENCES 1. Chew SSL, Kerr NM, Danesh-Meyer HV. Giant cell arteritis. J Clin Neurosci. 2009;16:1263-1268. 2. Liu GT, Glaser JS, Schatz NJ, et al. 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