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Show Third Nerve Palsy as the Initial Manifestation of Giant Cell Arteritis Matthew J. Thurtell, MBBS, FRACP, Reid A. Longmuir, MD Objective: Giant cell arteritis (GCA) is rarely reported as a cause of third nerve palsy. We describe the presentation and course of patients with third nerve palsy as the sole initial ocular manifestation of GCA. Methods: Retrospective chart review of patients with third nerve palsy as the presenting sign of GCA. Symptoms, signs, and inflammatory marker levels at presentation and on follow-up were analyzed. All patients had imaging of the brain and circle of Willis, to exclude a compressive or inflammatory lesion, and had a temporal artery biopsy showing granulomatous arteritis. Results: Four patients (aged 63-82) were identified and included. One patient had a complete third nerve palsy with pupil involvement, whereas the other 3 had third nerve pal-sies without pupil involvement. Three patients had ipsilat-eral periorbital/brow pain, and the other patient had temporal headache. Two patients reported no systemic symptoms of GCA but had elevated inflammatory markers. One patient had normal inflammatory markers but reported systemic symptoms of GCA. All patients had rapid improve-ment in symptoms and signs after high-dose oral predni-sone was started with all showing complete recovery within weeks. Conclusions: GCA can rarely present with acute painful third nerve palsy, mimicking the presentation of a microvascular cause. The third nerve palsy often improves rapidly after steroid treatment is started. The presence of GCA symp-toms or elevated inflammatory markers in a patient older than 50 years with an acute third nerve palsy should prompt initiation of high-dose steroid treatment and temporal artery biopsy. Journal of Neuro-Ophthalmology 2014;34:243-245 doi: 10.1097/WNO.0000000000000116 © 2014 by North American Neuro-Ophthalmology Society When third nerve palsy develops acutely with associated pain or headache in a middle-aged or elderly patient with risk factors for vascular disease, it is often secondary to microvascular ischemia (1). However, alternative possibilities, such as third nerve compression by an aneurysmal, neoplastic, or inflammatory lesion must also be considered, especially when there is pupil involvement (2). Imaging of the brain (e.g., magnetic resonance imaging [MRI] with contrast) and circle of Willis (e.g., magnetic resonance angiography [MRA] or computed tomographic angiography [CTA]) generally is obtained to exclude compression or enhancement of the third nerve (2,3). Because other causes of acute painful third nerve palsy are less common (1), other investigations, such as labo-ratory studies, initially may be deferred. We report 4 patients in whom third nerve palsy was the sole initial ocular manifes-tation of giant cell arteritis (GCA).We discuss the implications for the work-up of patients with acute third nerve palsy and the prognosis for recovery when GCA causes third nerve palsy. METHODS We retrospectively reviewed the charts of 4 patients presenting to our institution with third nerve palsy as the only ocular manifestation of GCA. All patients had been assessed in a standardized fashion, with a structured history and neuro-ophthalmic examination. Symptoms and signs at presentation and on subsequent follow-up were documented. All patients had undergone imaging of the brain and circle of Willis (with either MR or CT), to exclude third nerve compression and enhancement, and all had blood drawn to determine the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level. The diagnosis of GCA was confirmed on the basis of a temporal artery biopsy showing granulomatous arteritis. CASE REPORTS Patient 1 An 82-year-old woman reported a 1-week history of binocular diagonal diplopia and left-sided ptosis. She had developed Departments of Ophthalmology and Visual Sciences (MJT, RAL) and Neurology (MJT), University of Iowa, Iowa City, Iowa; and Depart-ments of Neurology (MJT) and Ophthalmology (RAL), VA Medical Center, Iowa City, Iowa. The authors report no conflicts of interest. Address correspondence to Matthew J. Thurtell, MBBS, FRACP, Department of Ophthalmology and Visual Sciences, University of Iowa Hospitals and Clinics, 200 Hawkins Drive PFP, Iowa City, IA 52242; E-mail: matthew-thurtell@uiowa.edu Thurtell and Longmuir: J Neuro-Ophthalmol 2014; 34: 243-245 243 Original Contribution Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. severe left-sided brow and periorbital pain about 12 hours before the ocular symptoms began. Her medical history was remarkable for Type 2 diabetes, hypertension, and dyslipide-mia. Review of systems was positive for malaise, weight loss, and proximal myalgias, but she denied scalp tenderness and jaw claudication. Visual acuity was 20/20, right eye and 20/50, left eye. Confrontation visual fields were full in both eyes. There was a left pupil-involving third nerve palsy with marked limitation of adduction, elevation, and depres-sion, complete ptosis, and a dilated unreactive pupil. There was no relative afferent pupillary defect. Slit-lamp examination showed nuclear sclerotic cataract in both eyes but was otherwise unremarkable. Intraocular pres-sures were 12 mm Hg in both eyes. Funduscopic examination revealed an epiretinal membrane in the left eye but was otherwise unremarkable. Postcontrast MRI showed chronic small vessel ischemic changes in the white matter, and CT angiogram of the circle of Willis was unremarkable. ESR was 37 mm/hr and CRP was ,0.5 mg/dL (normal ,0.5 mg/dL). Although her third nerve palsy was thought to be secondary to microvas-cular ischemia, she was started on 80 mg of oral prednisone daily because of her systemic symptoms. Temporal artery biopsy showed granulomatous inflammation consistent with GCA. Prednisone was continued and her symptoms rapidly improved. When she returned for follow-up 18 days after presentation, her motility, lid, and pupil abnormalities had significantly improved. Her motility, lids, and pupils were normal at subsequent visits. Patient 2 A 70-year-old woman experienced binocular, diagonal diplopia and right-sided ptosis for 2 days and a severe right-sided brow and periorbital ache 3 days before ocular symptoms began. Her medical history was remarkable for morbid obesity. Review of systems was negative; she denied having scalp tenderness, jaw claudication, or constitutional symptoms. Visual acuity was 20/25, right eye and 20/20, left eye. Confrontation visual fields were full bilaterally. There was a partial right-sided third nerve palsy, with moderate adduction, elevation, and depression deficits and partial ptosis. The pupils were equal and briskly reactive to light without a relative afferent pupillary defect. Slit-lamp examination showed nuclear sclerotic cataracts, and intraocular pressures were 22 mm Hg in both eyes. Fundu-scopic examination was unremarkable in both eyes. Brain MRI showed chronic small vessel ischemic white matter changes and a pituitary macroadenoma without extension into the cavernous sinus. MRA was unremarkable. ESR was 75 mm/hr and CRP was 2.6 mg/dL (normal ,0.5 mg/dL). The patient was started on oral prednisone, 80 mg daily, and temporal artery biopsy was consistent with GCA. Prednisone was continued and her symptoms rapidly improved. When she returned for follow-up 21 days after presentation, examination revealed full ductions and that the ptosis on the right had improved. The ptosis had completely resolved 1 month later. Patient 3 A 65-year-old woman described a 2-week history of increasing left-sided ptosis associated with severe left-sided brow and periorbital pain. Her medical history was remarkable for dyslipidemia and tobacco smoking. Her ophthalmic history was remarkable for anisometropic amblyopia of the left eye and longstanding mild left-sided ptosis. Review of systems was unremarkable; she denied scalp tenderness, jaw claudication, and constitu-tional symptoms. Visual acuity was 20/20, right eye and counting fingers at 1 foot, left eye (unchanged from previous examinations). Confrontation visual fields were full in both eyes. Pupils were equal and reactive to light with 1.2-1.5 log unit relative afferent pupillary defect in the left eye. There was a partial left third nerve palsy, with a severe elevation deficit, moderate adduction and depres-sion deficits, and moderate ptosis. Slip-lamp examination was unremarkable, and intraocular pressures were 16 mm Hg, right eye, and 14 mm Hg, left eye. Funduscopic examination revealed myopic degeneration with a posterior pole staphyloma and tilted optic disc in the left eye but was normal in the right eye. Brain MRI and MRA were within normal limits. ESR was 33 mm/hr and CRP was 2.2 mg/dL (normal ,0.5 mg/dL). Because of her symp-toms and elevated inflammatory markers, she was started on oral prednisone, 80 mg daily. Temporal artery biopsy showed granulomatous arteritis. Prednisone was continued and her symptoms rapidly resolved. When she returned for follow-up 10 days after presentation, examination revealed full ductions and mild left-sided ptosis that was back to her baseline. Patient 4 A 63-year-old man was evaluated for a 3-week history of binocular diplopia and right-sided ptosis. He had devel-oped a severe right temporal headache 10 days before ocular symptoms began. Both the headache and right ptosis had spontaneously improved before presentation. Medical history was remarkable for hypertension. Review of systems was positive for scalp tenderness; he denied jaw claudication or constitutional symptoms. Visual acuity was 20/20 bilaterally, and confrontation visual fields were intact. There was a partial right third nerve palsy, with moderate adduction and depression deficits, and partial ptosis. The pupils were equal in size and briskly reactive to light without a relative afferent pupillary defect. Slit-lamp examination showed mild cataracts, and intraocular pres-sures were 18 mm Hg in both eyes. Funduscopic examination was unremarkable. Brain MRI and MRA were within normal limits. ESR was 33 mm/hr and CRP was 2.2 mg/dL (normal ,0.5 mg/dL). Because of his symptoms and elevated inflammatory markers, he was started on oral prednisone, 80 mg daily. Temporal artery biopsy showed gran-ulomatous arteritis. The prednisone was continued and his 244 Thurtell and Longmuir: J Neuro-Ophthalmol 2014; 34: 243-245 Original Contribution Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. symptoms rapidly resolved. When he returned for follow-up 10 days after presentation, examination revealed full ductions and complete resolution of ptosis. DISCUSSION GCA is infrequently reported as a cause of acute painful third nerve palsy (4-12). In a recent prospective, multi-center, observational case series of patients aged 50 years or older presenting with isolated ocular motor cranial nerve palsies, GCA was not found as a cause of any third nerve palsies but was reported as the cause of 3/62 sixth nerve palsies (13). In most reported cases of third nerve palsy due to GCA, the presentation was similar to microvascular ischemia with pain and isocoric pupils. However, cases with pupil involvement have been reported (5,7,12). One of our 4 patients had a complete pupil-involving third nerve palsy, whereas the other 3 had pupils of equal size. Three of our patients re-ported severe ipsilateral periorbital and brow pain that began either concomitantly with ocular symptoms or up to 3 days prior. The characteristics and timing of the pain were similar to that reported with microvascular third nerve palsy (14). Our findings confirm that the presentation can mimic that of a microvascular third nerve palsy. The mechanism of third nerve palsy because of GCA is presumably similar to that of microvascular ischemia, in which there is ischemic demyelination of the nerve (15-17). It has been hypothesized that the ischemia arises due to vasculitis affecting branches of the posterior cerebral artery, which supply the third nerve (4). A notable finding in our series was that all patients showed rapid improvement in their symptoms and signs, often over days, after the initiation of steroids. This is significantly quicker than would be expected for a microvascu-lar third nerve palsy, which often takes about 3-4 months to recover (18). This rapid recovery in patients with GCA has been reported previously (6-9). Although some reports suggest that recovery of third nerve palsy due to GCA is incomplete (5), our patients experienced complete resolution of their cra-nial neuropathy.We considered the possibility of orbital ische-mia as a cause of our patients' ophthalmoplegia (19), but this seemed unlikely given the pattern of the motility deficits and lack of involvement of the superior oblique and lateral rectus muscles. Three of our 4 patients had elevated inflammatory markers, and 2 had no symptoms to suggest GCA. The 1 patient with normal inflammatory markers did have other symptoms of GCA. Given the potential for severe irreversible vision loss in untreated GCA (20,21), we propose that all patients older than 50 years with an acute onset third nerve palsy be questioned for symptoms of GCA and have laboratory testing to determine the ESR and CRP level. This should be done even if the patient has risk factors for vascular disease. REFERENCES 1. Richards BW, Jones FR Jr, Younge BR. Causes and prognosis in 4,278 cases of paralysis of the oculomotor, trochlear, and abducens cranial nerves. Am J Ophthalmol. 1992;113:489- 496. 2. Lee AG, Hayman LA, Brazis PW. The evaluation of isolated third nerve palsy revisited: an update on the evolving role of magnetic resonance, computed tomography, and catheter angiography. Surv Ophthalmol. 2002;47:137-157. 3. Trobe JD. Searching for brain aneurysm in third cranial nerve palsy. J Neuroophthalmol. 2009;29:171-173. 4. Fisher CM. Ocular palsy in temporal arteritis. Minn Med. 1959;42:1258-1268. 5. Davies GE, Shakir RA. Giant cell arteritis presenting as oculomotor nerve palsy with pupillary dilatation. Postgrad Med J. 1994;70:298-299. 6. Bondeson J, Asman P. Giant cell arteritis presenting with oculomotor nerve palsy. Scand J Rheumatol. 1997;26:327-328. 7. Loffredo L, Parrotto S, Violi F. Giant cell arteritis, oculomotor nerve palsy, and acute hearing loss. Scand J Rheumatol. 2004;33:279-280. 8. Lazaridis C, Torabi A, Cannon S. Bilateral third nerve palsy and temporal arteritis. Arch Neurol. 2005;62:1766-1768. 9. Polak P, Pokorny V, Stvrtina S, Tauchmannova H, Malis F, Rovensky J. Temporal arteritis presenting with paresis of the oculomotor nerve, and polymyalgia rheumatica, despite a low erythrocyte sedimentation rate. J Clin Rheumatol. 2005;11:242-244. 10. Day A, Malik N. Giant cell arteritis presenting as painful third nerve palsy. Br J Hosp Med. 2006;67:383. 11. Madge SN, Klys H, Twomey JM. Giant cell arteritis presenting as painful third nerve palsy with normal erythrocyte sedimentation rate. Br J Hosp Med (Lond). 2006;67:268. 12. Asensio-Sanchez VM, Morales-Gomez I, Rodriguez-Vaca I. Third nerve palsy as the only manifestation of occult temporal arteritis. Arch Soc Esp Oftalmol. 2009;84:395- 398. 13. Tamhankar MA, Biousse V, Ying GS. Isolated third, fourth, and sixth cranial nerve palsies from presumed microvascular versus other causes. Ophthalmology. 2013;120:2264-2269. 14. Wilker SC, Rucker JC, Newman NJ, Biousse V, Tomsak RL. Pain in ischaemic ocular motor cranial nerve palsies. Br J Ophthalmol. 2009;93:1657-1659. 15. Dreyfus PM, Hakim S, Adams RD. Diabetic ophthalmoplegia: report of case, with postmortem study and comments on vascular supply of human oculomotor nerve. AMA Arch Neurol Psychiatry. 1957;77:337-349. 16. Asbury AK, Aldredge H, Hershberg R, Fisher CM. Oculomotor palsy in diabetes mellitus: a clinic-pathological study. Brain. 1970;93:555-566. 17. Weber RB, Daroff RB, Mackey EA. Pathology of oculomotor nerve palsy in diabetics. Neurology. 1970;20:835-838. 18. Capo H, Warren F, Kupersmith MJ. Evolution of oculomotor nerve palsies. J Clin Neuroophthalmol. 1992;12:21-25. 19. Barricks ME, Traviesa DB, Glaser JS, Levy IS. Ophthalmoplegia in cranial arteritis. Brain. 1977;100:209-221. 20. Hayreh SS, Podhajsky PA, Zimmerman B. Ocular manifestations of giant cell arteritis. Am J Ophthalmol. 1998;125:509-520. 21. Gonzalez-Gay MA, Garcia-Porrua C, Llorca J, Hajeer AH, Branas F, Dababneh A, Gonzalez-Louzao C, Rodriguez Gil E, Rodriguez-Ledo P, Ollier WE. Visual manifestations of giant cell arteritis: trends and clinical spectrum in 161 patients. Medicine (Baltimore). 2000;79:283-292. Thurtell and Longmuir: J Neuro-Ophthalmol 2014; 34: 243-245 245 Original Contribution Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. |
