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Show Journal of Clinical Neuro- ophlhalmology 9( 21: 119- 121. 1989. © 1989 Raven Press, Ltd., New York Sixth Nerve Palsies, Temporal Artery Biopsy, and Necrotizing Vasculitis Lyn A. Sedwick, M. D., and Curtis E. Margo, M. D. A 79- year- old man with diplopia and weakness was found to have necrotizing vasculitis consistent with polyarteritis nodosa on temporal artery biopsy. Although he had no evidence of visceral involvement from vasculitis, he responded well to corticosteroid therapy. This case illustrates a nosological problem that can occur when dealing with a disease that has diverse clinical manifestations and nonspecific laboratory findings. Moreover, this case demonstrates the value of including a branch artery segment when biopsying the temporal artery. Key Words: Necrotizing vasculitis- Polyarteritis nodosa- Sixth nerve palsy- Temporal artery biopsy. From the Department of Ophthalmology, University of South Florida, Tampa, Florida. Address correspondence and reprint requests to Dr. L. A. Sedwick at 2501 N. Orange Avenue, Suite 501, Orlando, FL 32804, U. S. A. This work was presented in part before the Southeastern Neurophthalmology Meeting, Panama City, FL, June 1987, and the Bascom Palmer Neuro- ophthalmology Meeting, Miami, FL, December 1987. 119 In 1986 Coppeto and Miller ( 1) described a case of polyarteritis nodosa diagnosed by temporal artery biopsy. We report a patient with bilateral sixth nerve palsy in whom temporal artery biopsy showed necrotizing vasculitis consistent with polyarteritis nodosa without clinical evidence of visceral involvement. CASE REPORT A 79- year- old man was admitted to the hospital with a history of dizziness, nonspecific weakness, and intermittent fever. For 6 months, his cataract surgeon had placed increasing- strength Fresnel prisms in his glasses to control horizontal diplopia. Medical history was significant for arthralgias treated briefly with oral corticosteroids several years previously. Physical examination was unremarkable except for the ocular findings. At bedside, he showed normal visual function, no ptosis, normal optic disks, and a moderate esotropia with deficits of abduction, left eye more than right. Laboratory testing revealed an anemia with leukocytosis and elevated Westergren sedimentation rate of 80 mm! h. Fluorescent treponemal antibody absorption test was nonreactive as was anti- nuclear antibody, but rheumatoid factor was positive at 1: 160 and liver function tests were mildly abnormal. Serum creatinine was normal at 1.0 mg/ dl but clearance was slightly reduced at 62 mllmin ( normal 80- 120 ml/ min). Urinalysis was normal. Cerebrospinal fluid had 8 mononuclear cells, with repeat tap showing 17 with normal protein concentration. Cultures of spinal fluid, urine, and blood were negative. Computerized tomographic and magnetic resonance scanning with attention to the cavernous sinuses and base of brain were normal as was otolaryngological examination. A Tensilon test was negative. 120 L. A. SEDWICK AND C. E. MARGO Temporal artery biopsy disclosed no inflammation in the main segment of the artery but did show focal fibrinoid necrosis and chronic inflammation in a branch artery ( Fig. 1). The chronic inflammatory reaction consisted mainly of lymphocytes. Scattered histiocytes and some neutrophils were also present, but no giant cells were seen. The patient was treated with oral corticosteroids for presumed polyarteritis nodosa, and his systemic symptoms and ocular motility have improved during 2 years of follow- up. The patient has not developed any manifestations of systemic lupus erythematosis or rheumatic disease. DISCUSSION Currently, there is no completely satisfactory way to classify the human vasculitides. Since there is substantial overlap in the histologic appearance of many forms of vasculitis, diagnoses often are achieved by clinicopathologic correlation. In this case, the temporal artery biopsy revealed a focaL necrotizing vasculitis with features of polyarteritis nodosa. Without clinical evidence of mesenteric, renaL or hepatic involvement, however, the diagnosis of polyarteritis is presumptive and based somewhat on the exclusion of other forms of necrotizing vasculitis. Typically, polyarteritis affects medium- to smallsized arteries, particularly at the site of bifurcation, as in our case. The inflammatory reaction consists of neutrophils or mononuclear cells, depending on the stage of evolution of the lesion ( 2- 4). Areas of necrosis contain fibrin, which imparts a smudgy magenta appearance to the vascular wall on sections stained with hematoxylin and eosin. As necrotic vascular walls weaken, aneurysms usually develop in the crotch of branching vessels where a medium- sized artery gives rise to a vessel of much smaller caliber. When this finding occurs in mesenteric vessels, the angiographic appearance is diagnostic. Polyarteritis nodosa, however, can potentially affect any vascular bed in the body. Clinical manifestations reflect the degree and location FIG. 1. Cross section shows no inflammation or scarring in the temporal artery ( TA), but a small branch artery ( 8; curved arrow) has a marked inflammatory reaction. Hematoxylin- eosin, x 12. Inset: Occluded vascular lumen of the small branch artery. The vessel wall from about 2 to 7 o'clock ( straight arrows) has undergone fibrinoid ~,"~ r' 3t' 0" Hematoxylin- eosin, >< 80. SIXTH NERVE PALSY AND NECROTIZING VASCULITIS 121 of vessel involvement. While the morphologic changes described above are characteristic of polyarteritis, they are not specific. Similar lesions have been described in patients with systemic lupus erythematosis, hepatitis B antigenemia, and rheumatoid arthritis ( 2,3). Identical vascular changes have been reported in Wegener's granulomatosis as well ( 2). In contrast, temporal arteritis affects large- to medium- sized arteries, which have an elastic lamina. Fragmentation and destruction of the internal elastic lamina are constant features of the disease. Necrosis is uncommon, but when it does occur, the type is coagulative and not fibrinoid ( 5). Although the hallmark of temporal arteritis is giant cell formation in the vicinity of the internal elastic lamina, the cellular reaction may consist solely of lymphocytes and histiocytes, but only rarely includes neutrophils. The diagnosis of polyarteritis may be difficult in the absence of typical visceral involvement, since no specific chemical or serologic findings for the disease exist. Fever, anorexia, weight loss, and weakness are common albeit nonspecific symptoms. The diagnosis may require the exclusion of other diseases known to be associated with necrotizing vasculitis. One criticism of this approach, however, is that localized forms of necrotizing vasculitis may not be truly pathogenetically related to the visceral form of polyarteritis nodosa. Coppeto and Miller ( 1) reported a 76- year- old woman with complaints of transient visual loss whose temporal artery biopsy showed changes similar to those in our patient. The woman subsequently developed abdominal symptoms and the diagnosis of polyarteritis was further substantiated by small bowel biopsy. Three patients were described in a 1978 report who had non- giant cell temporal arteritis, two of whom had polyarteritis nodosa and one who had hypersensitivity angiitis ( 6). Literature review in that article discussed several well- documented cases of non- giant cell temporal arteritis, three with polyarteritis nodosa, three with necrotizing vasculitis, and one with polyarteritis nodosa with hepatitis B ( 7,8). Pathologically verified temporal arteritis and polyarteritis in the same patient have been reported twice ( 7,8). In addition, a 36year- old man with " posterior ischemic optic neuropathy," sixth and third nerve palsies, and polyarteritis nodosa has been described ( 9). Our patient, with probable bilateral sixth nerve palsies and necrotizing vasculitis, reflects the diagnostic difficulty presented by patients who may have an atypical manifestation of a disease whose pathogenesis is poorly understood. The case also stresses the value of including a branch artery segment in temporal artery biopsy specimens whenever possible. REFERENCES 1. Coppeto jR, Miller D. Polyarteritis nodosa diagnosed by temporal artery biopsy. Am JOphthalmoI1986; 102: 541. 2. McCluskey RT, Fienberg R. Vasculitis in primary vasculitides, granulomatoses, and connective tissue disease. Hum Pathol 1983; 14: 305- 15. 3. Fauci AS. Haynes BF. Katz P. The spectrum of vasculitis: clinical, pathologic. immunologic, and therapeutic considerations. AIIII Intern Med 1978; 89: 660- 76. 4. Zeek PM. Periarteritis nodosa and other forms of necrotizing angiitis. N Engl I Med 1953; 248: 764- 71. 5. McDonnell Pj, Moore GW, Miller NR, et al. Temporal arteritis: a clinicopathologic study. Opthalmology 1986; 93: 51830. 6. Morgan Gj, Harris ED. Non- giant cell temporal arteritis. Arthritis Rheum 1978; 21: 362-< i. 7. O'Neill WM, Hammer SP. Giant cell arteritis with visceral angiitis. Arch Intern Med 1976; 136: 1157-< i0. 8. Highton J, Anderson KR. Concurrent polyarteritis nodosa and temporal arteritis. NZ Med , 1984; 97: 766- 7. 9. Hutchinson jH. Polyarteritis nodosa presenting as posterior ischaemic optic neuropathy. , Roy Soc Med 1984; 77: 1043-< i. 1Clin Neuro- ophthalmol. 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