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Show Journal of Clinical Neuro- ophthalmology 9( 1): 3-- 6, 1989. Temporal Arteritis Associated with Borrelia Infection A Case Report Louis D. Pizzarello, M. D., Alan B. MacDonald, M. D., Robert Semlear, M. D., Frank DiLeo, M. D., and Bernard Berger, M. D. © 1989 Raven Press, Ltd., New York A 71- year- old man had sudden vision loss associated with headache. A temporal artery biopsy revealed a typical picture of giant cell arteritis. Subsequent steroid therapy failed to restore sight. A later blood culture contained spirochetes compatible with Borrelia species, and a silver stain of the temporal artery biopsy specimen demonstrated a similar spirochete. Treatment with Lv. ceftriaxone sodium led to some limited return of sight. To our knowledge, this is the first case report of a spirochete compatible with Borrelia found in a temporal artery biopsy specimen. Key Words: Borrelia burgdorferi- Giant cell arteritisLyme disease-- Spirochete-- Temporal arteritis. From Southampton Hospital, Southampton, New York. Address correspondence and reprint requests to Dr. L. D. Pizzarello, Southampton Hospital, 240 Meetinghouse Lane, Southampton, NY 11968 U. S. A. This work was presented at the American Academy of Ophthalmology Annual Meeting, Dallas, November 1987. 3 Lyme disease, a syndrome of rash, fever, and arthritis, was first described in 1975 in Connecticut ( 1). Subsequently, many other forms and manifestations of the disease have been described, including several involving the eye and visual structures ( 2- 4). There has also been a case of temporal arteritis associated with Lyme disease, without tissue confirmation ( 5). This is a report of a patient with biopsy- proven temporal arteritis who also had a spirochete compatible with a Borrelia species in a temporal artery biopsy specimen and in a dark- field preparation of a peripheral blood sample. CASE REPORT A 71- year- old male dentist had a I- day history of inferior visual field loss in his left eye. He complained at that time of a dull temporal headache that had persisted for 1 month and that had been treated by a chiropractor. He denied having a fever, rash, or tick bite but had experienced a small weight loss. His ocular history was pertinent for an arnblyopic right eye. At initial examination, vision with correction was 20/ 50 in the right eye and 20/ 80 in the left. Pupillary responses revealed a mild afferent defect in the left eye. Early nuclear sclerosis was present in each eye. Intraocular pressures were 18 rom Hg in each eye. The optic nerve in the left eye showed pale swelling in the superior half. The right disc was normal, with a cup- to- disc ratio of 0.4. Visual field testing demonstrated an altitudinal defect below in the left eye, with a normal field in the right eye. His erythrocyte sedimentation rate ( ESR) was 38 mmlh. In view of the history and findings, a diagnosis of ischemic optic neuropathy secondary to temporal arteritis was made. The patient was 4 FIG. 1. Temporal artery biopsy specimen showing typical appearance of giant cell arteritis. Giant cells are indicated by arrows ( hematoxylin- eosin, original magnification x 73). L. D. PIZZARELLO ET AL. c D FIG. 2. A: Multinucleated giant cell from temporal artery biopsy specimen ( Warthin Stary silver stain original magnification x 160). B: Borrelia spirochetes within multinucleated giant cell ( Fig. 3A) ( original magnification x800). C: Reference Borrelia spirochetes suspended in agar ( Warthin Stary silver stain, original magnification x800) ( American Type Culture Collection # 35211). 0: Borrelia spirochetes suspended in agar ( Warthin Stary silver stain, original magnification x 800) ( American Type Culture Collection # 35211). I eli" Neuro- ophthlllmlll, \" 1'/ 4 \ j, i 1. l~ lS~ TEMPORAL ARTERITIS 5 asked to take 80 mg of prednisone per day. Inadvertently, the patient took 240 mg of prednisone in the first 24- h period. Despite this, his vision had deteriorated to perception of hand motions by the next day, when bilateral temporal artery biopsies were performed. At that time, the left disc was swollen and pale. To preserve vision in the patient's nonamblyopic eye, he was admitted to the hospital and received i. v. Decadron, 8 mg every 6 h for 24 h. At this point, his vision in the left eye began to stabilize. He was discharged and continued to take 80 mg/ day prednisone, gradually tapering to 20 mg/ day. His ESR dropped quickly to 5 mrnlh. Vision slowly improved to counting fingers at 5 feet. Two months after his initial presentation, a Lyme disease titer was requested, and a specimen of blood was cultured for Borrelia species. These studies were obtained because the patient resides in a community where Lyme disease is endemic, and a previous report of temporal arteritis associated with Lyme disease had been discussed at a recent hospital staff seminar. The Lyme titer, done by the indirect immunofluorescent method, was reactive at a dilution of only 1/ 64, a titer that is not considered significant. However, a spirochete compatible with Borrelia but not B. burgdorferi was identified in peripheral blood cultured in modified Kelly's medium which supports the growth of Borrelia species. Serum FTA- ABS and VDRL tests were nonreactive. A course of ceftriaxone sodium was administered over 7 days at a dose of 2 g daily intravenously. A repeat culture of a peripheral blood sample yielded no evidence of spirochetes. The patient reported feeling better, but his vision remained at counting fingers at 5 feet. A second course of prednisone, 80 mg/ day, failed to improve vision. Currently, the optic nerve in the left eye shows marked pallor. The loss of vision in the patient's nonamblyopic eye necessitated his sudden retirement from dentistry. PATHOLOGICAL FINDINGS The temporal artery biopsy specimen demonstrated the classical appearance of temporal or giant cell arteritis ( Fig. 1). The internal elastic lamina of the vessel showed segmental obliteration by multinucleated giant cells, lymphocytes, and eosinophils ( Fig. 2). The intima was thickened, and the lumen of the vessel was severely narrowed. A unique observation was the identification of a spirochete by Warthin Starry silver impregnation in the cytoplasm of a multinucleated giant cell near the junction of elastica with the media of the temporal artery ( Fig. 3). The spirochete within the giant cell is morphologically indistinguishable from a reference strain of Borrelia grown in the laboratory, suspended in agar, and processed for histologic sections using ordinary pathological technique. DISCUSSION The patient described in this report had what appeared to be a clear case of ischemic optic neuropathy. The coexisting clinical features and positive temporal artery biopsy would confirm the diagnosis of temporal or giant cell arteritis. Treatment with high- dose corticosteroid therapy did not prevent marked vision loss. We evaluated the possibility of spirochetal infection because a previous article had described a patient with Lyme disease associated with a clinical picture of a temporal arteritis- like illness. In addition, we were aware of a case of syphilis with temporal arteritis in which FIG. 3. A:. ~ em'poral artery biopsy ( elastic stain, original magnlf. lcatlon. x 160). B: Temporal artery biopsy ( elastiC stain, onglnal magnification x800). I Clin Neuro- ophthalmol. Vol. 9, No. 1, 1989 6 L. D. PIZZARELLO ET AL. spirochetes were identified in the wall of the diseased temporal artery ( 7). Serology studies failed to confirm that our patient was exposed to the Lyme disease spirochete B. burgdorferi, but motile spirochetes morphologically consistent with another Borrelia species were seen on dark- field examination of a cultured blood sample. The precise species could not be identified, and following parenteral antibiotic therapy, the spirochetes could not be cultured from the patient's blood. To our knowledge the identification of spirochetes by silver impregnation within the cytoplasm of a multinucleated giant cell has not been previously reported. Multinucleated giant cells are one possible sign of intracellular bacterial pathogens ( i. e., Mycobacterium tuberculosis) and are also seen in conditions where antigens from bacterial pathogens provoke an immune response. Treponema pallidum has been identified within the cytoplasm of macrophages and nerve cells by electron microscopic techniques. Borrelia burgdorferi, the spirochete that causes Lyme borreliosis, has been seen in macrophages in experimentally infected animals. Therefore, certain pathogenic spirochetes may be found in intracellular locations, as was seen in this case. I Clill Neuro- ophtlzall1lol. Vol. 9. No. 1, jQS9 We recommend that physicians consider spirochetal infection ( syphilis, Lyme disease, or other borreliosis infections) when a case of temporal arteritis is encountered. Serology studies and special histochemical studies are currently the only methods generally available to evaluate the possibility of spirochetal infection. In retrospect, in the case we present, earlier therapy with a combination of antibiotics and corticosteroids may have prevented the patient's permanent vision loss. REFERENCES 1. Steere AC, Malawista SE, Snydman DR. Lyme arthritis: an epidemic of oligoarticular arthritis in children and adults in three Connecticut communities. Arthritis Rheum 1977; 20: 717. 2. MacDonald AB. Lyme disease: a neuro- ophthalmologic view. I Clin Neuro OphthalmoI1987; 7: 185- 90. 3. Wu G, Lincoff H, EUsworth RM, et aL Optic disc edema and Lyme disease. Ann OphthalmoI1986; 18: 252- 5. 4. Steere AC, Duray PH, Danny] H, et al. Unilateral blindness caused by infection with the Lyme disease spirochete, Borrelia burgdorferi. Ann Intern Med 1985; 103: 383- 4. 5. Schecter SL. Lyme disease associated with optic neuropathy. Am I Med 1986; 81: 143- 5. 6. Barbour AG. Isolation and cultivation of Lyme disease spirochetes. Yale I Bioi Med 1984; 57: 521- 5. 7. Smith ] L, Israel CW, Hamer RE. Syphilitic temporal arteritis. Arch Ophthalmol 1967; 78: 284- 8. [VBlymedisease] |
